DRH-EM Education

Perform Vigorous Bimanual massage and administer IV oxytocin.

The most common cause of post partum hemorrhage is uterine atony. Methylergonovine and prostaglandins are additional treatments that can be used. Maternal resuscitation should occur simultaneously, starting with NS fluid bolus and packed RBCs. Other less common causes of early postpartum hemorrhage include lacerations of lower genital tract, retained placenta, uterine rupture, and uterine inversions.

This patient has an inevitable abortion due to her open OS and products of conception in the vault. Expectant management is the most appropriate treatment. A dilation and curettage procedure would also be an appropriate option in this instance.

Significant risk factors of ectopic pregnancy include previous history of ectopic pregnancy, history of infection such as pelvic inflammatory disease, history of tubal surgery (leading to scar formation), the presence of IUD, and assisted reproductive therapy.

The correct answer is magnesium sulfate. This patient’s presentation is concerning for eclampsia. Magnesium sulfate is the mainstay of treatment because it will help to prevent future seizures. The recommendation is to give 4-6 g IV over 15-20 min. An alternative is to give 5 g IM into each buttock. After initial magnesium administration, a drip can be started at 2g/hr.
This patient’s presentation is concerning for ecclampsia. While persistently uncontrolled hypertension increase the risk of stroke, this patient should receive magnesium sulfate first to prevent recurrent seizures (which in turn may increase the blood pressure further).
This patient’s presentation is concerning for eclampsia. Lorazepam can be given for a prolonged seizure, but magnesium is the mainstay of treatment and will prevent future seizures.

This patient presents with abdominal pain and a positive pregnancy test raising the concern for an ectopic pregnancy. Ectopic pregnancy complicates about 1.5 – 2.0% of pregnancies and is potentially life threatening. There are a number of risk factors for ectopic pregnancy including pelvic inflammatory disease, prior tubal surgery, and previous ectopic pregnancy. This patient has an early pregnancy based on the low beta hCG. The transvaginal ultrasound shows an early gestational sac without a yolk sac or fetal pole within the uterus. This ultrasound does not rule out the diagnosis of an ectopic pregnancy as an ectopic pregnancy can cause a decidual reaction in the uterus, which appears similar to an early gestational sac. The definitive ultrasound finding for an intrauterine pregnancy would be the presence of a yolk sac or fetal pole. It is expected that above the discriminatory hCG zone of 1500-2500 mIU, a definitive IUP should be identified. Patients with a beta hCG below the discriminatory zone without a definitive IUP can be managed conservatively with a repeat hCG level in 48 hours (the level should double every 48 hours) and repeat ultrasound.

Rhogam (B & C) is recommended for patients who are Rh negative and have vaginal bleeding. If the mother is exposed to fetal blood, she may develop antibodies that threaten future pregnancies. This patient does not have vaginal bleeding and is Rh positive obviating the need for Rhogam. Methotrexate (A) is a chemotherapeutic agent that can be used for the treatment of early ectopic pregnancy. This approach is not indicated in a wanted pregnancy with a beta hCG below the discriminatory zone as repeat testing may show a viable intrauterine pregnancy.

Postpartum endometritis is the most common puerperal infection, usually developing on the 2nd or 3rd day postpartum. Typically, the lochia has a foul odor, and the patient develops a leukocytosis. The infection begins in the endometrium and can extend to the myometrium or parametrium. It is a serious infection that can lead to complications such as peritonitis, septic thrombophlebitis, and necrotizing fasciitis. The pathogens involved are typically the flora of the bowel, perineum, vagina, and cervix. The strongest risk factor for endometritis is a cesarean section.

In lactating women, mastitis typically presents within a few weeks postpartum, and occurs in 2-10% of breastfeeding women. The infection is almost always unilateral. The most common causative agent is Staphylococcus aureus, which actually originates mainly from the newborn’s pharynx. The antibiotic of choice for non-severe disease is a penicillinase resistant agent, such as dicloxacillin or cephalexin. If the patient has beta lactam sensitivity, clindamycin is recommended. If there is concern for maternal methicillin-resistant staphylococcus aureus (MRSA) colonization, trimethoprim-sulfamethoxazole or clindamycin is recommended. If the patient is unstable, inpatient intravenous vancomycin should be initiated after local and blood cultures are obtained.

Compression of the cord can be life threatening to the fetus. To reduce the risk of cord compression, the examiner should manually elevate the presenting part. An obstetrician should be called immediately because the patient needs an emergent cesarean section. The examiner’s hand should remain in the vagina, elevating the presenting part, while the patient is transported to the operating room and prepared for surgery.

Terbutaline (A) is a tocolytic and is not indicated for treatment of prolapsed umbilical cord. The provider should never try to reduce the cord (B) because this may cause compression and fetal distress. The provider should never cut the cord (C) and attempt delivery because this will cut off the fetus’s blood supply during delivery.

Symptoms of hypermagnesemia are not often seen unless significantly elevated (> 4 meq/L). Neurological symptoms are the most commonly seen symptoms of hypermagnesemia. The initial clinical manifestation of this problem is diminished deep tendon reflexes usually first noted when the plasma magnesium concentration reaches 4–6 meq/L. More severe hypermagnesemia can result in somnolence, loss of deep tendon reflexes, and muscle paralysis, potentially leading to flaccid quadriplegia and, because smooth muscle function is also impaired, decreased respiration and eventual apnea. Parasympathetic blockade—inducing fixed and dilated pupils, thereby mimicking a central brain-stem herniation syndrome—can also be seen in this setting. Treatment is based on renal function and ranges from withdrawal of the magnesium drip to treating with IV fluids, intravenous calcium, and/or dialysis.
Clonus (A) is less likely because hyperexcitability or upper motor neuron lesions typically cause clonus. Tachypnea (D) is also less likely, given magnesium also impairs smooth muscle, which can lead to respiratory depression and eventually apnea. Hypocalcemia, not hypercalcemia (B), is typically seen with hypermagnesemia because magnesium can inhibit the secretion of parathyroid hormone.

Rapid delivery of the head can result in 3rd- and 4th-degree perineal tears. Applying pressure at the perineum can help prevent rapid delivery. As the head emerges from the introitus, the perineum should be supported by a sterile towel placed along the inferior aspect, held in place with one hand. The other hand should support the fetal head. As the infant’s head presents, the inferior hand may then be used to control the fetal chin while the superior hand remains on the crown of the head, supporting the delivery. Once the head delivers, the fetus should be evaluated for nuchal cord.

Although the performance of episiotomies (B) remains controversial, prophylactic episiotomy in the ED is not routinely indicated. Upward pressure on the inferior portion of the head (C) may result in injuries to the anterior structures of the perineum, including the urethra and clitoris. Vigorous stretching of the perineum (D) can predispose patients to perineal tears.

The classic cyanotic congenital heart diseases can be remembered by the “5 Ts”: truncus arteriosus, transposition of the great vessels, tricuspid atresia, tetralogy of Fallot, and total anomalous pulmonary venous return. Other cyanotic cardiac lesions include Ebstein’s anomaly, pulmonary atresia, severe pulmonary stenosis, hypoplastic left heart syndrome, and hypoplastic right heart syndrome.

Atrial septal defect (A), patent ductus arteriosus (B), and ventricular septal defect (D) are lesions with left-to-right shunts and are associated with an increase in pulmonary blood flow. These acyanotic lesions usually manifest with congestive heart failure, rather than cyanosis, within the first 6 months of life.

Systemic perfusion depends on a patent ductus arteriosus (PDA). The closure of the ductus results in rapid, progressive cardiogenic shock and circulatory collapse. Therefore, administration of prostaglandin E1 (PGE1) will maintain a patent ductus arteriosus and should be given. Because 100% oxygen accelerates ductal closure in HLHS, it is acceptable to decrease the FiO₂ to 21% to increase pulmonary vascular resistance and promote systemic blood flow across the PDA. Controlled hypoventilation and positive end-expiratory pressure may also be utilized to augment systemic perfusion by increasing pulmonary vascular resistance. There is no role for diuretics, such as furosemide.

This newborn has a positive hyperoxia test, indicating probable cyanotic congenital heart disease. Transposition of the great arteries is the only cyanotic congenital heart disease listed. Other common causes of cyanotic congenital heart disease include tetralogy of Fallot, tricuspid atresia, total anomalous pulmonary venous return, and pulmonary atresia or stenosis.

A fever is a common concern of parents prompting many to present for medical care. Fevers are an important clinical symptom caused by a change in the thermoregulatory hypothalamic set point for body temperature. It is frequently a symptom of infection but can also be caused by drugs, immunization reactions, CNS dysfunction, malignancy, and chronic inflammatory conditions. Management is to find and treat the cause of the fever. There is no evidence that reducing a fever decreases the morbidity or mortality of an illness, with the possible exception of patients who are critically ill or cannot tolerate the metabolic demands of a fever. This includes patients with an already elevated metabolic state (shock, burns, and post-operative patients), patients with major head trauma, post-cardiac arrest, or those with underlying neurologic or cardiopulmonary disease such as cardiomyopathy (A).

Coarctation of the aorta usually presents as congestive heart failure and cardiogenic shock in the neonatal period due to increased afterload caused by narrowing of the aorta. In mild cases, patients can develop arterial collateral vesselsthat partially bypass the aortic obstruction. These patients usually remain asymptomatic. The diagnosis is often made when incidental hypertension is noted during the evaluation of other problems such as trauma or routine illness. In older children, adolescents, and adults, coarctation of the aorta is best diagnosed clinically by simultaneous palpation of femoral and brachial pulses. Blood pressure in both arms and one leg must be determined; a pressure difference of more than 20 mm Hg in favor of the arms may be considered evidence of coarctation of the aorta. The chest radiograph in these patients may demonstrate rib notching secondary to collateral vessels.

Referring the patient for follow-up (A) is always a good idea but, in this case, would delay her diagnosis of coarctation of the aorta. Many patients exhibit “white coat hypertension,” and a repeat blood pressure is usually normal. However, hypertension in the pediatric age group may have a secondary cause and mandates a thorough evaluation. Renal artery stenosis due to fibromuscular dysplasia (C) is a secondary cause of hypertension seen most commonly in young female patients. Renal ultrasound may show stenosis of the renal artery. However, patients with this condition will have no pulse or blood pressure discrepancies. Patients with hypertension should be started on antihypertensive medications (D) to reduce the future risk of cardiovascular disease. While this patient may require such therapy in the future, her underlying pathology (i.e., coarctation of the aorta) should first be addressed.

A useful bedside test to determine the etiology of a child’s cyanosis (respiratory versus cardiac) is the hyperoxia test. This test is conducted through use of arterial blood gases and 100% oxygen. With application of 100% oxygen an increase in pO₂ of 220 mm Hg suggests lung disease, a pO₂ of 100 to 220 mm Hg requires an evaluation for congenital heart disease, a pO₂100 mm Hg suggests cyanotic congenital heart disease, and a pO₂ 40 to 50 mm Hg would be likely due to transposition of the great arteries (TGA) with poor mixing. If an ABG is not available, the test can also be performed by measuring the oxygen saturation before and after application of 100% oxygen. The oxygen saturation should increase by at least 10% in the presence of a pulmonary process. Therefore, this patient probably has lung disease, such as bronchiolitis. Of note, bronchiolitis may not be associated with cough, rhinorrhea, or wheezing in infants less than 1 month of age.

Based on the hyperoxia test this newborn has cyanotic congenital heart disease, ruling out critical aortic stenosis and coarctation of the aorta. Based on the chest radiograph and ECG, she most likely has tetralogy of Fallot. Boot shaped heart is a buzz word on the boards for ToF and represents RV hypertrophy. This is also hinted at on the ECG. In concert this likely indicates a TofF. TofF is also more common than other cyanotic congenital lesions (represents 10% of congenital heart defects, while transposition of the great arteries is only 5%).

Recall that transposition of the great vessels produced the “egg on a string” type heart on CXR

The radiograph demonstrates rib notching which is characteristic of coarctation of the aorta. Rib notching occurs secondary to development of collateral arteries that form to bypass the coarctation. Rib notching most often involves ribs four to eight but never involves ribs one or two. Most cases of coarctation are diagnosed in the neonatal period. Adolescents and adults usually present with hypertension. Most persons over 20 years with coarctation will display rib notching. Another radiographic finding is the “figure 3 sign” which represents pre-stenotic dilation of the aortic notch and left subclavian.

TAPVR (B) is diagnosed in the neonatal period. These patients classically exhibit a figure 8 or snowman shaped heart on chest radiograph. Transposition of the great arteries (C) is also diagnosed in the neonatal period and is associated with the classic “egg-on-a-string” chest radiograph that describes the narrow mediastinum from superimposed vessels. An isolated ventricular septal defect (D) is not associated with a characteristic chest radiograph. Minor defects typically go undiagnosed, but larger defects can lead to heart failure if not corrected.

Ventricular septal defect (VSD) is most common, responsible for 25% of all congenital heart defects. Most are small and close spontaneously over time. However, larger lesions may persist, eventually leading to pulmonary hypertension and right-sided heart failure. The classic VSD murmur is holosystolic and best heard at the left lower sternal border.

Atrial septal defects (ASD) (A) are rarely recognized in the neonatal period and typically manifest during adolescence, with dyspnea on exertion or, during adulthood with atrial dysrhythmias. An ASD is associated with a fixed split S2 and a loud S1. Coarctation of the aorta (B) involves a narrowing around the ductus arteriosus. Mild cases usually present later in life with hypertension and a chest radiograph that shows rib notching. The ductus arteriosus (C) usually closes by one week of life. Rarely, it will remain open beyond this, but when it does, a continuous machine-like murmur can be heard at the left upper sternal border.

The most common dysrhythmia in children is paroxysmal supraventricular tachycardia (PSVT). It is differentiated from sinus tachycardia by its abrupt onset, rates >230, the absence of normal P waves, or by little rate variation during stressful activities. Symptoms of PSVT in infants include poor feeding, tachypnea, and irritability. Application of ice to the face has been shown to be an effective method of converting a hemodynamically stable child in SVT to NSR. It is important that when performing this maneuver that you do not occlude the nose or mouth, and apply the ice only over the patient’s forehead, eyes, and bridge of the nose for 10–15 seconds. Adenosine, an AV-nodal blocking agent, can also be administered in stable patients.

It will also cause the patient to cry which will increase intra-abdominal and thoracic pressure, temporarily decreasing venous return, and upon release (for a breath, etc.), there will be venous return that will distend the RA and lead to a greater vagal response. This is an added bonus in infants, as ice to the face alone causes a strong vagal response (ask Dr. Berk).

Applying external ocular pressure (A) is contraindicated in children because it can lead to globe rupture. Having a patient blow on an occluded straw may be an acceptable vagal maneuver, but blowing on the patient’s face with a straw (C) has no benefit. Carotid massage (D) is not recommended in infants or children.

The criterion standard for diagnosing urinary tract infections is the isolation of a pathogen from a urine culture obtained via suprapubic aspiration. Although not commonly performed, this is the gold standard technique. Most experts consider any bacterial growth from a suprapubic aspiration sample positive, whereas others require >1000 CFU of a single organism. The only exception is the growth of coagulase-negative staph. such as Staph. epidermidis, which are normal skin flora that may contaminate an aspiration performed with poor technique. In such cases, there would have to be >3000 CFU of S. epidermidis to be considered positive.

Bag collection (A) has a high false positive rate. Urine cultures are considered positive with the identification of >100,000 CFU of a single organism. Suprapubic aspiration (B) of 2,000 CFU of Staph. epidermidis is generally considered contamination because S. epidermidis is normal skin flora. Urethral catheterization (D) is considered positive with >50,000 CFU of a single organism.

A child that presents with a new onset seizure that is unresponsive to multiple doses of conventional medications should alert the physician to the possibility of hyponatremia or other electrolyte abnormality as the cause of the seizure. Hyponatremia can occur in infants who are fed formula that is improperly made resulting in excess free water consumption. Sodium levels < 120 mEq/L can cause seizures and will respond to sodium correction. Typically raising the sodium levels only 3-5 mEq will result in cessation of seizure activity. A seizing patient with hyponatremia should be treated with 3% hypertonic saline 2 mL/kg over 20-30 minutes. The goal is to raise the sodium level above 120 mEq/L acutely and then correct to normal levels slowly over the next 24 hours. 25% dextrose (A) can be given to correct hypoglycemia which may cause a seizure. However, this infant has a normal glucose level. Levetiracetam (C) and midazolam (D) can be given to control seizures but are likely to be ineffective in this child given the likelihood of hyponatremia as the cause of her seizures.

This patient’s history is consistent with tracheoesophageal fistula (TEF). The most common TEF discovered outside of the newborn period is the H-type fistula. A water soluble contrast swallow is the diagnostic test of choice as barium provokes an extensive inflammatory reaction in the lungs and hinders surgical exploration. In combination with decubitus views, the fistula tract will be identified. Tracheoesophageal fistula is the most common cause of esophageal obstruction in neonates. During embryonic development, the trachea and esophagus normally separate and develop in a linear fashion. With a TEF, there is abnormal communication between the trachea and esophagus.

An arterial blood gas (A) would help to determine if an arterial-alveolar gradient exists for shunting problems or issues with diffusion of oxygen, but it would not be helpful in determining this patient’s diagnosis. A chest radiograph (C) can help in diagnosing an associated pneumonia but will not assist in determining the etiology of this condition. Barium (B) provokes an extensive inflammatory reaction in the lungs and hinders surgical exploration.

This child is exhibiting signs and symptoms consistent with breast milk jaundice. Breast milk jaundice is an indirect hyperbilirubinemia in an exclusively breastfed newborn that develops after the first 4 to 7 days of life, persists longer than physiologic jaundice, and has no other identifiable cause. An unusual metabolite of progesterone, a substance in the breast milk that inhibits uridine diphosphoglucuronic acid (UDPGA) glucuronyl transferase frequently causes the indirect hyperbilirubinemia seen in breast milk jaundice. There is also evidence that exclusive breastfeeding delays establishment of the gut flora and results in increased enterohepatic circulation and reabsorption of bilirubin. Management of breast milk jaundice is by continued breastfeeding as this is most often a benign condition. In rare instances, a breastfeeding holiday may be required for bilirubin levels > 20 mg/dL.

ABO incompatibility (A) often presents in the first day of life with severe jaundice, lethargy, and poor feeding. These children require phototherapy for treatment to prevent kernicterus. Breastfeeding failure jaundice (B) is seen in exclusively breastfed infants who are not getting enough breast milk. This leads to decreased stool output and accumulation of bilirubin. Physiologic jaundice (D) is usually seen at 2 or 3 days of life due to decreased conjugation of bilirubin by the immature liver.

This patient is presenting with signs and symptoms consistent with necrotizing enterocolitis (NEC). NEC is defined as bowel necrosis of an unknown etiology. It classically affects premature infants at a much higher rate than those born at term. Signs and symptoms of NEC include abdominal distension, poor feeding, vomiting, and bloody stool. Abdominal wall erythema seen on physical exam is a rare, but pathognomonic finding of NEC. Diagnosis of NEC is with abdominal radiograph, which reveals pneumatosis intestinalis (intramural air within the bowel wall). This radiographic finding is pathognomonic for NEC. Management is first with prolonged bowel rest, nasogastric decompression, and antibiotics.

Intussusception (A) presents with vomiting and colicky severe abdominal pain. A late finding of intussusception is currant jelly stool. Midgut volvulus (B) presents with bilious emesis. Pyloric stenosis (D) presents with projectile nonbilious vomiting after feeding followed by immediate hunger.

This patient had a brief resolved unexplained event (BRUE). Clinicians should use the term BRUE to describe an event occurring in an infant younger than 1 year when the observer reports a sudden, brief, and now resolved episode of ≥ 1 of the following: (1) cyanosis or pallor; (2) absent, decreased, or irregular breathing; (3) marked change in tone (hyper- or hypotonia); and (4) altered level of responsiveness. Moreover, clinicians should diagnose a BRUE only when there is no explanation for a qualifying event after conducting an appropriate history and physical examination. Approximately50% of all cases of BRUE have no apparent cause. Among the other 50% of cases, the 3 most common conditions are gastroesophageal reflux (GER), seizure, and lower respiratory tract infection. These 3 accounts for 50% of all diagnoses eventually made. GER is a normal physiologic process defined by the passage of gastric contents into the esophagus with or without vomiting or regurgitation that occurs in healthy infants, unlike gastroesophageal reflux disease (GERD) in adults, which is a pathological process. The infant in this vignette has a clinical history that suggests a diagnosis of GER that was exacerbated by overfeeding. The best approach is to reduce feeding volumes and increase feeding frequency.

A diagnosis of GER in infants is based upon a history of spitting up. A formal intraesophageal pH monitoring (C) is of no value in routine evaluation. Although seizure is 1 of the most common causes of BRUE, the patient in this vignette has no historical features in the parental report that suggest seizures; therefore, an EEG (B) is not indicated. In addition, an EEG is difficult to obtain in the ED and has low sensitivity for diagnosing chronic epilepsy. Most patients who have epilepsy return with a 2nd episode. Therefore, it has been suggested that EEG be reserved for patients with recurrent BRUE. Older studies had suggested that a mechanism for reflux-induced apnea involves acid stimulation of pharyngeal and esophageal chemoreceptors that lead to laryngospasm. However, recent large studies and additional studies have failed to show correlation between acid reductions with a H₂ blocker or a proton pump inhibitor (PPI) and apneic events. Therefore, lansoprazole (A), a PPI, would not help this patient.

In the neonatal period, the most likely cause of lower gastrointestinal bleeding is swallowed maternal blood, from either delivery or cracked nipples during breast-feeding. The Apt test differentiates fetal from maternal blood. The blood in question is mixed with alkali to detect conversion of oxyhemoglobin to hematin. Fetal hemoglobin is more resistant to denaturation than adult hemoglobin is. If the supernatant stays pink after addition of alkali, the blood is fetal in origin (a positive test). This should not be confused with the Kleihauer-Betke test, used to detect fetal-maternal hemorrhage in the pregnant female.

Necrotizing enterocolitis (C) is a complication of premature infants and presents with abdominal distension, bloody stools, and feeding intolerance. Milk protein allergy (B) should be suspected after introduction of a new formula. Infants who are breastfed are not immune to this condition if Mom consumes cow’s milk in her diet. Since the mother in this vignette is a vegan and is breastfeeding, it is unlikely that a milk protein allergy is responsible for the patient’s guaiac-positive stool. In older infants with painless hematochezia, congenital malformations such as small intestine duplication and Meckel’s diverticulum (A) should be considered. Meckel’s diverticulum is most common at two years of age.

This patient is exhibiting signs and symptoms consistent with severe neonatal jaundice. Jaundice is defined as discoloration of the skin and sclera caused by the accumulation of unconjugated or conjugated bilirubin. Jaundice on the first day of life is frequently caused by blood group system or ABO incompatibility. Severe jaundice with a total bilirubin > 25 mg/dL places this patient at risk for kernicterus, which is defined as neurotoxic hyperbilirubinemia. Kernicterus presents in phases. The first phase, seen on the first few days of life, is characterized by lethargy, hypotonia, and poor feeding. Phase 2 is characterized specifically by hypotonia of the extensor muscles. Phase 3, which is seen with infants over 1 week of age with persistent severe jaundice, is characterized by diffuse severe hypotonia. Chronic bilirubin encephalopathy can lead to persistent hypotonia, hyperreflexia, delayed motor milestones, and variable cognitive deficits. MRI will classically reveal bilateral symmetric high-intensity signals in the globus pallidus in patients with kernicterus.

Gastric reflux disease (A) is not related to jaundice or elevated bilirubin levels. Necrotizing enterocolitis (D) will present with feeding intolerance and abdominal distension instead of jaundice. Abdominal radiographs reveal pneumatosis intestinalis. Muscular dystrophy (C) is a genetic disorder characterized by progressive muscle weakness, most often seen in the proximal lower extremities first. It is not associated with jaundice.

Placental abruption may cause severe anemia and hypovolemia. This situation requires volume expansion and/or blood. Immediate O negative blood transfusion may be lifesaving. When intravenous access cannot be established and vascular access is needed, the umbilical vein is the most accessible site. Using sterile technique, a tie is placed around the base of the umbilical stump to control bleeding, and a scalpel is used to cut the cord 0.5 to 1 cm above the skin. The umbilical vein (always at the 12:00 o’clock position toward the head) is easily distinguished from the two smaller arteries, which are usually constricted. Flush a sterile 3.5F (premature) or 5.0F (term) umbilical venous catheter with normal saline and attach it to a three-way stopcock. The catheter is advanced into the umbilical vein just below the skin level until good blood return is obtained (usually of 2 to 4 cm). When other means of vascular access are not available, the intraosseous route may be attempted, as can a cut-down saphenous line; however the latter use is not common. Placing a line into the soft tissue of the lower back (clysis) was used prior to the advent of intravascular techniques.

The initial steps of newborn resuscitation are position, clear airway (including tracheal suctioning if necessary), dry, remove linen, reposition, administer oxygen as necessary, then simultaneously evaluate the infant’s respiratory effort, heart rate (HR), and color. Spontaneous respiratory effort is adequate if the infant is able to maintain his HR above 100 beats/minute and becomes pink. Following delivery, most infants respond to initial resuscitation by crying, with the heart rate immediately rising, and the patient gradually becoming pink. Some infants, however, require additional brief stimulation and 100% free-flow oxygen. Infants who remain apneic throughout the initial steps or who have gasping respirations require PPV with bag–mask ventilation with 100% oxygen. Ventilate for about 30 seconds, then reassess. In the uncompromised infant, the HR should remain greater than 100 beats/minute. A HR less than 100 needs to be closely monitored and PPV initiated (or continued). If the HR remains less than 60, further intervention with chest compressions, medications, and appropriate diagnostic evaluation is indicated. Therefore, in this patient, PPV should be continued and he should be closely observed. If the patient’s HR becomes less than 60, chest compressions should begin. Intubation may be necessary if the patient remains apneic. Epinephrine is recommended for asystole or bradycardia (HR less than 60) if not responsive to PPV with 100% oxygen and chest compressions for a minimum of 30 seconds.

This patient has acrocyanosis, which is a transient blue discoloration of the hands and feet when a newborn is cold. The perioral region can also be involved; however, it should not involve the lips and mucous membranes as this would be more indicative of true cyanosis. Acrocyanosis is a benign, self-limited condition that must be differentiated from true cyanosis, where the mucous membranes are also blue. Given that the remainder of this patient’s exam is normal, the oxygen saturation by pulse oximetry should be in the normal neonatal range of >94% and should not be associated with arterial desaturation.

Readings of 64%–73% (A), 74%–83% (B), 84%–93% (C) are indicative of hypoxia.

Rapid sequence intubation (RSI) is the preferred and most successful method of intubation in children. Medications used for RSI include an induction agent and a paralytic. The most commonly used induction agents are etomidate, ketamine, and propofol. Etomidate (0.3 mg/kg) preserves hemodynamic stability making it useful in hypotensive trauma patients. Ketamine (1-2 mg/kg) preserves respiratory drive and is a bronchodilator and cardiovascular stimulant making it an ideal drug for intubation in asthma and sepsis. Propofol (1-2 mg/kg) can also be used but may cause hypotension. Succinylcholine and rocuronium are paralytic agents used in RSI. Succinylcholine (1-2 mg/kg) is a depolarizing agent that has a shorter duration than rocuronium, but may cause hyperkalemia and bradycardia. Rocuronium (1 mg/kg) is a nondepolarizing agent that has a longer time to onset and longer duration than succinylcholine. In this asthmatic child, the preferred medications include ketamine and rocuronium.

Laryngeal stimulation associated with intubation can result in reflex bradycardia in children. Pretreatment with atropine (A) was the standard for all rapid sequence intubations, but is no longer recommended. Etomidate and rocuronium (B) could be used for intubation of this child, but the correct dose of etomidate is 0.3 mg/kg. Ketamine and succinylcholine (D) could also be used, however, the correct dose of succinylcholine is 1-2 mg/kg.

Intraosseous (IO) lines can be used as first-line access in a patient in cardiopulmonary arrest and is second-line access (after 90 seconds of peripheral access attempt or 3 attempts) in a critically ill patient, such as the one described in this vignette. IO lines are also reasonable in nonemergent situations when multiple IV access attempts have failed. After the line is placed, signs that confirm accurate placement include aspiration of bone marrow, fluid infuses without extravasation, and needle stands alone without support. However, imaging is not required for placement confirmation, although it can be done with an ultrasound. The IO line may be used immediately to administer fluids and medications as well as draw labs. Several studies have compared lab values obtained from venous blood samples to bone marrow samples from IO lines. Studies have shown poor correlation with white blood cell (WBC) count, platelets, AST, ALT, potassium, and ionized calcium.

Studies have shown good correlation between peripheral venous samples and IO samples for the following: glucose (A), hemoglobin, hematocrit (B), chloride (C), BUN, creatinine, serum drug levels, cultures, pH, PCO2, and blood/Rh type. There are conflicting data regarding the correlation with potassium.

Umbilical vein catheterization is an essential procedure in the resuscitation of the newborn. It is preferred over the umbilical artery in an emergency because cannulating the vein is technically easier. The umbilical vein may remain patent for approximately one week after birth and can be used for exchange transfusions and short-term central venous access.

The umbilical vein is technically easier to cannulate (A) and is therefore the recommended structure for access in an emergent setting. The lone umbilical vein (B) is located in the 12 o’clock position and is thin-walled. There are two thick-walled umbilical arteries (D) located at the 5 o’clock and 7 o’clock positions.

Vascular access (IO or IV) is the preferred method for drug delivery during CPR. But if it is not possible, lipid-soluble drugs such as Lidocaine, Atropine, Naloxone and Epinephrine (mnemonic LANE) can be administered via an endotracheal tube. However, the effects may not be uniform with tracheal as compared with intravenous administration. Optimal endotracheal doses of medications are unknown; in general, expert consensus recommends doubling or tripling the dose of lidocaine, atropine, or naloxone given via the ETT. For epinephrine, a dose 10x the intravenous dose (0.1 mg/kg or 0.1 mL/kg of 1:1000 concentration) is recommended. The effectiveness of endotracheal epinephrine during cardiac arrest is controversial. Some studies have shown it to be as effective as vascular administration; other studies have not found it to be as effective.

Non-lipid-soluble drugs such as adenosine (A), calcium gluconate (B), and sodium bicarbonate (D) may injure the airway and should not be administered via the endotracheal route.

There are important differences between the pediatric and adult airway that need to be taken into consideration when performing intubation. The pediatric epiglottis is longer, narrower, and shaped differently (omega) than the adult epiglottis. This makes direct laryngoscopy more difficult because the angle between the base of the tongue and the glottis opening is more acute. The epiglottis in the infant and young child (up to about eight years of age) is more easily picked up by a straight (e.g., Miller) blade.

The mucosa (B) is more vulnerable in infants, not adults. Minor trauma with the laryngoscope can lead to bleeding, which can worsen visualization of the airway. The narrowest portion of the trachea (C) is below the vocal cords in infants. This is why uncuffed tubes are used in the pediatric population. The risk of mainstem intubation (D) is greater in pediatrics due to the existence of a short trachea and bronchus.

Approximately 10% of newborns require some resuscitative assistance at birth, and approximately 1% require more advanced resuscitative measures. Bradycardia in the newborn (HR <100) usually reflects inadequate ventilation with poor oxygenation and is a major indicator of hypoxia. If there is no meconium present and the infant is bradycardic and apneic, positive-pressure ventilation should be initiated. Initially, this can be achieved with a bag mask, but endotracheal intubation should be considered if ongoing resuscitation is required. The first breaths may require higher pressure to remove lung fluid and to get the chest to rise.

Atropine (A) has no role in neonatal resuscitation. The pediatric heart is regulated by sympathetic, not vagally mediated, tone. Epinephrine (B) and chest compressions (C) should be administered if the HR drops below 60.

Acidosis and alkalosis are processes that lead to acidemia (pH < 7.40) and alkalemia (pH > 7.40). Primary metabolic disorders result from a change in bicarbonate, while primary respiratory disorders result from a change in partial pressure of carbon dioxide. Compensation occurs when other system alterations bring the blood gas toward a normal pH of 7.35. A metabolic acidosis is present in any patient with a pH of < 7.35 and bicarbonate < 24. Causes of an increased anion gap acidosis [Na+ – (Cl- + HCO3-)] > 12 can be remembered by the MUDPILES mnemonic (Methanol, Metformin, Uremia, Diabetic (or alcoholic) ketoacidosis, Paraldehyde, Propylene glycol, Isoniazid, Iron, Lactic acidosis, Ethylene glycol, Salicylates). Normal anion gap acidosis is caused by: renal losses (tubular acidosis, acetazolamide), GI losses (diarrhea, malabsorption), and adrenal insufficiency. Compensation for an acid-base disorder never completely normalizes the pH. A pH of 7.45 in a patient with low bicarbonate indicates a second disorder (such as a primary respiratory alkalosis).

Metabolic alkalosis (B) is caused by an increase in bicarbonate leading to a pH > 7.35. This occurs secondary to gastric acid loss from vomiting or NG tube suctioning, diuretic use, and adrenocortical hormone excess. Respiratory acidosis (C) is caused by an increase in the partial pressure of carbon dioxide > 40 leading to a pH < 7.35. This is primarily a result of inadequate ventilation or increased dead space. Causes include head or chest trauma, oversedation, obtundation, neuromuscular disorders, Pickwickian syndrome (obesity-hypoventilation syndrome), and COPD. Respiratory alkalosis (D) is caused by a decrease in the partial pressure of carbon dioxide < 40 leading to a pH > 7.35. In this condition, carbon dioxide ventilation outpaces production.

The patient has a likely pseudo-obstruction (ogilvie’s syndrome). While we would want to get CT imaging to rule out mechanical obstruction, a pseudo-obstruction can occur in the elderly/nursing home patients. It’s cause is unknown, but is due to gut motility dysfunction.  Dehydration and associated hypokalemia are common. Hypokalemia can cause weakness of muscles, and when low can lend to gut motility dysfunction. Treatment measures include IV hydration, potassium repletion, nasogastric tube placement, and surgical consultation.

Hypercalcemia (A) can occur with malignancy, hyperparathyroidism, drugs, immobilization, Paget’s disease, or excessive intake (vitamin D toxicity, milk-alkali syndrome). Major clinical findings can be remembered with the phrase “stones, bones, groans, and psychiatric overtones” (nephrolithiasis, weakness, abdominal pain/constipation, confusion/depression). Hypercalcemia shortens the QT interval. Hypocalcemia (B) can be seen in renal failure, hypoparathyroidism, pancreatitis, or chronic malabsorption syndromes. Neurologic symptoms include paraesthesias, carpopedal spasm, Chvostek’s or Trousseau’s sign, and hyperreflexia. Cardiovascular signs include hypotension, heart failure, dysrhythmias, and prolonged QT interval. Hyperkalemia is more commonly seen in renal failure patients, and would be expected to primarily cause cardiac arrhythmias.

Hyponatremia is defined as sodium less than 135 mEq/L. Hyponatremia can occur in a hypovolemic, euvolemic, or hypervolemic state. Hypervolemic hypo-osmolar hyponatremia is is associated with fluid overload. The etiology is usually from a perceived low intravascular volume by the kidneys and active water reabsorbtion in excess to sodium retention. If urine sodium is low (<20) causes include liver failure, cirrhosis, hepatorenal syndrome, nephrotic syndrome, and CHF. If urine sodium is high (>20) causes include acute or chronic renal failure, such as that caused by hypertensive nephropathy. Treatment of hypervolemic hypo-osmolar hyponatremia is dialysis.

Cirrhosis (A) and congestive heart failure (B) is often the cause of hypervolemic hypo-osmolar hyponatremia when the urine sodium is low (<20). SIADH (D) results in euvolemic hyponatremia with urine osmolality greater than serum osmolality. The excess ADH causes total body water to increase thereby diluting total body sodium. Despite the increased total body water, these patients typically do not show evidence of edema or heart failure as the increased water is intracellular not intravascular.

This ECG is indicative of hyperkalemia, one of the most lethal complications of chronic kidney disease encountered in the ED. A potassium level of 6 mEq/L should be considered potentially dangerous, even though many patients with ESRD chronically tolerate serum levels above this and do not manifest ECG changes. The most rapid treatment for hyperkalemia is intravenous calcium (gluconate with peripheral access, chloride with central access), which transiently reverses cardiac effects of hyperkalemia by antagonism of potassium at the cardiac membrane. Calcium is indicated in all patients with suspected hyperkalemia who have widening of the QRS, an unstable dysrhythmia, bradycardia, or hypotension.

Cardiology consultation (B) is not needed; the patient’s ECG findings are due to an underlying electrolyte abnormality, not a primary cardiac condition. Defibrillation (C) may be necessary if the rhythm deteriorates to ventricular fibrillation. However, should that occur, the definitive management remains removal of potassium from the serum. Transcutaneous pacing (D) can be used as a temporizing measure in patients with symptomatic bradycardia. Hyperkalemic bradycardia responds poorly to pacing. The primary treatment is cardiac membrane stabilization with calcium and subsequent lowering of the serum potassium.

Hypokalemia frequently occurs concomitantly in patients with hypomagnesemia. Because magnesium is required for the normal functioning of the Na+/ K+ ATPase pump, as well as K+ reabsorption channels within the nephrons, hypomagnesemia can result in refractory hypokalemia that is not correctable by the administration of potassium alone. For potassium levels to increase, magnesium must also be administered.

Hypermagnesemia (A) enhances potassium retention. Hypernatremia (B) and hyponatremia (D) will not interfere with increasing the level of potassium in patients receiving potassium supplementation for hypokalemia.

D. Correct. Hyperventilation causes a respiratory alkalosis. This in turn increases serum pH and therefore enhances protein binding of calcium in serum. The end result is decreased free ionized calcium. This induced hypocalcemia causes muscle spasm and in severe cases may lead to a tetany like state

A. Incorrect. hyperventilation causes the opposite of the proposed mechanism

B. Incorrect. Increased PaO2 causes neuronal activation
Increased PaO2 is not the cause of carpal spasm

C. Incorrect. Increased serum pH causes vasodilation of capillary beds
Hyperventilation would not increase serum pH and is not the cause of carpal spasm

The syndrome of inappropriate secretion of ADH (SIADH) is defined by the secretion of ADH in the absence of an appropriate physiologic stimulus. Its hallmark is an inappropriately concentrated urine, despite the presence of a low serum osmolality and a normal circulating blood volume. Causes of SIADH include central nervous system disorders, pulmonary disease, drugs, stress, pain, and surgery. Therefore, the above patient, with a known history of lung cancer and hyponatremia, most likely has SIADH and exhibits the following lab findings: serum osmolarity low, urine osmolarity high, urine sodium high.

Psychogenic polydipsia (D) is a rare cause of euvolemic hyponatremia and is seen in psychiatric patients who consume large amounts of free water (in excess of 1 L/hr). This large consumption overwhelms the kidney’s ability to excrete free water. Patients will exhibit serum osmolarity low, urine osmolarity low, urine sodium low. Diabetes insipidus (B) results in the loss of large amounts of dilute urine from the loss of concentrating ability in the distal nephron. This may be due to a central cause—such as the lack of ADH secretion from the pituitary—or a nephrogenic cause—such as the lack of responsiveness to circulating ADH. Laboratory workup that invariably shows serum osmolarity high, urine osmolarity high, urine sodium low (A) rarely occurs.

The patient is in an acute metabolic acidosis as is evident by the pH and low bicarbonate. A quick calculation shows that the anion gap is 26 and that the respiratory response with a drop in PaCO2 to 22mmHg follows the “rule of 15’s” with the HCO3 of 8, displaying a proper hyperventilatory response to an acute metabolic acidosis and no evident mixed acid/base disorder. Anion gap is calculated by Na -HCO3 – Cl. For the rule of 15’s, HCO3 + 15 should = the pCO2 and the last 2 digits of the pH. In this patient, 8+15 = 23. This is the correct CO2 indicating an appropriate response. It is also the last 2 digits of the pH (7.23). Thus this is not a mixed acid base disorder but rather an acidosis with appropriate compensation. There are no ketones in the patient’s urine, and the lactate is elevated, thus this patient is in an acute lactic acidosis secondary to metformin rather than DKA.

A. Incorrect. Acute anion gap diabetic ketoacidosis
There are no ketones in the patient’s urine to suggest diabetic ketoacidosis.

C. Incorrect. Acute non-anion gap metabolic acidosis
This patient has an anion gap of 26 which is elevated and consistent with an anion gap acidosis. Recall anion gap is calculated by Na -HCO3 – Cl.

D. Incorrect. Mixed acid/base disorder
This patient has an appropriate compensatory response. Recall the rule of 15’s – HCO3 + 15 should = the pCO2 and the last 2 digits of the pH. In this patient, 8+15 = 23. This is the correct CO2 indicating an appropriate response. It is also the last 2 digits of the pH (7.23). Thus this is not a mixed acid base disorder but rather an acidosis with appropriate compensation. In addition if you apply the delta gap rule (AG-12/24-HCO3) you find that there is no underlying metabolic alkalosis. Recall that this rule tries to determine any underlying metabolic acidosis or alkalosis in an AGMA. if the gap <1: probable additional underlying metabolic acidosis, if it =1: pure AGMA, if it >1: additional underlying metabolic alkalosis

This patient presents with diabetic ketoacidosis as indicated by the laboratory findings of hyperglycemia, increased anion gap, acidemia, and low bicarbonate concentration. However, there is also a secondary primary metabolic acidosis present, likely caused by the patient’s diarrhea. The patient’s anion gap is increased by 8 mmol/L above the upper limit of normal of 12 mmol/L. In an isolated metabolic acidosis, the increase in anion gap correlates with the decrease in bicarbonate concentration (normal level is 24 mEq/L), and this difference is referred to as “delta gap” with normal values of 0 +/- 6. Thus, in this patient, the expected bicarbonate level would be 16 mEq/L +/- 6. However, the measured bicarbonate level is much lower than expected at 6 mEq/L. This indicates that a second non-anion gap metabolic acidosis is present. Diarrhea is a common cause of hyperchloremic (non-anion gap) metabolic acidosis.

This is a classic presentation of acute iron toxicity in a child. Iron exposure is one of the leading causes of poisoning deaths in children younger than six years. Iron is usually not considered poisonous and is often stored unsafely in many homes. It is easily accessible in prenatal vitamins and is often packaged as an appealing red sugar-coated tablet. Iron toxicity is associated with an anion-gap metabolic acidosis resulting primarily from increased lactate. Classic teaching describes five clinical stages of iron toxicity based on the pathophysiology of iron poisoning. Although conceptually important, they are of limited clinical benefit in managing the poisoned patient. Therefore, it is more clinically relevant to think about poisoning in two clinical stages. Nausea, vomiting, abdominal pain, and diarrhea characterize clinical stage one. The absence of symptoms, particularly vomiting, in the first six hours postingestion, excludes significant iron toxicity. Systemic toxicity is seen in clinical stage two, characterized by hepatotoxicity, coagulopathy, myocardial dysfunction, and neurologic symptoms, including seizures and altered mental status. Management is mainly supportive. Activated charcoal does not bind iron. Whole-bowel irrigation should be considered in a large ingestion. Intravenous deferoxamine is a chelating agent that should be considered in patients with severe iron toxicity. The PCO₂ should be decreased (A) as part of respiratory compensation, not increased. The patient should be acidemic, not have a normal pH (C) or alkalemic pH (D)

Bupropion can induce seizures even at therapeutic levels. Other adverse effects include tachycardia, tremulousness, hallucinations, and QRS prolongation. Cyclic antidepressants (amitriptyline), SSRIs (fluoxetine), and monoamine oxidase inhibitors (phenelzine) can also cause seizures but less frequently than bupropion and usually with other symptoms of serious intoxication, such as central nervous system depression or QRS prolongation. Delayed seizures are seen with Bupropion overdose especially the extended release formulation.

Abstract about seizure with bupropion.

LITFL Bupropion 

This patient has significant valproic acid (VPA) toxicity, as evidenced by altered mental status, elevated VPA level, and hyperammonemia. Valproic acid is used to treat seizures, migraines, and mood disorders. The range of therapeutic concentration for VPA is 50–100 mg/L. In the setting of overdose, CNS depression, encephalopathy, metabolic acidosis, hepatoxicity, pancreatitis, renal failure, and pancytopenia may occur. Hyperammonemia is caused by depletion of L-carnitine and acetyl CoA, which inhibits the urea cycle. L-carnitine administration can improve these metabolic abnormalities and is the treatment of choice for severe VPA overdose. Serial VPA and ammonia concentrations should be obtained.

Activated charcoal (A) binds VPA effectively, and multiple dose-activated charcoal can be of utility due to enterohepatic recirculation; however, the patient in this scenario ingested valproic acid 24 hours prior to presentation, thus limiting the potential benefit of activated charcoal. Additionally, the patient is lethargic and vomiting, so charcoal administration is potentially harmful in the setting of an unprotected airway. Hemodialysis (B) and hemoperfusion can be of utility for very large VPA overdoses with significant acidosis and renal failure. The patient in this scenario has normal renal function and normal pH. Although VPA is an acid, urinary alkalinization (D) does not increase urinary excretion and is not indicated. Whole-bowel irrigation with polyethylene glycol electrolyte solution (E) can be of utility if a delayed or extended-release product is ingested. Once again, the utility of this therapy is limited 24 hours postingestion and requires a secure airway.

Indications for Multi-Dose Activated Charcoal

Neuroleptic malignant syndrome (NMS) a life-threatening condition characterized by muscle rigidity, autonomic instability, altered mental status, and hyperthermia that occurs soon after initiation or dose adjustment of adopaminergic or antipsychotic drug. This patient was likely started on an antipsychotic mediation when he was diagnosed with schizophrenia. Other risk factors for the development of NMS include high dosage, high-potency antipsychotic medications, parenteral formulation, dehydration, preceding psychomotor agitation, and previous episodes of NMS. The diagnosis is made when a patient develops severe muscle rigidity and hyperthermia while taking a neuroleptic or antipsychotic medication and develops two or more of the following symptoms: diaphoresis, dysphagia, tremor, incontinence, altered mental status, tachycardia, mutism, hypertension or labile blood pressure, rhabdomyolysis, leukocytosis and symptoms are not caused by another substance, neurologic, medical or psychological disorder.

A) The patient in the question stem is suffering from cocaine induced chest pain. The EKG demonstrates left ventricular hypertrophy, R wave in aVL >11mm, with strain pattern in I and aVL. Given the active chest pain, in the absence of ST-segment elevation, immediate treatment involves supportive care and serial troponin/ECGs.

B) There is no ST-segment elevation to indicate thrombolytics

C) There is no ST-segment elevation to indicate percutaneous coronary intervention

D) There is no evidence the patient is suffering from an aortic dissection and would thus not require further imaging.

.Phenytoin has delayed and erratic gastrointestinal absorption; therefore, multiple-dose activated charcoal can be of utility for phenytoin overdose. Phenytoin has also been known to form concretions in the intestinal tract. In fact, phenytoin levels may continue to rise for days after ingestion. Phenytoin is bound by activated charcoal; charcoal should be administered to patients with a secure airway. Multiple-dose activated charcoal can be administered every two to four hours (provided the patient has active bowel sounds) to minimize drug absorption and decrease the half-life of phenytoin. The most common manifestations of phenytoin toxicity include nystagmus, somnolence, dizziness, gastrointestinal disturbances, and headache.

Cardiac toxicity (A) occurs almost exclusively with intravenous preparations of phenytoin. Even though phenytoin is a sodium channel blocker with Vaughan-Williams class 1B antiarrhythmic effects, cardiotoxic manifestations are largely attributed to diluents (i.e., propylene glycol and ethanol) used in the IV formulation. Clinical manifestations include hypotension and (somewhat paradoxically) dysrhythmias, which can lead to cardiovascular collapse and death. These effects are rarely observed with the oral formulation due to the lack of diluent. Phenytoin is highly protein bound. Because dialysis (B) can remove only free, unbound phenytoin, it is of limited utility in the setting of an overdose. Significant cardiovascular toxicity generally occurs at infusion rates (D) of greater than 50 mg/min (not 25 mg/min); therefore, higher infusion rates are not recommended. Cardiovascular toxicity from phenytoin appears to be a dose- and rate-related phenomenon.

Note, phenytoin may not reflect free drug concentrations. This is important because phenytoin, in general, is highly protein bound, and only free drug exerts pharmacologic effects. Free phenytoin concentrations can be elevated in states of low albumin (even with a normal total phenytoin level). In these cases, free levels should be monitored. Patients at risk for having decreased protein-binding capacity include the elderly, neonates, patients with liver disease or uremia, or patients taking medications that would displace phenytoin from albumin. The normal range for total phenytoin levels is 10–20 mg/L, whereas free concentrations should be between 1.0 and 2.1 mg/L.

.The boy has signs and symptoms consistent with opioid ingestion. Methadone ingestion can manifest with the classic opioid toxidrome of respiratory depression, sedation, and miosis. Signs of more severe toxicity can include bradycardia, hypotension, and hypothermia. Methadone has been associated with a prolonged QTc interval and risk of torsades de pointes. Patients with significant respiratory or CNS depression should be treated with naloxone, which is a mu receptor antagonist. Because the half-life of methadone is longer than naloxone, patients can require multiple doses of naloxone. Also, serial ECGs are needed to monitor for the development of a prolonged QTc interval. If a patient does develop a prolonged QTc, management includes close cardiac monitoring, repletion of electrolytes, and having magnesium readily available should the patient develop torsades de pointes.

Clonidine (A) toxicity manifests as lethargy, miosis, and bradycardia. Although, findings may be similar to opioid overdose, QTc interval prolongation and torsades de pointes are typically only seen with opioid overdose. Propanolol (C) toxicity causes bradycardia and hypotension that typically develops within six hours of ingestion. Heart block and hypoglycemia may also be seen. Clinical manifestations of salicylate (D) toxicity include nausea, vomiting, diaphoresis, and tinnitus. Moderate cases can manifest as tachypnea, tachycardia, and altered mental status.

This patient presents with Wernicke syndrome, a clinical diagnosis that can be corroborated by improvement of symptoms after thiamine administration. Wernicke’s encephalopathy is a medical emergency with a high mortality rate (10-20%). Unfortunately, it often goes unrecognized. Diagnosis requires two of the following criteria: 1) dietary deficiency, 2) oculomotor abnormalities, 3) cerebellar dysfunction and 4) altered mental status or memory impairment. Oculomotor findings include nystagmus and ophthalmoplegia. Cerebellar signs typically manifest as ataxia. Patients with chronic malnourishment including alcoholics, patients with eating disorders and patients with advanced cancer are at risk for Wernicke’s. Emergent treatment consists of thiamine administration. Ophthalmoplegia and nystagmus may resolve in hours and confirms diagnosis.

.Flumazenil remains a controversial antidote, but it can be used safely both diagnostically and therapeutically in a very select group of patients. Contraindications to the use of flumazenil include the coingestion of a proconvulsant drug (such as bupropion) and a history of convulsions. Bupropion, both therapeutically and in overdose, can cause convulsions. In the setting of an ingestion of both a benzodiazepine and a proconvulsant drug, flumazenil can reverse the protective effect of the benzodiazepine on seizure prevention and is thus contraindicated. In the setting of an overdose of unknown medications, the presence of findings inconsistent with a pure benzodiazepine overdose or suggestive of a proconvulsant coingestant (such as convulsions, mydriasis, tachycardia, and QRS width prolongation) contraindicates flumazenil administration.

.Alcoholic ketoacidosis typically occurs in undernourished alcoholics who have recently binged on alcohol and have had limited food intake. Abdominal pain and vomiting are common. These symptoms might be related to the condition, but a thorough evaluation for other etiologies such as pancreatitis is necessary. These patients characteristically have a normal mental status despite the presence of potentially severe acidosis, and this helps distinguish alcoholic ketoacidosis from poisoning with a toxic alcohol. The acidosis in alcoholic ketoacidosis predominantly results from the presence of beta-hydroxybutyrate (a ketoacid that is not typically detected as a ketone on urinalysis). As with most acidosis, tachypnea is expected. Treatment of alcoholic ketoacidosis involves hydration with glucose-containing solutions, thiamine, food intake, and treatment of any other underlying medical conditions.

.Opioid withdrawal is characterized by a constellation of clinical manifestations that can include abdominal pain, anxiety, diaphoresis, diarrhea, irritability, myalgias, mydriasis, piloerection (involuntary erection of the skin hairs “gooseflesh’), rhinorrhea, vomiting, and yawning. Although very uncomfortable, opioid withdrawal is not life-threatening nor is it characterized by altered level of consciousness.

Hyperthyroidism refers to disorders that result from overproduction of hormone from the thyroid gland. Thyrotoxicosis refers to any cause of excessive thyroid hormone concentration. Thyroid storm represents the extreme manifestation of thyrotoxicosis. This patient has thyroid storm, a condition predominantly seen in patients with Grave’s disease. There are many precipitants of thyroid storm, but infection and sepsis are the most common. Clinically, patients often present with fever, diaphoresis, tachycardia, altered mental status, restlessness, agitation, abdominal pain, and vomiting. The presentation mimics many other hyperadrenergic states such as cocaine intoxication or ethanol withdrawal. It can also be confused with heatstroke and neuroleptic malignant syndrome. The prompt recognition and treatment of thyroid storm is crucial for patient survival. If untreated, the condition is uniformly fatal. Propranolol is the first-line treatment for patients with symptomatic thyrotoxicosis or thyroid storm because it blocks peripheral hyperadrenergic activity in addition to the conversion of T4 to T3. This should be immediately followed by administraiton of propylthiouracil or methimazole to futher block the conversion of T4 to T3. Iodine (after PTU administration), corticosteroids, fluid resuscitation, rapid cooling, and treatment of precipitating illness follows.

After administration of propranolol, management of thyroid storm should proceed with the initiation of propylthiouracil to inhibit the production of thyroid hormone. Once the primary measures are taken, and there is concern that the inciting event may be due to sepsis, then vanc/cefepime should be administered. Inorganic iodine (potassium iodide) blocks the release of thyroid hormone that is still stored in the thyroid gland, but its administration should be delayed at least 60 min after propylthiouracil is administered. It is important to first inhibit the synthesis of thyroid hormone; otherwise, the administered iodine will be incorporated into new hormone. While Methimazole or PTU should be administered expediently, controlling the sympathetic hyperactivity with propanolol is the first priority.

The appropriate dose of intravenous dextrose for a 2-year old child with hypoglycemia is 24 mL of 25% dextrose. To find the correct dose, you must perform a two-step calculation.

1. determine concentration of dextrose. In general think in terms of infants, pediatric pt, adult-ish patient. Formally though, <1 o: D10, 1-8 yo: D25, >8 yo: D50
2. Determine the dose

An easy way to remember the dosing is by the “50 rule” in which you divide 50 by the concentration you intend to use (50, 25, or 10) and you are given the dose you should administer:

Adult (D50): 50/50 = 1 mL/kg

1-8 yrs (D25): 50/25 = 2 mL/kg

< 1 yr (D10): 50/10 = 5 mL/kg

A dose of 12 mL of 50% dextrose (B) is incorrect because a 2-year old child should not be given 50% dextrose as it is a highly concentrated, hypertonic solution. A dose of 12 mL of 25% dextrose (A) is too low of a dose. Although you can give a 2-year old a 10% dextrose solution, 24 mL (C) it is too low of a dose.

Note that in the ED we often don’t have rapid access to D25 or D10. We can rapidly make these solutions though. Take a D50 push (50 cc), and dilute it by 50% with NS (i.e. waste half the syringe and replace it with 25 CC NS), this will give you D25. Then take that syringe and dilute it by 50% again with NS, this will give you D12.5 which is reasonably close to D10.

The patient has hypocalcemia. Calcium is critical to normal cell functioning, neural transmission, membrane stability, bone structure, blood coagulation, and intracellular signaling. Calcium levels are tightly regulated by parathyroid hormone, which induces the bones and kidneys to increase serum calcium levels. Hypocalcemia occurs in 1 to 2% of patients after total thyroidectomy and is due to injury or destruction of the parathyroid glands during surgery. Other causes of hypocalcemia include hypoparathyroidism, vitamin D deficiency, chronic renal failure, hyperphosphatemia, hypomagnesemia, and severe pancreatitis. Because calcium is bound to albumin in the blood, hypoalbuminemia will cause a fall in measured serum calcium. However the active form of calcium, ionized calcium, is not affected by changes in albumin. Hypocalcemia causes primarily neuromuscular and cardiovascular manifestations. Symptoms of mild hypocalcemia include muscle cramping and perioral or finger paresthesias. Severe hypocalcemia can lead to congestive heart failure, dysrhythmias, hypotension, and cardiovascular collapse. Chvostek’s sign is seen in hypocalcemia. Chvostek’s sign refers to twitching of the facial or eye muscles elicited by tapping on the facial nerve. Trousseau’s sign may also be present. In Trousseau’s sign, a blood pressure cuff is inflated to 20 mm Hg above systolic blood pressure for 3 minutes, and a positive sign is carpal spasms due to local ulnar and median nerve ischemia.

This patient presents with signs and symptoms consistent with hyperglycemic hyperosmolar state (HHS) and intravenous fluids should be given aggressively early in management. HHS is a syndrome characterized by dehydration, hyperglycemia, hyperosmolarity and altered mental status. Patients may present with confusion, lethargy, seizures, focal neurologic deficits or frank coma. Pathophysiologically, decreased insulin (or insulin action) leads to gluconeogenesis and increased circulating glucose levels. This in turn draws fluid from the intracellular space into the intravascular space. The resultant osmotic diuresis leads to profound intravascular dehydration, electrolyte abnormalities and hyperosmolarity. Typically, patients will have a blood glucose >600 mg/dl and an osmolarity >320 mOsm/L. Blood urea nitrogen and creatinine are usually elevated. Initial management focuses on supportive care and aggressive fluid resuscitation. Patients with HHS are estimated to be about 8 liters hypovolemic. In addition to fluid administration, electrolyte repletion is paramount.

HCT is used in the diagnosis of ischemic stroke. Although HHS can mimic a stroke, this is not the most important intervention at this time. Unlike in diabetic ketoacidosis, patients with HHS have preserved insulin secretion. While a continuous intravenous insulin infusion and even bolus therapy may be used to help lower the blood glucose level in a controlled manner, intravenous fluids are of greater importance in the initial management of the patient. For the board exam, fluid management for HHS ALWAYS precedes insulin administration.

This patient has evidence of diabetic ketoacidosis (DKA) on his laboratory evaluation. In DKA, patients become hyperglycemic, dehydrated and acidemic with multiple electrolyte abnormalities. The syndrome results from a deficiency of insulin as well as excess of glucagon. Excess serum glucose overwhelms the renal tubules leading to glucosuria and an osmotic diuresis. With glucose in the renal tubules, electrolytes (sodium, potassium, magnesium, calcium and phosphorus) and water are pulled into the tubules and excreted. With the absence of glucose available for metabolism (either due to insulin deficiency or resistance), the body begins to metabolize adipose tissue using fatty acid oxidation. This leads to the production of free fatty acids which are converted to ketones (acetoacetate and beta-hydroxybutyrate) by the liver. Ketosis leads to acidosis and may cause abdominal pain, vomiting and a characteristic fruity odor on the breath. Treatment includes aggressive IV hydration to correct severe dehydration. Patients with DKA need continuous insulin in order to reverse fatty acid metabolism. Insulin is administered as a continuous infusion of 0.1-0.14 units/kg/hour IV up until max of 10 u/hr until the anion gap and ketosis resolve. The ADA recommends 0.14 u/kg/hr while the Joint British Diabetes Societies Inpatient Care Group recommends 0.1 u/kg/hr. while controversial, some may give an initial bolus of 0.1 u/kg IV Insulin followed by infusion of 0.1 u/kg/hr. While this can lead to increased incidence of hypoglycemia/hypokalemia, it is currently controversial whether this actually causes harm. For all purposes and benefits for the test, generally starting with an infusion without a bolus is recommended.

An insulin IV bolus (B) is no longer recommended as first line with concerns for rapid shifts of glucose/potassium, though this is controversial. Regardless, 1.0 u/kg will be far too high of a starting dose. Potassium chloride 20 mEq IV prior to initiation of insulin infusion (C) would be indicated in patients with an initial potassium less than 3.3 mEq/L. Patients with an initial potassium of 3.3 – 5.2 mEq/L can be started on insulin and should also be given potassium with each liter of fluid. Patients with DKA are total body potassium depleted although their serum values are often normal on initial measurement. This results from shifting of potassium ions due to acidemia. As the pH normalizes, potassium values will drop and require repletion. A bolus of sodium bicarbonate 50 mEq IV (D) is not recommended in the treatment of DKA, even in severely acidotic patients as overly aggressive correction of acidemia may worsen outcomes.

The neurologic deficits associated with most toxic and metabolic causes of significant altered levels of consciousness (including hypoglycemia) are typically symmetrical (nonfocal). However, a small but not insignificant number of patients with hypoglycemia presents with focal neurologic deficits, including hemiplegia. The rapid identification and correction of hypoglycemia in all patients, including those with focal neurologic deficits, is critical to avoid severe complications of a rapidly reversible condition and to avoid unnecessary imaging (brain imaging) and potential interventions (thrombolytic therapy).

Although adrenergic symptoms such as diaphoresis and tachycardia are common manifestations of hypoglycemia, bradycardia is less common, and none of these symptoms should be relied on to determine if a patient is hypoglycemic.

Release of the counterregulatory hormone epinephrine in the setting of hypoglycemia can result in a variety of adrenergic symptoms (anxiety, diaphoresis, palpitations, tachycardia, tremors). Hypotension is not expected and is not common.

Syncope is a transient loss of consciousness associated with a loss of postural tone that spontaneously reverses without medical intervention. When a patient becomes comatose from hypoglycemia, the body has exhausted its ability to counteract the hypoglycemia. Spontaneous reversal is very unlikely to occur. Syncope is not a common manifestation of hypoglycemia.

Destruction of the adrenal cortex in primary adrenal insufficiency (Addison disease) manifests with signs and symptoms of steroid deficiency (mineralocorticoids, glucocorticoids, gonadocorticoids) and increased adrenocorticotropic hormone (ACTH). The mineralocorticoid aldosterone stimulates the kidneys to reabsorb sodium and to excrete potassium. Aldosterone deficiency contributes to the presence of hyponatremia (seen 90% of the time), and hyperkalemia (seen 60% of the time). Deficiency in the glucocorticoid cortisol can lead to hypoglycemia, a common finding in children and infants. Signs and symptoms of gonadocorticoid deficiency are more common in women and include decreased axillary and pubic hair and decreased libido. Increased ACTH leads to skin hyperpigmentation. Causes of primary adrenal insufficiency include autoimmune disease (most common cause in Western countries), congenital conditions, drugs, hemorrhage, infections (tuberculosis is traditionally a common cause but is uncommon now in Western countries), infiltrative diseases (such as amyloidosis and sarcoidosis), and metastatic cancer.

Cortisol is involved with maintaining euglycemia. Cortisol deficiency in primary adrenal insufficiency leads to hypoglycemia, not hyperglycemia.

Hypercalcemia, not hypocalcemia, is seen in primary adrenal insufficiency. The hypercalcemia is thought to be a result of increased mobilization from bone and diminished renal excretion. It generally corrects quickly with hydration.

Aldosterone causes sodium resorption and potassium excretion. Aldosterone deficiency in primary adrenal insufficiency causes hyperkalemia, not hypokalemia.

Metanephrine is an intermediate metabolite between epinephrine and vanillylmandelic acid. It is considered the most sensitive and specific test for identifying the presence of pheochromocytoma. Pheochromocytomas are catecholamine-producing adrenal tumors that can lead to hypertensive crises that may be lethal. The average age at diagnosis is approximately 40 years. The classic “rule of 10s” for pheochromocytomas is that approximately 10% are bilateral, 10% are extra-adrenal, and 10% are malignant. These tumors may arise sporadically or as a feature of an inherited condition such as multiple endocrine neoplasia, neurofibromatosis type 1 or von Hippel-Lindau disease. Typical clinical presentations include paroxysms of headaches, palpitations and profuse sweating: the classic triad of pheochromocytomas. Paroxysms usually last less than one hour and are brought about by exercise, position changes, surgery, urination or certain medications (e.g., metoclopramide, tricyclic antidepressants). Patients may present in a hypertensive crisis with acute pulmonary edema, intracranial hemorrhages, heart failure or dysrhythmias. Other clinical features may include anxiety or panic attacks, nausea, flushing, abdominal or back pain, pallor or generalized weakness. The first step in diagnosis is measuring biochemical metabolites called metanephrines, the methylated products of catecholamine catabolism. Diagnostic imaging for tumor localization is a secondary diagnostic method. Treatment includes total or partial adrenalectomy. Preoperative blood pressure management with oral alpha-adrenergic antagonists (e.g., phenoxybenzamine or prazosin) or beta-blockers (e.g., propranolol) can be used to keep blood pressure consistently less than 160/90 mm Hg.

5-HIAA (A), or 5-hydroxyindoleacetic acid, is a serotonin metabolite used in the diagnosis of carcinoid tumors. Serotonin (C) levels are not utilized for diagnosing neuroendocrine tumors, whereas serotonin metabolites (e.g., 5-HIAA) are. Vanillylmandelic acid (D) is the least specific catecholamine metabolite in testing for pheochromocytoma. It has greater utility in the initial diagnosis and surveillance testing for neuroblastomas.

Confusion, confabulation, ataxia, ophthalmoplegia, and nystagmus suggest Wernicke-Korsakoff encephalopathy. Although Wernicke syndrome and Korsakoff syndrome are clinically distinct, they are both caused by thiamine deficiency. Wernicke encephalopathy is often underdiagnosed and carries a mortality rate of 10% to 20%. It is diagnosed clinically with two of the following findings: oculomotor abnormalities (most commonly nystagmus), dietary deficiencies, cerebellar dysfunction, and altered mental status (e.g., lethargy, inattentiveness) or memory impairment. Korsakoff psychosis, also referred to as alcohol-induced persisting amnestic disorder, is an untreatable form of dementia that involves recent retrograde amnesia, anterograde amnesia (inability to learn new information), apathy, and confabulation. Confabulation is not essential to the diagnosis. Treatment is with thiamine followed by dextrose administration, along with an adequate diet and abstinence of alcohol. Ocular manifestations usually respond well to thiamine within days, but ataxia and mental changes usually do not improve as rapidly and portend a worse prognosis.

Dextrose (A) should be given after thiamine for the theoretical prevention of Wernicke-Korsakoff syndrome. Older literature suggests that prolonged treatment with hypertonic IV glucose “precipitated” Wernicke encephalopathy, but this did not occur with single or repeated bolus of IV glucose. Haldol is useful in agitated patients or those with psychosis, but is not required at this time as patient is neither agitated and there are no definitive signs of psychosis. Pyridoxine (C), or vitamin B6, is not the cause of Wernicke-Korsakoff syndrome and has no role in its prevention or treatment.

The patient is likely suffering from myxedema coma based upon her clinical features. Myxedema coma is a rare life-threatening expression of hypothyroidism. It may be precipitated by infection, cold exposure, medications, trauma, or myocardial infarction. Classically it occurs in the winter months in elderly women who are undiagnosed or undertreated. Patients present with hypothermia, bradycardia, hypotension, and altered mental status. Respiratory failure is common, and a difficult airway may be encountered secondary due macroglossia and oropharyngeal edema that can occur. Myxedema coma is a clinical diagnosis, and although thyroid studies and other lab testing should be sent, treatment should not be delayed for test results. Treatment consists initially of medical stabilization, starting with the patient’s ABCs. Initial treatment of hypotension should be with fluids, as vasopressors may be ineffective until thyroid replacement is initiated. Passive rewarming should be the primary method of rewarming, as active rewarming may worsen the patient’s condition. Administration of levothyroxine is key and should be co-administered with stress dose steroids (100 mg hydrocortisone) as there is usually a degree of adrenal crisis which also occurs.

Graves disease is a type of hyperthyroidism. Hyperthyroidism is defined by having excess thyroid hormone circulating due to hyperactivity of the thyroid gland. Thyroid storm is the known life-threatening state of thyrotoxicosis that is most commonly seen in patients with Graves’ disease. Although altered mental status and coma are both associated with thyroid storm other clinical features include fever, tachycardia, dysrhythmias, and heart failure. Sepsis  is defined as an infection that results in a systemic inflammatory response (SIRS). SIRS criteria are HR greater than 90, temperature greater than 38°C or less than 36°C, respiratory rate greater than 20 or PaCO2 less than 32 mm Hg, and white blood cell count greater than 12 x 109/L or less than 4 x 109/L. At least two of the four criteria must be met in combination with an infection or suspected infection. Treatment of sepsis is focused on fluid resuscitation, especially in cases of severe sepsis or septic shock. Broad-spectrum antibiotics as early as possible are also part of early treatment. However, this patient’s clinical picture is consistent with myxedema coma. DKA is a result of improper glucose uptake causing an increase in ketogenesis. Patient’s will often have deep and rapid breathing, tachycardia, and at times a “fruity odor” to their breath.

The correct answer is immediate reversal of the anticoagulation (whether with fresh frozen plasma, prothrombin complex concentrate, recombinant factor VIIa). High dose vitamin K intravenously should be administered, however its reversal mechanism takes approximately 12 to 24 hours, during which time the intracerebral hemorrhage may continue to enlarge. Plasma, prothrombin complex concentrate, or recombinant factor VIIa are more appropriate choices for immediate reversal of warfarin’s anticoagulant action. Protamine sulfate is the reversal agent for heparin, not coumadin. The 2010 AHA/ASA guidelines recommend against the use of prophylactic antiepileptic drugs. However, if a seizure occurs, then antiepileptic drugs would be appropriate to administer, in order to prevent recurrent seizures.

Transient ischemic attack is defined as a transient neurologic deficit that has complete resolution within 24 hours. The ABCD2 score can be used to predict likelihood of subsequent stroke within 2 days. Low risk patients (scores of 0-3) may be discharged home if they have reliable follow-up within two days, whereas moderate risk (4-5 points) and high risk (6-7 points) patients should be admitted for echocardiography, carotid dopplers, and likely carotid endarterectomy.

The scoring system is as follows:

Age of 60 years or greater = 1 point
Initial SBP of 140 or greater OR DBP of 90 or greater = 1 point
Clinical features (Unilateral weakness = 2 points; Speech disturbance without weakness = 1 point; Other symptoms = 0 points)
Duration of symptoms (<10 minutes = 0 points; 10-59 minutes = 1 point; 60 minutes or greater = 2 points)
History of diabetes = 1 point

This patient’s score = 4. He receives 1 point for age and 2 points for unilateral weakness as well as 1 point for history of diabetes.

The correct answer is internal capsule (likely posterior limb) or thalamus. This patient has a pure motor lacunar stroke syndrome, manifested by: pure motor contralateral hemiparesis without any sensory deficits. Lateral frontoparietal, superior temporal is a complete middle cerebral artery distribution, which is manifested by: contralateral hemianesthesia, hemiparesis, hemianopia with gaze preference; If dominant hemisphere: aphasia and apraxia; if nondominant hemisphere: aprosodia, hemineglect. Lateral medulla also known as Wallenberg syndrome, this is a brainstem syndrome manifested by: ipsilateral facial sensory loss, Horner’s syndrome, palatal weakness, dysphagia and ataxia, contralateral body pain and temperature loss. Medial frontoparietal lobes is descriptive of anterior cerebral artery distribution stroke, which is manifested by: contralateral anesthesia, leg > arm hemiparesis, abulia. If dominant hemisphere: mutism; if nondominant hemi- sphere: acute confusional state.

The patient had a transient ischemic attack. Two interventions that reduce mortality in patients with TIA are anticoagulation for patients with atrial fibrillation or LV thrombus, and carotid endarterectomy for carotid stenosis > 70%.  The patient only has 65% stenosis, thus anticoagulation is the better answer choice. While long-term blood pressure control is important in decreasing morbidity and mortality for this patient, emergent blood pressure control has not been shown to decrease the immediate morbidity of this patient. Long-term blood glucose control is also important in decreasing morbidity and mortality for this patient, emergent blood glucose control has not been shown to decrease the immediate morbidity of this patient

The indications for tPA include age at least 18 years, clinical diagnosis of ischemic stroke (no finding on noncontrast Head CT), and time of symptoms onset <4.5 hours.

There are multiple contraindications to systemic thrombolysis (tPA) including: significant stroke/head trauma in prior 3months, prior intracranial hemorrhage, intracranial neoplasm/AVM/aneurysm, recent intracranial/intraspinal surgery, arterial puncture at noncompressible site in past 1 week, sustained SBP at least 185 or DBP at least 110 mmHg, serum glucose <50 mg/dL (<2.8 mmol/L), active internal bleeding or acute bleeding diatheses, current use of anticoagulants or INR > 1.7 and platelet count < 100,000/μL.

The patient has a subarachnoid hemorrhage, of which there are 3 major causes: ruptured aneurysm, leaking AVM, or a peri-mesencephalic bleed. Of ruptured aneurysms, the most common blood vessel affected is the anterior communicating artery in the circle of Willis.

Several historical clues in this scenario point to tuberculosis being the “bad lung infection” in this patient. In patients with seizures that are refractory to benzodiazepines, isoniazid (a common medication for tuberculosis) overdose is a possibility and should be considered. Pyridoxine is the only fully effective pharmacologic treatment for toxic isoniazid seizures, although benzodiazepines have been shown to suppress seizure activity in some cases.

Early, aggressive benzodiazepine administration is associated with decreased morbidity and mortality in status epilepticus. Intramuscular midazolam is superior to intravenous lorazepam; in addition, the dose of lorazepam is inadequate. Endotracheal intubation may ultimately be required, but is a secondary priority; use of a long-acting neuromuscular blockade agent, such as vecuronium, should be avoided. Cranial computed tomography may or may not be needed in this patient, depending on the response to benzodiazepine therapy. Bedside electroencephalograms are most useful in diagnosing nonconvulsive status epilepticus.

Cerebral venous thrombosis may present with headache, lethargy, cranial nerve deficits, seizures, or even psychiatric complaints. CT scan and/or MRI/magnetic resonance angiography (MRA) are likely to reveal the diagnosis. Treatment includes heparin. Neurosurgical consultation is not useful. Subarachnoid hemorrhage would not be expected to cause a focal neurologic deficit. Eclampsia and meningitis would be expected to give characteristic findings on history and examination.

Seizure activity may occur when the serum glucose concentration is less than 45 mg/dL. Although cation derangements may also cause seizures (hyponatremia, hypocalcemia, and hypomagnesemia), sodium levels less than 120 mEq/L are typically seen, with these levels having been achieved rapidly rather than slowly. This level of hypocalcemia is appropriate for the decreased albumin level, likely rendering ionized calcium levels normal. Hyperkalemia does not cause seizures. Uremia may cause status epilepticus, but only at higher levels in cases of a more rapid onset of untreated renal failure or during rapid fluid shifts such as during dialysis.

Periodic paralysis is a rare neuromuscular disorder related to a defect in muscle ion channels. There are several types of periodic paralysis: hypokalemic, hyperkalemic, and thyrotoxic. The hallmark of periodic paralysis is painless muscle weakness precipitated by heavy exercise, fasting, stress, high-carbohydrate meals, or in the case of hyperkalemic periodic paralysis, consumption of potassium rich foods. Hypokalemic periodic paralysis occurs typically in the first or second decade of life and will last from hours to days. It is caused by an autosomal dominant defect in calcium or sodium channels on the membranes of myocytes. Diagnosis can be made with a basic metabolic panel in the presence of paralysis. The potassium level will be < 3.5 mEq/L. A transtubular potassium gradient ((UK+ x POSM) / (UOSM x PK+)) can also be used to diagnose hypokalemic periodic paralysis , a value < 3 mEq/L is highly suggestive of hypokalemic periodic paralysis (normal range is 8 to 9 mEq/L). Hypokalemic periodic paralysis is more common in men and can be treated with carbonic anhydrase inhibitors or potassium-sparing diuretics.

An edrophonium test (C) is used to diagnose myasthenia gravis, a neuromuscular disease causing muscle weakness due to antibodies that block acetylcholine receptors at the postsynaptic neuromuscular junction. Edrophonium blocks the breakdown of acetylcholine by inhibiting acetylcholinesterase, thereby temporarily reversing the weakness caused by myasthenia gravis. This test is rarely performed due to the potential of life threatening bradycardia. Magnetic resonance imaging of the brain (D) is helpful in the diagnosis of multiple sclerosis, a demyelinating disease of the brain and spinal cord characterized by neurologic deficits that change over time, but plays no part in the diagnosis of hypokalemic periodic paralysis. Cerebrospinal fluid (CSF) protein and cell count (B) are useful in the evaluation of Guillain-Barré syndrome (GBS), a rapid-onset ascending paralysis resulting from autoimmune damage to the peripheral nervous system nerves and myelin. Common causes are recent infection, upper respiratory infections, or gastroenteritis with Campylobacter jejuni. Cytomegalovirus has also been linked to GBS, but up to a third of cases of GBS are idiopathic. Treatment is supportive and includes airway protection if the paralysis rises to the level of the diaphragm. CSF analysis will show an elevated protein level with low numbers of white blood cells.

In the emergency department intubating a patient with a NM disease is made less on hard numbers and more on the patient’s overall clinical picture. However, guidelines do exist for these disorders which indicate that an elective intubation should be performed. The Negative Inspiratory Force (NIF)  or Maximal Inspiratory Pressure (MIP) test is one we can perform in the ED if we ask RT for assistance. As the name implies it measures the force of inhalation, it is measured in terms of “negative” values, and >-30 cmH2O indicates the need for likely intubation. FVC (B) is also a metric that can be used, however the normal FVC for an adult ranges from 3,000-5000 mL. There is not a hard indication for intubating a NM disease patient based on PCO2 (C) alone. While 47 is slightly elevated, it is not an isolated reason to intubate this patient. While requiring previous intubations in the past (D) may increase this patient’s risk for respiratory failure, it is not an indication to intubate.

Other metrics for respiratory failure in patient’s with NM disease:

FVC: A forced vital capacity is the total volume exchanged from maximal inhalation/exhalation (i.e. the biggest breath you can take). An FVC less than 15-20 mL/kg in the sitting position. The normal adult human has an FVC of about 3,000-5,000 mL.

NIF or MIP: Maximal negative pressure generated on inspiration. It is an indirect measure of the diaphragm’s force. In general values > -30 cmH2O (i.e. between 0 to -30) should raise concern for impending respiratory failure. For reference, -60 cmH2O is equivalent to “a weak cough” and predicts some difficulty with clearing secretions, and normal is < -100 cm H2O

MEP: Maximal expiratory pressure, which is the opposite of the MIP or NIF as it measures the expiratory force. In general values < 40 cm H2O is a worrisome sign of respiratory failure.

*In concert the above three values comprise the “20-30-40” rule: FVC <20mL/kg, MIP >-30 cmH2O, MEP >40 cmH2O. These parameters are NOT hard and fast rules… they are guidelines and should never override a strong clinical inclination to intubate.

Progressively worsening respiratory acidosis

Inadequate clearing of secretions

Clinical respiratory distress

Cauda equina syndrome is a neurologic emergency and is the result of external compression of the cauda equina by a tumor, abscess, herniated disc, expanding hematoma, or other mass. Because the cauda equina is composed of spinal nerve roots, compression results in a peripheral neuropathy as opposed to a central neuropathy. Signs and symptoms include back pain, urinary retention (measured by a post-void residual bladder volume usually > 100 cc), overflow urinary incontinence, saddle or perianal anesthesia, and lower motor neuron findings such as lower extremity hyporeflexia and flaccidity, which are usually unilateral. Patients with findings concerning for cauda equina syndrome need emergent neurosurgical consultation and advanced imaging with MRI.

Conus medullaris syndrome is another neurosurgical emergency that may present with similar features as cauda equina syndrome, including saddle anesthesia and bladder incontinence. However, because the conus medullaris is the terminal spinal cord and, by definition, a central structure, exam findings will be consistent with upper motor neuron deficits such as lower extremity hyperreflexia or a positive Babinski sign (upgoing toes). A positive straight leg raise test indicates sciatica.

Botulism is a neuromuscular disorder caused by Clostridium botulinum. Through a toxin-mediated process, the release of acetylcholine is inhibited, leading to muscle weakness and paralysis. Classically, botulism causes a symmetric descending paralysis. Cranial nerves and bulbar muscles are often the first to be affected. As the disease progresses, patients can develop respiratory failure through paralysis of respiratory muscles. Patients will typically present with diplopia, dysarthria, and dysphagia. On physical exam, patients with often have dilated pupils that are nonreactive to light. In adults, botulism can occur after the ingestion of canned foods contaminated with Clostridium botulinum. Symptoms typically present within six to 48 hours of exposure to the toxin.

An ischemic stroke can present with signs and symptoms similar to botulism. However, a history of recent ingestion of canned food product and nonreactive dilated pupils is most consistent with botulism, also given this patient’s age a stroke is far less likely. Myasthenia gravis  is a neuromuscular disorder caused by antibodies that lead to ineffective acetylcholine at the neuromuscular junction. It can present similarly to botulism, but symptoms of botulism will develop more rapidly. Also, patients with myasthenia gravis will have reactive and normal-appearing pupils. Scrombroid poisoning is associated with larger game fish such as tuna, and causes a histamine reaction including flushing, headaches, blurred vision, cramping abdominal pain, and GI upset.

This patient presents with a radial neuropathy also called “Saturday Night Palsy.” The radial nerve arises from the C5-T1 roots. It controls extension of the fingers, thumb, wrist and elbow. Symptoms of radial neuropathy depend on the location of compression. Compression in the axilla (typically from improper use of crutches) causes weakness of extension at the elbow, wrist and fingers. More typically, the nerve is compressed between the humeral shaft and another hard surface and results in weakness with extension at the wrist and fingers. This typically occurs from deep sleep (often secondary to inebriation). About 90% of radial nerve mononeuropathies that occur during sleep, coma or anesthesia recover within 6-8 weeks. Patients should be placed in a wrist splint with 60 degrees of dorsiflexion to prevent atrophy and contractures.

Radiographic imaging and pain control are not necessary as the patient does not have any concerning physical exam findings or history of trauma. Activation of stroke protocol would be inappropriate for several reasons. He is outside of any TPA window as he awoke with these symptoms, the symptoms are consistent with a nerve palsy, and his NIHSS scale is low (likely just a 1 for his sensory deficit). Consultation of neurology and MRI of the brain is not needed emergently as the patient has a clinical diagnosis of a radial nerve palsy, he can follow up as an outpatient.

Peripheral Neuropathies
• Sciatic: buttock injury, hip dislocation, ↓ knee flexion, foot drop, rx: analgelsics, physical therapy,
• Common peroneal: proximal fibula injury, footdrop, rx: ankle splint
• Radial: crutches, wrist/finger drop, rx: wrist splint
• Ulnar: elbow injury, ↓ finger adduction/thumb grasp, 4th/5th digit paresthesias
• Lateral femoral cutaneous: inguinal ligament entrapment, upper thigh dysesthesia/numbness

MRI is the diagnostic modality of choice for multiple sclerosis (MS). Multiple sclerosis is characterized by demyelination of axons within the central nervous system. Patients present with symptoms that are “scattered in time and space” meaning that they have intermittent symptoms in anatomically disparate locations. Patients may have patchy motor and sensory deficits and bladder dysfunction. Spinal cord lesions typically involve the posterior columns, lateral spinothalamic tracts and the corticospinal tract. Spinal MRI is the diagnostic modality of choice because it can be used to rule out causes of cord compression while also showing lesions diagnostic for MS.

CT scan may be performed but is NOT a diagnostic test for MS. Carotid duplex scans are used when there is concern for TIA, not in MS. CSF oligoclonal bands are helpful in the diagnosis of MS, but are not the diagnostic test of choice, furthermore serum oligoclonal bands are of no help.

The patient is most likely suffering from Guillain-Barré syndrome, a progressive, ascending demyelinating polyneuropathy that typically begins with lower extremity weakness. Classically, GBS presents with symmetric lower extremity weakness, decreased or absent deep tendon reflexes, and little or no sensory involvement (although variable). It follows a respiratory or gastrointestinal infection by weeks to days. Common organisms implicated include Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, and Mycoplasma pneumoniae. Elevated protein with only a mild pleocytosis on cerebrospinal fluid analysis (termed albuminocytologic dissociation) is highly specific in the clinical setting of suspected GBS. Patients with probable GBS should receive a neurology consult and be admitted for either intravenous immunoglobulin (IVIG) therapy or plasma exchange. Both IVIG and plasma exchange have been demonstrated to be more effective than placebo has, but no advantage is incurred upon administration of both therapies. Neither therapy has been proven more effective than the other.

 The edrophonium test is used for myasthenia gravis, not for GBS. Corticosteroids alone are no longer recommended, but IV steroids coadministered with IVIG may speed recovery. Regardless, steroids have no role in the diagnosis of GBS. Pleocytosis and oligoclonal bands of immunoglobulin G is characteristic of MS

This patient has myasthenia gravis, caused by antibodies to the acetylcholine receptor (AChR) at the neuromuscular junction. Failure to respond to acetylcholine stimulation leads to decreased muscle fiber activationand muscle weakness. The antibodies cause AChR degradation, dysfunction, and blockade. Ptosis and diplopia are most commonly the first symptoms. Patients may also experience dysphagia, proximal muscle weakness, and dyspnea. A full myasthenic crisis may lead to respiratory failure due to flaccid paralysis of the muscles of respiration.

Botulinum toxin inhibits acetylcholine release at the synapse (B). It is produced by Clostridium botulinum, an anaerobic spore-forming bacterium. C. botulinum occurs naturally in the soil and causes human disease when ingested. Botulism is associated with a descending symmetric paralysis. Amyotrophic lateral sclerosis (ALS) is caused by a mutation of superoxide dismutase (C) leading to an abnormal response to stress and cell death. Central and peripheral nerves are affected with sparing of sensory and cognitive function. Initial symptoms are muscle weakness, atrophy, fasciculations, dysarthria, and dysphagia. Parkinson’s disease is associated with depigmentation and dopaminergic neuron loss (D) in the midbrain, especially the substantia nigra. It is also characterized by Lewy bodies, which are cytoplasmic inclusions seen under the microscope. Parkinson’s disease is characterized by tremor, cogwheel rigidity, bradykinesia or akinesia, and postural and equilibrium impairment.

Normal pressure hydrocephalus (NPH) is characterized by the following characteristics: wet, wacky, and wobbly. More specifically, it includes a triad of progressive dementia, ataxia and urinary frequency or incontinence. It is a reversible cause of dementia and results from defective uptake of CSF. Head CT will reveal ventricular enlargement. Atrophy may occur in chronic alcoholics or the elderly, but is neither sensitive nor specific for NPH. A brain mass can cause a variety of symptoms, but headache with a neurologic deficit would be a more common presentation

A patient in myasthenic crisis should receive immunomodulatory therapy. This includes either IVIG or plasmapharesis. The decision to start either should be made in concert with a consulting neurology service. The literature does show a benefit of IVIG over placebo, however the difference in IVIG vs. plasmapharesis is unclear. In general, if resources are available, plasmapharesis is preferred as it directly removes the AChR ab from the blood, whereas IVIG acts by both binding harmful ab, as well as downregulating the patient’s immune response. This is a costly medication and requires repeated doses.

Pyridostigmine and neostigmine are used orally for outpatient maintenance and not for acute crisis. They act by inhibiting AChE, increasing the amount of ACh at the NMJ. The IV dose might cause complications from the cholinergic excess such as increased secretions. Rituximab is not a first line treatment in MG, though it may play a role in outpatient therapy by decreasing the production of ab from B-cells. Edrophonium is used for the diagnosis of MG, and this patient already has established MG. It is a short-acting AChEi . Administration in patients with MG should lead to decreased symptoms in 30-90 seconds. Use of edrophonium can result in potentially significant complications, such as symptomatic bradycardia and bronchospasm. These effects can be reversed with atropine. It should be used with caution, if at all, in older patients, those with asthma/COPD and those with underlying cardiac disease.