Question: A 46-year-old man presents with his wife to his primary care provider for depression and strange movements. His wife reports that her husband has not been himself for the last 2 months. Whereas he was previously outgoing and “the life of the party,” the patient is now irritable and withdrawn. He is a partner at an accounting firm, but his colleagues are threatening to fire him if he continues to perform poorly at work. The patient cannot explain the recent changes to his mood and tearfully admits he fears there is something seriously wrong with him. His wife says that he is getting worse. The patient’s medical history is significant for hypertension, for which he takes lisinopril. His family history is unknown as he was adopted. The patient met his mother once and never knew his father but was told he died in his 50s. He drinks a few glasses of wine per week and has never smoked. His temperature is 98.6°F (37°C), blood pressure is 141/87 mmHg, pulse is 81/min, and respirations are 12/min. On physical exam, the patient has a flat affect with a facial grimace and sudden jerky movements of his upper extremities. Which of the following is most likely to be seen on further workup?

Alpha-synuclein aggregates on brain biopsy

Answer: Dorsal striatum atrophy on head CT

Explanation:
This patient presents with recent mood changes, jerky movements of the upper extremities, and a history of early death in his father, which suggests a diagnosis of Huntington disease. Huntington disease is characterized by atrophy of the caudate nucleus, which is a structure of the dorsal striatum.

Huntington disease (HD) is an autosomal dominant trinucleotide (CAG) repeat disorder located on chromosome 4. HD typically presents with mood changes and abnormal movements (particularly chorea or myoclonus) over weeks to months. Although executive function can be affected early on, memory is usually preserved until relatively late in the disease. Frontotemporal dementia, in contrast, usually presents with behavioral changes, and Alzheimer disease has memory impairment as an early sign. Caudate atrophy is typically seen on head imaging in Huntington disease. This disease cannot be cured but supportive treatment includes benzodiazepines and tetrabenazine for symptomatic relief of muscle spasms.

Walker presents a review of the evidence regarding the etiology of Huntington disease. He discusses that typically the onset of symptoms is in middle age after affected individuals have had children. He recommends a better understanding of the function of the mutant protein in this disease.

Incorrect Answers:
Answer A: Alpha-synuclein aggregates would be found on brain biopsy in Parkinson disease and Lewy body dementia. Lewy body dementia is characterized by parkinsonism and visual hallucinations. Patients would present with cogwheel rigidity, bradykinesia, and a resting tremor. Treatment is with dopamine agonists such as carbidopa/levodopa.

Answer C: Frontotemporal atrophy is consistent with a diagnosis of frontotemporal dementia. Frontotemporal dementia presents primarily with early behavioral changes, and it would not present with myoclonus. Treatment is supportive as there is no cure for this disease. Patients and their spouses may require psychiatric support for changes in behavior.

Answer D: Neurofibrillary tangles on brain biopsy would suggest a diagnosis of Alzheimer disease. This disease presents with a slowly progressive cognitive decline in elderly patients. Patients have early loss of memory but have maintained motor function and behavior until later in the course of the disease.

Answer E: A positive 14-3-3 CSF assay would confirm the diagnosis of Creutzfeldt-Jakob disease (CJD). Although CJD may also present with mood changes and myoclonus, CJD most prominently causes rapidly progressive dementia early in life. Treatment is supportive and death occurs quickly.

Bullet Summary:
Huntington disease is an autosomal dominant trinucleotide repeat disorder located on chromosome 4 that causes caudate atrophy and presents with mood changes, abnormal movements, and subcortical dementia.


Question: A 36-year-old woman, G1P1001, presents to her gynecologist for an annual visit. She has a medical history of hypertension for which she takes hydrochlorothiazide. The patient’s mother had breast cancer at age 68, and her sister has endometriosis. She states that she feels well and has no complaints. Her temperature is 98.6°F (37.0°C), blood pressure is 138/74 mmHg, pulse is 80/min, and respirations are 13/min. Her BMI is 32.4 kg/m^2. A pelvic exam reveals a non-tender, 12-week-size uterus with an irregular contour. A transvaginal ultrasound is performed and demonstrates two intramural leiomyomas. Which of the following is a likely complication of this patient’s condition?

Answer: Iron deficiency anemia

Explanation:
This patient presents with an irregularly enlarged uterus on examination consistent with the intramural leiomyomas (fibroids) seen on ultrasound. Leiomyomas increase the risk of iron deficiency anemia due to heavy menstrual bleeding.

Leiomyoma are benign overgrowths of myometrial tissue. The most common presentation is heavy or prolonged menstrual bleeding or symptoms related to mass effect such as pelvic pressure or pain.. Affected women often develop these symptoms in their 30s or 40s, though they may occur at any age, and many are asymptomatic. Risk factors include early menarche, obesity, family history, and African American race. Submucosal fibroids that disrupt the endometrium may cause heavy menstrual bleeding. Intramural fibroids also commonly cause heavy bleeding despite the fact that they do not disrupt the endometrium due to alterations in the uterine vasculature. Patients may be managed medically with a wide range of options including oral contraceptive pills, tranexamic acid, and GnRH agonists. Definitive management is with surgical intervention. Myomectomy is preferred for patients that desire child bearing in the future. For those that do not have plans for pregnancy in the future, hysterectomy is recommended.

Yang et. al review the diagnosis and management of uterine fibroids. They note the range of clinical presentations to include heavy menstrual bleeding. They discuss management to include surgical intervention with myomectomy or hysterectomy.

Incorrect Answers:
Answer A: Amenorrhea, or the absence of menstrual bleeding, is often due to anovulation or uterine adhesions. Uterine fibroids are not associated with amenorrhea.

Answer B: Endometrial cancer is a known cause of heavy menstrual bleeding. However, this patient’s ultrasound notes the presence of intramural leiomyomas. Her age, history, and ultrasound findings are not suggestive of endometrial cancer.

Answer D: Uterine prolapse is associated with multiparity, chronically increased intra-abdominal pressure (from chronic obstructive pulmonary disease, obesity, etc.), and connective tissue diseases. While fibroids rarely may prolapse, they typically do not cause the entire uterus to prolapse.

Answer E: Uterine sarcoma may very rarely result from uterine fibroids, as both arise from the uterine smooth muscle. However, this patient’s history and ultrasound findings are more suggestive of uterine leiomyoma.

Bullet Summary:
Patients with uterine leiomyomata (fibroids) are at risk for iron deficiency anemia due to heavy bleeding.


Question: A 47-year-old man presents to the emergency department with a headache. He suddenly developed a throbbing, bitemporal headache about 5 hours ago "out of nowhere." He has a history of migraine headaches, but he feels that this headache is significantly more painful than his typical migraines. The patient took his prescribed sumatriptan with no relief of his symptoms. He also endorses nausea and he reports that he vomited once before arrival in the emergency department. The patient denies any recent trauma to the head. His medical history is significant for migraines and hypertension. He has a 20-pack-year smoking history and a history of cocaine use. He drinks 5-6 beers per week. His temperature is 98.6°F (37°C), blood pressure is 147/91 mmHg, pulse is 62/min, and respirations are 12/min. On physical exam, he appears to be in moderate distress and has pain with neck flexion. He has no focal neurologic deficits. A head CT is performed and can be seen in Figure A. This patient’s condition affects the space in between which of the following brain layers?

Answer: Arachnoid mater and pia mater

Explanation:
This patient presents with a sudden, severe headache and a head CT showing bleeding in the subarachnoid space, which confirms a diagnosis of subarachnoid hemorrhage. The bleeding in a subarachnoid hemorrhage occurs between the arachnoid and pia mater.

Subarachnoid hemorrhages present with a sudden, severe headache referred to as a “thunderclap” headache. Patients typically complain that the headache is the "worst headache of their life." Some patients also present with symptoms of meningeal irritation such as nausea and vomiting, nuchal rigidity, or focal neurologic deficits. Although most cases of subarachnoid hemorrhage are secondary to trauma, patients with risk factors such as hypertension and cocaine use can have non-traumatic presentations. Patients with an expanding bleed or acute neurologic changes require surgical clipping or embolization of the bleeding vessels.

Macdonald and Schweizer review the evidence regarding the treatment of patients with subarachnoid hemorrhage. They discuss how survival rates have improved with early aneurysm repair, prescription of nimodipine, and advanced intensive care support. They recommend early diagnosis in order to improve outcomes in these patients.

Figure/Illustration A is an axial slice of a head CT showing hyperintensity in the basal cisterns (red circle). This finding is consistent with bleeding in the subarachnoid space.

Incorrect Answers:
Answer B: Bleeding between the dura and arachnoid mater describes a subdural hematoma. A subdural hematoma presents as a headache with progressive confusion. It appears as a crescent-shaped lesion on head CT. Patients with an expanding bleed will require surgical decompression to avoid herniation syndromes.

Answer C: Bleeding between the periosteum and galea aponeurosis describes a subgaleal hemorrhage. Subgaleal hemorrhages occur in newborns and are caused by vacuum-assisted deliveries. Treatment is usually supportive as the bleeding will resolve by itself. Some patients with severe bleeds may require transfusion due to excessive blood loss.

Answer D: Bleeding between the periosteum and skull describes a cephalohematoma. Cephalohematomas occur in newborns and are caused by rupture of the blood vessels crossing the periosteum. They are usually secondary to a prolonged second stage of labor. Treatment is supportive as the blood will resolve and be resorbed.

Answer E: Bleeding between the skull and dura mater describes an epidural hematoma. An epidural hematoma presents as a brief loss of consciousness followed by a lucid interval and then progressive confusion or somnolence. It appears as a biconvex lesion on head CT. Treatment is with urgent surgical decompression as patients will rapidly decompensate after the lucid interval.

Bullet Summary:
Subarachnoid hemorrhage classically presents as a thunderclap headache with a brief loss of consciousness and meningismus.


Question: A 25-year-old man presents to his primary care physician with anxiety and fatigue. During this past week, he has been unable to properly study and prepare for final exams because he is exhausted. He has been going to bed early but is unable to get a good night’s sleep. He admits to occasional cocaine and marijuana use. Otherwise, the patient has no significant medical history and is not taking any medications. His temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. On physical exam, the patient is a tired and anxious appearing young man. His neurological exam is within normal limits. The patient states that he fears he will fail his courses if he does not come up with a solution. Which of the following is the most appropriate initial step in management?

Answer: Sleep hygiene education

Explanation:
This patient is presenting with trouble sleeping without a known organic cause, for which the most appropriate initial step in management is to discuss sleep hygiene with the patient.

In any patient that presents with a chief complaint of fatigue, it is always best to assess how they are sleeping. After a physical exam is performed in order to rule out signs or symptoms of a severe organic cause (neurologic deficits), the most appropriate initial step in management is to discuss sleep hygiene with the patient. Sleep hygiene includes discussing the patient’s exposure to light (eliminate exposure to blue light at night), altering diet (no large meals or caffeine before bed), and behavioral modification (only using the bed for sleep). Once a trial of proper sleep hygiene has been tried, then it would be appropriate to potentially move on to other interventions including pharmacological interventions.

Horne presents evidence regarding the best sleep hygiene practices. He discusses how exercise and avoidance of caffeine near bedtime is beneficial to helping with insomnia. He recommends using these interventions prior to pharmacologic treatment.

Incorrect Answers:
Answer A: Alprazolam is a benzodiazepine that could be used to help with this patient’s anxiety and could help them sleep. However, benzodiazepines are potent drugs with addictive properties and would not be used as first-line treatment for insomnia. Lifestyle modifications should be tried first to determine if these will be effective in helping the patient sleep.

Answer B: Melatonin is a supplement that is often used to improve sleep quality. Starting the patient on a supplement is plausible as an initial step in management, but it would not take precedence over educating the patient regarding sleep hygiene. These interventions often are very effective and have no side effects.

Answer C: Polysomnography could be the most appropriate initial step in management if an organic cause for this patient’s sleep problems (such as obstructive sleep apnea) is suspected. There is nothing suggesting an organic cause for this patient’s sleep issues and it is highly likely that a college-age student near finals period would benefit from a discussion on sleep hygiene prior to an expensive diagnostic test such as polysomnography.

Answer E: Zolpidem is a muscle relaxant and hypnotic with a similar mechanism of action to a benzodiazepine. Zolpidem has the potential to be addicting and would not be tried prior to sleep hygiene interventions. Some patients who are refractory to other interventions may benefit from the use of Zolpidem.

Bullet Summary:
Any patient that presents with fatigue and trouble sleeping without a clear organic cause should first be counseled in sleep hygiene habits prior to any other interventions or diagnostic tests.


Question: A 27-year-old man presents to his primary care physician for exposure to toxic materials. The patient states that when he left for work this morning he was certain that he had closed the door to his pantry. Upon returning home, he saw that the door to his pantry was wide open. The patient is certain that his neighbors have been tampering with his food and potentially poisoned him. He further states that he knows they have been trying to break into his house and steal his things. He has tried multiple times to get them evicted from the building to no avail. It is for this reason that he is certain that they are trying to get their revenge upon him. His temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. The physician performs a physical exam and tells the patient that he thinks there is nothing to be concerned about, but that he should call him or come into the office if he experiences any symptoms. The patient is outraged at this news and requests a competent doctor who is not colluding with his enemies. He storms out of the office angrily, stating that he deserves the best in medical care. Which of the following is the most likely disorder that this patient is suffering from?

Answer: Paranoid personality disorder

Explanation:
This patient is presenting with a history of mistrust and suspicion of others without frankly psychotic features suggesting a diagnosis of paranoid personality disorder.

Paranoid personality disorder is a cluster A personality disorder that is genetically associated with schizophrenia. These patients present with a pervasive mistrust of others based on little or no evidence. These patients are inclined to believe others have bad intentions that are directed toward them. They are often odd, very emotionally cold, and typically are involved in frequent litigations. Patients can be hard to manage though they often benefit from cognitive behavioral therapy. Anxiolytics may also be helpful in calming these patients.

Triebwasser et al. review the evidence regarding the diagnosis of paranoid personality disorder. They discuss how there is relatively little research on these patients because it is difficult to recruit them for studies. They recommend considering the removal of this diagnosis and replacing it with a domain of paranoia.

Incorrect Answers:
Answer A: Borderline personality disorder presents with emotional instability, unstable relationships, and recurrent self-harm behaviors. These patients will typically demonstrate the defense mechanism of splitting (seeing things as all good or all bad). Dialectical behavioral therapy can be used in order to help these patients mitigate self-injurious behaviors.

Answer B: Intermittent explosive disorder presents with sudden episodes of aggression out of proportion to the stressor. This patient’s response is a result of his paranoia rather than an excessive response to reasonable stimuli. Cognitive behavioral therapy can be used in order to help these patients control their emotional outbursts.

Answer C: Narcissistic personality disorder presents with a grand sense of self-importance and preoccupation with success and admiration. These individuals are typically selfish and lack empathy. Though this patient’s demand for the best medical care may be reflective of narcissistic personality disorder, the majority of his behaviors point more toward a diagnosis of paranoid personality disorder.

Answer E: Schizoid personality disorder presents in patients with isolated and emotionally restricted behavior. These patients are typically alone but enjoy being alone and often seem disinterested in others. These patients typically do not seek relationships and do not seek therapy as they are not bothered by their behaviors.

Bullet Summary:
Paranoid personality disorder presents with mistrustful behavior, suspicion of others, oddness, emotional coldness, and they are typically involved in many litigations.


Question: A 32-year-old man presents to his primary care provider for a headache. He reports that he has headaches at night several times a week. He first developed these headaches over 1 year ago, but they started up again 3 weeks ago. The episodes start suddenly and feel like a stabbing, electrical pain over his left eye. He also reports tearing of the left eye during these episodes. The headaches self-resolve over the course of 2-3 hours, but the patient complains that he is avoiding going to sleep for fear of waking up in pain. His medical history includes type 1 diabetes mellitus and an episode of herpes zoster on his right flank 1 year ago. His only home medication is insulin. His temperature is 98.6°F (37°C), blood pressure is 112/69 mmHg, pulse is 61/min, and respirations are 14/min. On physical exam, his extraocular muscles are intact and his eyes are not injected. A CT of the head and sinuses shows no acute abnormalities. Which of the following is most likely to prevent future episodes of headache in this patient?

Answer: Verapamil

Explanation:
This patient presents with an episodic, stabbing pain in the periorbital region that occurs at night, which is consistent with a diagnosis of cluster headaches. Verapamil is used as prophylaxis against cluster headaches.

Cluster headaches usually occur at night and may wake patients out of sleep. Cluster headaches may occur several times a week to multiple times daily and the attacks may be followed by a period of remission. Cluster headaches also typically involve the periorbital region and are unilateral. Patients also classically describe their headaches as “stabbing”. Tension-type headaches are more commonly described as dull and migraines are usually described as pulsating or throbbing. Treatment is with high-flow oxygen during acute episodes and prophylaxis against long-term headaches includes calcium channel blockers such as verapamil.

Hoffman and May review the evidence regarding the treatment of cluster headaches. They discuss how high-flow oxygen and triptans are the most effective abortive treatment for acute attacks. They recommend using chronic prophylactic medications such as verapamil.

Incorrect Answers:
Answer A: Carbamazepine is the treatment of choice for trigeminal neuralgia, which presents as shooting pain in the face. They have exquisite tenderness to palpation over the affected neurologic distribution.

Answer B: High-flow oxygen is an abortive treatment for cluster headaches. Cluster headaches typically present in men with unilateral pain around the eye that is worse at night. Associated symptoms include spasms of the unilateral face as well as tearing and visual abnormalities in the eye. This treatment is not effective for long-term prophylaxis.

Answer C: Sumatriptan is used as an abortive treatment for cluster headaches and migraines, but it is not used as prophylaxis for either. Migraines present as unilateral, throbbing headaches that may be associated with nausea and/or vomiting, photophobia, phonophobia, and aura.

Answer D: Topiramate is used as prophylaxis for migraines rather than cluster headaches. Migraines present as unilateral, throbbing headaches that may be associated with nausea and/or vomiting, photophobia, phonophobia, and aura.

Bullet Summary:
Cluster headaches classically present as sharp or stabbing periorbital headaches that occur at night, with verapamil being the prophylactic treatment of choice and high-flow oxygen being the most effective abortive treatment.


Question: A 28-year-old woman presents to her primary care provider with a headache. Every few weeks she has an episode of a right-sided, throbbing headache. The episodes began several years ago and are accompanied by nausea and bright spots in her vision. The headache usually subsides if she lies still in a dark, quiet room for several hours. The patient denies any weakness, numbness, or tingling during these episodes. Her medical history is significant for acne, hypothyroidism, obesity, and endometriosis. Her home medications include levothyroxine, oral contraceptive pills, and topical trans-retinoin. Her temperature is 98.6°F (37°C), blood pressure is 125/81 mmHg, pulse is 64/min, and respirations are 11/min. She has 2 glasses of wine with dinner several nights a week and has never smoked. She works as a receptionist at a marketing company. On physical exam, the patient has no focal neurologic deficits. A CT of the head is performed and shows no acute abnormalities. Which of the following is the most appropriate treatment for this patient during these episodes?

Answer: Sumatriptan

Explanation:
This patient presents with episodic, unilateral throbbing headaches with photophobia, which suggests a diagnosis of migraine. The most appropriate treatment during these episodes is an abortive medication such as sumatriptan.

Migraines present with unilateral, "throbbing" pain and are associated with nausea and vomiting, photophobia, and phonophobia. Migraines can occur after exposure to specific triggers or they can occur idiopathically. Patients may also endorse visual auras such as bright lines or shapes in the visual field and sensory auras such as paresthesias. Medications like NSAIDs or sumatriptan are usually used as abortive treatment during acute episodes of migraine. Patients with recurrent migraines can be managed with prophylactic medications like topiramate, beta-blockers, or amitriptyline.

Mayans and Walling present evidence regarding the treatment of migraines. They discuss how acetaminophen, nonsteroidal anti-inflammatory drugs, triptans, antiemetics, ergot alkaloids, and combination analgesics are effective against this disease. They recommend providing these treatments in order to mitigate symptoms.

Incorrect Answers:
Answer A: Acetazolamide is used to treat idiopathic intracranial hypertension (IIH). IIH is most commonly seen in obese women of childbearing age and can be caused by medications such as oral isotretinoin (not topical tretinoin, as in this case) and tetracyclines. The initial symptom is usually a pulsatile headache that worsens with lying down and improves upon standing.

Answer B: High-flow oxygen is an abortive treatment for cluster headaches. Cluster headaches typically present in male patients with unilateral pain around the eye that is worse at night. Associated symptoms include spasms of the unilateral face as well as tearing and visual abnormalities in the eye.

Answer D: Topiramate can be used as prophylaxis for migraines but is not commonly used as an abortive medication. Chronic prophylactic medications generally function by decreasing spasms of the cranial blood supply in order to prevent a migraine from developing. They have minimal effectiveness after a migraine has already developed.

Answer E: Verapamil is a prophylactic medication used for cluster headaches. In addition to presenting in the peri-orbital region, cluster headaches are typically accompanied by lacrimation, rhinorrhea, and focal findings such as ptosis. It would not be effective in terminating a migraine.

Bullet Summary:
Triptans are used as an abortive treatment for migraines, which present as unilateral, throbbing headaches that may be associated with nausea and/or vomiting, photophobia, phonophobia, and aura.


Question: A 54-year-old man is brought to the emergency department following a skiing accident. The patient lost control of his skis and collided with a tree. He lost consciousness for about 30 seconds. When he woke up, he was mildly confused but able to ski down the rest of the mountain. The patient initially denied medical treatment but his wife convinced him to be taken by ambulance to the emergency department for evaluation. The paramedics report that at the scene the patient had a Glasgow Coma Score (GCS) of 15. During the ride, he became gradually more somnolent. His medical history is significant for hypertension, hyperlipidemia, coronary artery disease, and a prior transient ischemic attack. His medications include atorvastatin and lisinopril. In the emergency department, his temperature is 97.8°F (36.6°C), blood pressure is 141/84 mmHg, pulse is 71/min, and respirations are 16/min. He is difficult to arouse with a GCS of 7. A head CT is performed and can be seen in Figure A. Which of the following is the most likely cause of this patient’s presentation?

Secondary hemorrhage into prior area of infarction

Answer: Damage to the sphenoid bone

Explanation:
This patient presents with a lucid interval followed by worsening somnolence and a biconvex lesion on head CT, which suggests a diagnosis of acute epidural hematoma. Acute epidural hematomas are most commonly caused by damage to the middle meningeal artery from trauma to the sphenoid bone.

Patients with an epidural hematoma typically have an initial loss of consciousness followed by a lucid interval, during which their mental status returns to baseline or near baseline. As the hematoma expands, patients display increasing somnolence from the increase in intracranial pressure. For that reason, epidural hematomas are sometimes referred to as the “talk and die” syndrome. If not relieved, this increase in intracranial pressure can lead to uncal herniation or death. Patients with neurologic findings or those with expanding bleeds require urgent surgical decompression.

Dadashi et al. present cases of patients with epidural hematoma from acute trauma. They discuss how patients often sustain these injuries from extreme sports. They recommend being aware of this syndrome so that acute interventions can be taken.

Figure/Illustration A demonstrates a biconvex hyperdensity that is limited by suture lines (red circle). This is the typical appearance of an epidural hematoma on head CT.

Incorrect Answers:
Answer A: Damage to the cerebral bridging veins results in a subdural hematoma (SDH). An acute SDH more commonly presents with a gradual increase in headache and confusion rather than the “talk and die” syndrome presented in this vignette. However, a minority of patients with acute SDH may have a lucid interval followed by progressive neurological decline. SDH would have a crescent-shaped appearance on head CT rather than the biconvex appearance of an epidural hematoma.

Answer C: Occlusion of cerebral small penetrating arteries describes a lacunar stroke. Although this patient has 2 risk factors for lacunar stroke (hypertension and smoking), this patient’s head CT demonstrates an epidural hematoma.

Answer D: A ruptured cerebral aneurysm would most likely lead to a subarachnoid hemorrhage. A subarachnoid hemorrhage presents as a sudden, severe headache (“thunderclap headache”), confusion or somnolence, nausea and vomiting, and meningismus. Surgical clipping or embolization of the bleeding vessel may be required.

Answer E: Secondary hemorrhage into a prior area of infarction usually occurs in the setting of a recent embolic stroke. Although this patient has a history of TIA and other risk factors for stroke, his head CT demonstrates an epidural hematoma. Treatment is supportive with the control of underlying risk factors such as hypertension.

Bullet Summary:
Epidural hematomas are caused by damage to the middle meningeal artery as it passes through the foramen spinosum of the sphenoid bone.


Question: A 38-year-old woman presents to the emergency department with difficulty eating. Over the past week, she has had increasing difficulty chewing her food and progressive pain in the left side of her jaw. This morning, she was unable to close her mouth for several minutes after taking a bite of her breakfast. Two months ago, the patient had a root canal on a left molar. Her medical history is significant for hyperlipidemia, mild intermittent asthma, and type 2 diabetes mellitus. She has never smoked and has 10-15 alcoholic drinks per week. She works as an aide at a nursing home. Her temperature is 100.8°F (38.2°C), blood pressure is 133/74 mmHg, pulse is 105/min, and respirations are 14/min. On physical exam, there is a bluish hue to the skin on the lower left side of the patient’s face. A 3x4 cm non-tender mass can be palpated inferior to the angle of the left mandible. Thick exudate is draining from an opening in the skin. The gram stain of the exudate can be seen in Figure A. Which of the following is the most appropriate treatment for this patient?

Answer: Penicillin

Explanation:
This patient presents with fever, trismus, and cutaneous drainage of sulfur granules in the setting of a recent dental procedure, which points to a diagnosis of cervicofacial actinomycosis. The most appropriate treatment for this disease is penicillin.

Actinomyces is a gram-positive, filamentous rod that causes a cervicofacial infection after direct inoculation during a dental procedure or trauma. It typically presents in an indolent fashion over a time course of weeks to months in which a hard, indurated mass evolves into multiple abscesses that drain through sinus tracts through the skin. The exudate is classically thick with small yellow “sulfur” granules, although it may also be serosanguinous. Since the infection spreads by direct extension, patients may complain of pain when the infection infiltrates or compresses nearby structures. Trismus may occur for the same reasons. The treatment of choice is penicillin.

Valour et al. review the evidence regarding the treatment of Actinomyces infection. They discuss how prolonged (6- to 12-month) high-dose penicillin or amoxicillin is required for the treatment of this disease. They recommend preventive measures such as improvement of dental hygiene to prevent this disease.

Figure/Illustration A demonstrates the histologic appearance of a basophilic “sulfur granule” (red circle). These granules are found in the draining exudate in patients with actinomycosis.

Incorrect Answers:
Answer A: Amphotericin B is the treatment of choice for fungal infections such as mucormycosis. This disease generally presents with a rapidly progressive soft tissue infection that can be fatal if left untreated. Patients would present with black fungal lesions generally in immunocompromised or diabetic hosts.

Answer B: Clindamycin may be used in the treatment of empyema as it has activity against many anaerobes. Although this patient has a substantial weekly alcohol intake and has a chief complaint of “difficulty eating,” she has no true pulmonary symptoms and her presentation is more likely related to her recent dental procedure.

Answer C: Metronidazole may be used in the treatment of empyema. Although this patient has a substantial weekly alcohol intake and has a chief complaint of “difficulty eating,” she has no true pulmonary symptoms and her presentation is more likely related to her recent dental procedure.

Answer E: Trimethoprim-sulfamethoxazole is the treatment of choice for Nocardiosis, which may cause cutaneous lesions in immunocompromised hosts. This disease more commonly causes pulmonary or central nervous system disease. Nocardia would not explain the granule seen in histology.

Bullet Summary:
Actinomyces is a gram-positive anaerobic rod that presents as an indurated mass with draining sinus tracts and should be treated with penicillin.


Question: A 17-year-old girl presents to the emergency department with a headache. The patient has had headaches in the past but this is the worst headache of her life. Her symptoms started yesterday and have been getting progressively worse. The patient states that the pain is mostly on the left side of her head. There has been a recent outbreak of measles at the patient’s school and the patient’s mother has been trying to give her daughter medicine to prevent her from getting sick. Her mother fears that her daughter may have caught measles. Her temperature is 98.6°F (37°C), blood pressure is 123/74 mmHg, pulse is 85/min, and respirations are 13/min. On exam, the patient is an obese girl who is clutching her head with the light in the room turned off. Her neurological exam is within normal limits. Fundoscopic exam reveals mild bilateral papilledema. An MRI of the head is obtained and reveals cerebral edema. A lumbar puncture reveals an increased opening pressure with a normal glucose level. Which of the following is the most likely diagnosis?

Answer: Fat-soluble vitamin overuse

Explanation:
This patient is presenting with headache, bilateral papilledema, obesity, and increased opening pressure on lumbar puncture suggesting a diagnosis of Idiopathic intracranial hypertension (IIH or pseudotumor cerebri). This disease is associated with hypervitaminosis A (a fat-soluble vitamin).

Pseudotumor cerebri classically presents in an obese woman with symptoms of headache and double vision. Overuse of supplements containing vitamin A is a risk factor for developing idiopathic intracranial hypertension. Vitamin A is often used to decrease the duration and severity of a measles infection. On exam, patients will have bilateral papilledema, an increased CSF opening pressure, and cerebral edema. A lumbar puncture is the most accurate diagnostic test for this disease. Treatment is with cessation of offending agents and with carbonic anhydrase inhibitors such as acetazolamide to decrease the rate of cerebrospinal fluid production. Optic nerve sheath fenestration can also be performed in patients who fail medical management.

Fuerst et al. present evidence regarding patients with hypervitaminosis A. They discuss how these patients can present with pseudotumor cerebri. They recommend being vigilant about vitamin A toxicity.

Incorrect Answers:
Answer A: Bacterial meningitis presents with a stiff neck, photophobia, fever, and a CSF finding of low glucose in contrast to this patient’s normal glucose. The most common causes of bacterial meningitis in young adults are N. meningitidis or S. pneumonia. Patients should be treated empirically with ceftriaxone and vancomycin because this is a medical emergency.

Answer C: Migraine headache can present with a unilateral pulsing/pounding headache and photophobia; however, it would not present with physical exam findings of papilledema or cerebral edema. Acute migraines can be treated with NSAIDs and triptan medications. Chronic prophylaxis is with beta-blockers or calcium channel blockers.

Answer D: Subarachnoid hemorrhage presents with the classic, “worst headache of my life,” as this patient has; however, there would be blood visible on MRI which was not described. In addition, there are no symptoms of meningeal irritation from a subarachnoid bleed. Patients may need surgical embolization or clipping of the bleeding vessel.

Answer E: Viral meningitis would present with a stiff neck, photophobia, fever, and CSF that would only demonstrate lymphocytic pleocytosis. Though this patient’s CSF glucose is normal, her other symptoms do not point toward a diagnosis of meningitis. Treatment is either supportive or with antiviral agents depending on the causative organism.

Bullet Summary:
Hypervitaminosis A can cause pseudotumor cerebri, which typically presents in overweight women with headache, double vision, papilledema, and cerebral edema on MRI.


Question:
A 45-year-old man with history of IV drug use presents to the outpatient clinic with a history of fever for 5 days. He also endorses sweats, headache behind the eyes, sore throat, muscle and joint pain, and a new rash seen in Figure A. He reports that he traveled to Africa last year and returned from a vacation to Thailand 10 days ago. His immunizations are up to date, although he missed the pre-departure travel medicine consult before his trip to Thailand. His temperature is 101.5°F (38.6°C), blood pressure is 125/80 mmHg, pulse is 105/min, and respirations are 14/min. Laboratory testing reveals the following:

Leukocyte count: 2,400/mm^3
Hemoglobin: 13.4 g/dL
Hematocrit: 40%
Mean corpuscular volume: 81 µm^3
Platelets: 92,000/mm^3

Serum:
AST: 112 IU/L
ALT: 69 IU/L
ALP: 78 IU/L
Bilirubin: 0.9 mg/dL

Which of the following is the diagnostic test of choice for this disease?

Answer: Serologic testing

Explanation:
This patient is presenting with fever, headache with retro-orbital pain, muscle aches, joint pain, rash, leukopenia and thrombocytopenia, which are classic findings for dengue fever. The diagnostic test of choice for dengue is serology.

Dengue is a viral infection endemic to South Asia, Central and South America, and the Caribbean. Although most infections are asymptomatic, notable characteristics include headache with retro-orbital pain, bone pain ("break-bone fever"), leukopenia, and thrombocytopenia (< 100,000 /µL). Symptoms typically develop several days to 2 weeks following the bite of an infected mosquito. The diagnostic test of choice is a serologic antibody test, although PCR can also be used in the acute (< 3 days) phase of illness. Treatment is with supportive care including rehydration.

Jasamai et al. present the current treatment options for dengue fever. They discuss how supportive therapy is the mainstay of treatment as no antibody or antiviral treatments have been effective at this time. They recommend using effective preventive measures such as mosquito control in endemic areas.

Figure A is a clinical photograph demonstrating the maculopapular rash seen in dengue fever.

Incorrect Answers:
Answer A: Blood culture is used for the detection of bacterial infections of the blood. This patient's IV drug use puts him at risk for bacterial endocarditis, but he does not have other expected findings, such as new murmur, Janeway lesions, Roth spots, or Osler nodes. Endocarditis should be treated with intravenous antibiotics.

Answer B: Blood smear is the diagnostic test of choice for other mosquito-borne diseases such as malaria. It is not used in the detection of dengue. Malaria presents with cyclical fevers and organisms on blood smear. It can be treated with chloroquine and artesunate.

Answer D: Sputum with acid fast stain is the test of choice for tuberculosis. This patient has a risk factor of TB as he recently traveled to Africa, but he does not have any pulmonary symptoms. Treatment for tuberculosis is rifampin, isoniazid, pyrazinamide, and ethambutol therapy (among other possible regimens).

Answer E: Stool culture may be used to diagnose typhoid fever. Typhoid fever presents with many of the same non-specific symptoms as dengue but would also present with prominent abdominal pain and gastrointestinal symptoms. Typhoid can be treated with antibiotics such as ciprofloxacin.

Bullet Summary:
Dengue fever is characterized by fever, headache with retro-orbital pain, muscle and joint pain, rash, leukopenia and thrombocytopenia that can be diagnosed through serologic testing.


Question: A 21-year-old man presents to the emergency room with right lower quadrant pain that started this afternoon at his wrestling match. He also endorses loss of appetite and subjective fever. He has had vague, mild discomfort in the right upper quadrant since the start of this wrestling season. He endorses a history of unprotected sex with multiple partners and intravenous drug use during a recent vacation to Mexico but has no other significant medical history. He drinks socially and has never smoked. His temperature is 98.6°F (37°C), blood pressure is 119/72 mmHg, pulse is 102/min, and respirations are 12/min. Physical exam demonstrates pain on palpation of the right lower quadrant and mild pain in the right upper quadrant. He has significant acne on his face, chest, and back. A CT of the abdomen confirms appendicitis. The scan also shows a large liver mass measuring 6 cm as seen in Figure A. Aside from addressing his appendicitis, which of the following is the most appropriate next step in management?

Obtain follow-up CT of the abdomen in 3 months

Answer: Perform liver mass resection

Explanation:
This patient with a history of possible anabolic steroid use (wrestler with acne) who presents with right upper quadrant pain and an enhancing lesion in the liver most likely has a hepatic adenoma. The treatment of hepatic adenomas is surgical removal and discontinuation of precipitating agents.

Hepatic adenomas are benign epithelial tumors that typically arise in the right hepatic lobe. They are typically solitary masses but multiple lesions can be seen in patients with prolonged contraceptive or anabolic steroid use and those who have glycogen storage disorders. Patients present with abdominal pain that is localized in the epigastrium or in the right upper quadrant. Sudden, severe abdominal pain with hypotension suggests rupture of the hepatic adenoma into the peritoneum. The lesions can be confirmed on imaging such as ultrasound, CT, or MRI. For hepatic adenomas that are < 5 cm and asymptomatic, simply discontinuing the causative drug and observing are acceptable. Large or symptomatic lesions requiring surgical resection in addition to discontinuation of the offending agent. Because these tumors are highly vascular, failure to resect may result in rupture, hemoperitoneum, and shock. Biopsy is contraindicated to avoid rupturing the mass and causing hemorrhage.

Klompenhouwer et al. studied the current management strategies in patients with hepatocellular adenoma. They found that various molecular groups have been identified allowing for subtyping of these lesions. They recommend performing a CT scan to better characterize these lesions.

Figure/Illustration A is a CT with an enhancing mass with a hypoattenuating fatty component. This is characteristically seen in large hepatic adenomas, which are solitary well-marginated lesions.

Incorrect Answers:
Answer A: Metronidazole would be an effective treatment if this patient had contracted Entamoeba histolytica from contaminated food or water on his recent trip to Mexico. While this amoeba can lead to a liver abscess presenting as a mass on CT, it often also causes abdominal pain and dysentery due to intestinal involvement.

Answer B: Sofosbuvir is an antiviral medication used for hepatitis C. This patient does have risk factors for hepatitis C (risky sexual behavior and intravenous drug use). Although hepatitis C could lead to hepatocellular carcinoma and cause a liver mass, this patient’s presentation is not consistent with that diagnosis. Even if he did have hepatocellular carcinoma, treating hepatitis C alone without surgical resection or adjuvant therapies would be insufficient.

Answer C: Follow-up CT in 3 months would be dangerous for this man with a large hepatic adenoma. In the interim, the mass will likely continue to grow if he continues to use anabolic steroids, increasing the risk of rupture. Observation and repeat imaging may be suitable for asymptomatic hepatic lesions that are of uncertain but likely benign etiology.

Answer D: Biopsying the liver mass is appropriate if malignancy is suspected and there is no concern for hepatic adenoma. If an adenoma is on the differential, biopsy is contraindicated due to the risk of rupture and hemorrhage intraoperatively.

Bullet Summary:
Hepatic adenomas are benign, vascular liver masses associated with oral contraceptives and anabolic steroids that should be promptly resected if large and symptomatic.


Question: A 67-year-old man presents to the emergency room reporting sudden-onset abdominal and back pain that began 3 hours ago. He was eating dinner when he started feeling severe pain in his abdomen that made him lose his appetite. He denies any diarrhea or hematochezia and says the pain is not affected by movement. He has a history of hypertension, hyperlipidemia, and depression. He currently takes amlodipine and atorvastatin and is compliant with his medications. He has a 45-pack-year smoking history, but he does not drink alcohol or use any illicit drugs. His temperature is 98.6°F (37°C), his blood pressure is 110/64 mmHg, pulse is 97/min, and respirations are 15/min. Physical exam is notable for diffuse abdominal tenderness without rigidity or guarding. During evaluation, the patient becomes diaphoretic and pale and reports that he feels fatigued. Repeat blood pressure is 87/50 and pulse is 127. Intravenous fluid boluses are administered. Which of the following is the most likely diagnosis?

Answer: Ruptured abdominal aortic aneurysm

Explanation:
This patient who presents with sudden-onset abdominal pain, hemodynamic instability (symptomatic hypotension with tachycardia), and significant history of hypertension and smoking most likely has a ruptured abdominal aortic aneurysm (AAA).

Abdominal aortic aneurysms are dilations in the vessel wall that can predispose to rupture. Risk factors for AAA include advanced age (> 60 years), smoking history, male sex, and history of atherosclerosis. AAA can be asymptomatic or mildly symptomatic until it markedly expands or ruptures, leading to abdominal pain, back pain, or flank pain. The evaluation of suspected AAA depends on the hemodynamic stability of the patient and prior knowledge (or lack thereof) of the existence of an AAA. A hemodynamically unstable patient with a known history of AAA should be assumed to have a ruptured AAA and undergo surgical intervention without further workup (though bedside ultrasound can support the diagnosis). In an unstable patient without a known history, a bedside ultrasound to confirm the diagnosis should precede surgical intervention. In a hemodynamically stable patient with or without a known AAA history, an abdominal CTA is appropriate.

Sakalihasan et al. present a review of the presentation and treatment of abdominal aortic aneurysms. They discuss how patients are often asymptomatic until catastrophic rupture occurs. They recommend immediate surgical repair of the aneurysm during ruptures to prevent death.

Incorrect Answers
Answer A: Acute pancreatitis also presents with abdominal pain and can present with hemodynamic instability in severe cases of hemorrhagic conversion of pancreatitis. This is a rare entity and is less likely in this patient. Treatment is immediate transcatheter arterial embolization for unstable patients with hemorrhagic pancreatitis.

Answer B: Aortic dissection has similar risk factors to AAA (history of hypertension, advanced age) and also presents with acute onset back pain. Dissection normally occurs in the setting of hypertension. This diagnosis is possible but less likely in this man with a history of smoking.

Answer C: Ischemic colitis can also present with abdominal pain in a patient with risk factors for atherosclerotic disease. It is associated with hematochezia and diarrhea and is unlikely to cause the level of hypotension in this patient. Treatment is with restoration of blood flow to the ischemic bowel segment or resection if this is not possible. Management is usually centered on medical optimization.

Answer D: Perforated viscus is a surgical emergency that can also present acutely with hemodynamic instability and severe abdominal pain. However, signs of peritoneal irritation are usually present (guarding, rigidity). Treatment is with immediate surgical exploration and repair or resection.

Bullet Summary:
Patients with new-onset back and abdominal pain and a positive smoking history that progresses to hemodynamic instability should be suspected of having a ruptured abdominal aortic aneurysm.


Question: A 15-year-old girl presents to the clinic due to concern that she may be pregnant. She had unprotected sexual intercourse earlier that morning and requests emergency contraception. She has had regular menses since menarche, and her last menstrual period ended 8 days ago. She has no significant medical history. Her temperature is 98.6°F (37°C), blood pressure is 114/71 mmHg, pulse is 81/min, and respirations are 12/min. A physical exam is unremarkable. A urine pregnancy test is negative. The patient asks for an emergency contraceptive pill and would prefer that her parents not be notified about her visit. In most states, which of the following is the most appropriate next step in the management for this patient?

Obtain parental consent before providing emergency contraception

Answer: Prescribe levonorgestrel pill

Explanation:
This patient with recent unprotected intercourse and a negative pregnancy test is a candidate for postcoital emergency contraception. She should receive the levonorgestrel or ulipristal pill, which in most states does not require parental consent.

Patients with a positive pregnancy test are not candidates for emergency contraception because implantation has already occurred, but patients with recent unprotected intercourse but a negative pregnancy test should receive the levonorgestrel pill (plan B) or ulipristal pill. These pills should be administered as soon as possible after intercourse. This is because the efficacy of the pills decrease after 72-120 hours post-intercourse. The high doses of progestin in these pills delay ovulation by blocking the LH surge, thereby preventing pregnancy. In most states, adolescents can receive emergency contraception without parental consent or notification.

Batur et al. present the current options available for emergency contraception. They discuss how ulipristal acetate tends to be more efficacious compared to levonorgestrel when used more than 72 hours post-coitally. They recommend that all providers who treat young women be familiar with the options and indications for emergency contraception.

Incorrect Answers:
Answer A: It is inappropriate to not administer emergency contraception despite the patient being under 18 years old. Adolescent patients at risk of pregnancy after unprotected intercourse who request emergency contraception should be presented with viable options.

Answer B: Obtaining parental consent before providing emergency contraception is an incorrect choice in most states. Most states allow adolescents to receive confidential care for contraception and pregnancy without parental involvement, consent, or notification.

Answer C: Prescribing high-dose oral contraceptives can be done in special circumstances to delay ovulation by patients taking multiple pills at once. However, combination oral contraceptives are less commonly used for post-coital contraception as they have significant side effects at high doses and are less effective than levonorgestrel pills.

Answer E: In patients with a positive pregnancy test (after implantation has occurred), misoprostol can be used for pregnancy termination by stimulating myometrial contractions. It does not prevent pregnancy. The use of drugs to terminate pregnancy varies significantly from state to state.

Bullet Summary:
Adolescents seeking emergency contraception may do so without parental consent or involvement in most states.


Question: A 69-year-old man presents to his primary care physician with hip and back pain. He states this weekend he had a barbecue. When he was lifting a heavy object, he suddenly felt pain in his lower back. He describes the pain as in his buttocks but states that at times it travels down his leg. He states that it feels electrical and burning in nature. The patient has a medical history of obesity, diabetes, depression, anxiety, diverticulosis, constipation, and a surgical repair of his anterior cruciate ligament. His current medications include metformin, insulin, lisinopril, fluoxetine, and sodium docusate. His temperature is 98.6°F (37°C), blood pressure is 131/82 mmHg, pulse is 82/min, and respirations are 14/min. On physical exam, he is found to have an intact motor and sensory function in his lower extremities bilaterally. No clonus or Babinski sign is appreciated. An exam of the cranial nerves is intact bilaterally. Which of the following is most likely to help confirm the diagnosis?

Answer: Straight leg raise

Explanation:
This patient is presenting with lower back pain that is electrical and travels down his legs after lifting an object suggesting a diagnosis of a herniated disc. The straight leg test can help confirm the diagnosis.

A herniated disc classically presents in a middle-aged or older individual who was lifting a heavy object with improper form. They will typically experience lower back pain that can be described as radicular. The pain will typically travel down the legs or "shoot down" the legs and is typically described as feeling electrical. The straight leg test on physical exam supports the diagnosis without an MRI. When the individual raises their leg, this causes compression of the spinal nerve roots leading to an exacerbation of symptoms. The most appropriate initial step in management for these patients is limiting strenuous activity, physical therapy, and NSAIDs. Patients who have chronic symptoms that have failed conservative management can undergo surgical laminectomy.

Camino and Piuzzi present the data regarding the diagnostic value of the straight leg raise test. They discuss how this test can exacerbate patients with radicular symptoms. They recommend all practitioners learn how to perform and interpret this test.

Incorrect Answers:
Answer A: Flexion, abduction, and external rotation of the thigh is referred to as the FABER test and is used to evaluate for sacroiliitis which can present with lower back pain and pain in the buttocks. This exam maneuver will cause intense pain in patients who have inflammation of the joint. Treatment is with physical therapy and NSAIDs.

Answer B: Internal rotation of the extended hip is the Freiberg test which evaluates for piriformis syndrome. Piriformis syndrome presents with lower back pain similar to a herniated disc; however, is less likely to have occurred after a single lifting episode and does not typically present with electrical pain that originates in the buttocks.

Answer C: Palpating the paraspinal muscles is a non-specific test that could further support a diagnosis of a muscle strain. It will likely not provide much information in the diagnosis of a herniated disc. Patients with muscle strain can be treated conservatively with relative rest and NSAIDs.

Answer D: Radiography is unlikely to offer information regarding this patient's herniated disc. While it can detect bony abnormalities, the detail of this imaging modality will not depict a herniated disc. An MRI is required to offer the detail necessary to make the diagnosis and is the most accurate test for a herniated disc.

Bullet Summary:
A herniated disc typically presents with pain in the lower back that radiates down the legs and is exacerbated by the straight leg raise test.


Question: A 25-year-old man presents to his primary care physician for trouble with focus and concentration. He states that he lives at home with his parents his entire life and recently was able to get a job at a local factory. Ever since the patient started working, he has had trouble focusing on his job. He is unable to stay focused on any task. His boss often observes him "daydreaming" with a blank stare off into space. His boss will have to yell at him to startle him back to work. The patient states that he feels fatigued all the time and sometimes will suddenly fall asleep while operating equipment. He has tried going to bed early for the past month but is unable to fall asleep until 2 hours prior to his alarm. His temperature is 98.6°F (37°C), blood pressure is 112/68 mmHg, pulse is 81/min, and respirations are 12/min. A physical exam is within normal limits. The patient fears that if this continues he will lose his job. Which of the following is the most appropriate initial step in management?

Bright light therapy and decreased light at night

Answer: Bright light therapy and decreased light at night

Explanation:
This patient is presenting with fatigue and an inability to fall asleep until late in the morning suggesting a diagnosis of delayed sleep phase syndrome. The most appropriate initial therapy is light therapy, behavioral therapy, and sleep hygiene practices in addition to decreasing exposure to light at night.

Delayed sleep phase syndrome typically presents with individuals who naturally fall asleep very late and wake up very late. This becomes problematic when they attend school or have a job that requires them to wake up early. The most appropriate initial therapy for delayed sleep phase syndrome is behavioral therapy, sleep hygiene practices, and bright light therapy. Bright light therapy involves consistent timed exposure to a light box to simulate natural outdoor light, with the hope of resetting the circadian rhythm and moving the sleep phase earlier. If these measures fail, or if a different diagnosis is suspected, a referral for polysomnography may be indicated.

Sletten et al. studied whether melatonin was effective in the treatment of delayed sleep phase syndrome. They found that using this medication as an adjunct to sleep hygiene was effective in improving symptoms. They recommend using multimodal therapy for this disease.

Incorrect Answers:
Answer A: Ethosuximide is the treatment of choice for absence seizures. Though this patient does have episodes of blank staring, this likely does not represent a seizure as he is arousable to return to work by his boss. Patients with absence seizures typically present in childhood and are unable to be aroused during seizure episodes.

Answer B: Polysomnography or a sleep study would be indicated after sleep hygiene practices have failed. Polysomnography could be the most appropriate initial step in management if there was a suspected diagnosis of obstructive sleep apnea or an unknown cause of this patient's fatigue. Patients with sleep apnea typically snore and this disease is associated with obesity and increasing age.

Answer D: Modafinil is a treatment for narcolepsy. Though this patient does have sudden episodes of falling asleep while at work, this is more likely to be related to his sleep schedule. Patients with narcolepsy generally do not feel tired immediately before their episodes of sleep.

Answer E: Zolpidem is a muscle relaxant and hypnotic with the mechanism of action of a benzodiazepine. Though a drug like zolpidem could be used to help this patient fall asleep, the most appropriate first step is to address the patient's sleep hygiene prior to prescribing a potentially addictive drug.

Bullet Summary:
The most appropriate initial step in the management of patients with delayed sleep phase syndrome is behavioral therapy, bright light therapy, and sleep hygiene.


Question:
A 44-year-old woman presents to the emergency department with fluctuating right upper quadrant abdominal pain. The pain was initially a 4/10 in severity but has increased recently to a 6/10 prompting her to come in. The patient has a medical history of type 2 diabetes mellitus, depression, anxiety, and irritable bowel syndrome. Her current medications include metformin, glyburide, escitalopram, and psyllium husks. Her temperature is 99.2°F (37.3°C), pulse of 95/min, blood pressure of 135/90 mmHg, respirations of 15/min with 98% oxygen saturation on room air. On exam, the patient is an obese woman with pain upon palpation of the right upper quadrant. Initial labs are are below:

Na+: 140 mEq/L
K+: 4.0 mEq/L
Cl-: 100 mEq/L
HCO3-: 24 mEq/L
AST: 100 U/L
ALT: 110 U/L
Amylase: 30 U/L
Alkaline phosphatase: 125 U/L

Bilirubin
Total: 2.5 mg/dL
Direct: 1.8 mg/dL

The patient is sent for a right upper quadrant ultrasound demonstrating an absence of stones, no pericholecystic fluid, a normal gallbladder contour and no abnormalities noted in the common bile duct. MRCP with secretin infusion is performed demonstrating patent biliary and pancreatic ductal systems. Her lab values and clinical presentation remain unchanged 24 hours later. Which of the following is the most appropriate next step in management?

Answer: ERCP with manometry

Explanation:
This patient is presenting with classic symptoms of acute cholecystitis or choledocholithiasis such as right upper quadrant pain and direct hyperbilirubinemia with ultrasound findings demonstrating the absence of gallstones. In this setting, sphincter of Oddi dysfunction should be suspected, and ERCP with manometry should be performed to confirm the diagnosis.

Cholecystitis is inflammation of the gallbladder caused by irritation due to an intraluminal stone and possible bacterial translocation. The classic risk factors for this disease can be remembered as "fat, female, fertile and forty". Symptoms of acute cholecystitis are right upper quadrant pain that relapses and remits over time. Lab values will show elevated AST, ALT, alkaline phosphatase, and bilirubin which are also seen in patients where a stone is impacted in the common bile duct. A right upper quadrant ultrasound and MRCP can be used to confirm the diagnosis. Dysfunction of the Sphincter of Oddi should be suspected in patients with classic findings but negative imaging studies. In these cases, the most appropriate test is ERCP with manometry of the sphincter of Oddi. Ultimately, a sphincterotomy will be needed once the diagnosis is confirmed.

Coucke et al. present the current evidence for the diagnosis and treatment of biliary obstruction. They discuss how the most common etiology of biliary obstruction is choledocholithiasis or gallstones. They recommend having a high index of suspicion for cholangitis as this condition can be rapidly fatal if untreated.

Incorrect Answers:
Answer A: Analgesics and await resolution of symptoms (in addition to NPO and IV fluids) would be appropriate management for pancreatitis. The diagnosis in this patient has not yet been confirmed (given the absence of findings on ultrasound) thus prompting another diagnostic test.

Answer B: An elective cholecystectomy would be the most appropriate next step in management if the patient had cholecystitis.

Answer D: Laparoscopy is an inappropriate and invasive method of diagnosing and treating disease of the biliary tree as there are better alternatives such as MRCP or ERCP. Laparoscopy would be effective in the treatment of intestinal tract disorders such as appendicitis.

Answer E: An MRI of the abdomen would not confirm the diagnosis of sphincter of Oddi dysfunction. Though a CT scan could be performed as a more cost-effective alternative, given the absence of stones on ultrasound it is likely that a CT scan will not give more information.

Bullet Summary:
Sphincter of Oddi dysfunction can be diagnosed with ERCP and manometry.


Question: A 23-year-old man presents to the emergency department. He was brought in by police for shouting on a subway. The patient claims that little people were trying to kill him and he was acting within his rights to defend himself. The patient has a medical history of marijuana and IV drug use as well as multiple suicide attempts. He is currently homeless. While in the ED, the patient is combative and refuses a physical exam. He is given IM haloperidol and diphenhydramine. The patient is transferred to the inpatient psychiatric unit and is continued on haloperidol throughout the next week. Though he is no longer aggressive, he is seen making "armor" out of paper plates and plastic silverware to defend himself. His temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. The patient's treatment history is significant for failure to obtain symptom control with risperidone, haloperidol, and ziprasidone. Which of the following is the most appropriate next step in management?

Answer: Clozapine

Explanation:
This patient is presenting with symptoms of schizophrenia that are refractory to treatment with both typical and atypical antipsychotics. The most appropriate next step in management is treatment with clozapine.

Schizophrenia presents with auditory and/or visual hallucinations among many other findings. The most appropriate initial treatments are with a typical antipsychotic (haloperidol) or an atypical antipsychotic (risperidone). Once a patient has failed treatment with multiple medications (at least 2) from different classes, the next best step in management is clozapine. This medication is reserved for patients who have sustained treatment failure (because of the side effects of clozapine). These include weight gain, life-threatening agranulocytosis, and aplastic anemia. Regular complete blood count laboratory testing should be drawn while on clozapine to check for this side effect. Patients who develop this complication should immediately stop the medication but the side effect can be irreversible.

Khokhar et al. review the evidence regarding the use of clozapine to treat schizophrenia. They discuss how side effects of this medication include agranulocytosis, myocarditis, and seizures. They recommend using this medication with care and monitoring for the development of these complications.

Incorrect Answers:
Answer A: Chlorpromazine is a typical antipsychotic that is likely to fail given that haloperidol did not work for him. Important side effects of this medication include hypotension and sedation due to anticholinergic properties. This medication also has an idiosyncratic complication of corneal deposits.

Answer C: Fluphenazine is another typical anti-psychotic that is a strong D2 antagonist like haloperidol. If haloperidol failed, it is unlikely that another typical antipsychotic would work with such a similar mechanism of action. This medication has a high association with extrapyramidal side effects such as dystonia, akathisia, and tardive dyskinesia.

Answer D: Olanzapine is another atypical antipsychotic. Switching to another atypical antipsychotic is a viable option. Given that this patient has already failed another atypical antipsychotic and that his symptoms remain, the best therapy at this point would be clozapine. Side effects include weight gain and dyslipidemia.

Answer E: Thioridazine is a low-potency anti-psychotic that would likely not work if more potent drugs such as haloperidol or risperidone have already failed. Important side effects to be aware of include hypotension and sedation due to anticholinergic properties. This medication also has an idiosyncratic complication of retinal deposits.

Bullet Summary:
Clozapine is the treatment of choice in patients who have failed therapy with multiple different classes of antipsychotics and who have severe symptoms.


Question: A 16-year-old girl who recently immigrated to the United States from Bolivia presents to her primary care physician with a chief complaint of inattentiveness in school. The patient's teacher describes her as occasionally "daydreaming" for periods of time during where the patient does not respond or participate in school activities. Nothing has helped the patient change her behavior, including parent-teacher conferences or punishment. The patient has no other concerns. The only other concern that the patient's mother has is that, upon awakening, she notices that sometimes the patient's arm will jerk back and forth. The patient states she is not doing this intentionally. The patient has an unknown medical history and is currently not on any medications. She is not sexually active and does not intend to become pregnant in the near future. Her temperature is 98.6°F (37°C), blood pressure is 112/74 mmHg, pulse is 63/min, and respirations are 11/min. On physical exam, you note a young, healthy girl whose neurological exam is within normal limits. Which of the following is the most appropriate initial treatment?

Answer: Valproic acid

Explanation:
This patient is presenting with both absence seizures (periods of inattentiveness in school) and juvenile myoclonic epilepsy (abnormal, involuntary spasms of her upper extremity). The most appropriate treatment for these conditions is valproic acid.

Absence seizures classically present with cessation of motor activity and speech with a blank facial expression and occasionally automatisms such as flickering of the eyelids. They can be mistaken for inattentiveness in school-aged children. Juvenile myoclonic epilepsy presents with a "jerky" movement of the patient's extremity that typically occurs in the morning and begins around adolescence. The only medication that can be used to treat both absence seizures and juvenile myoclonic epilepsy is valproic acid. Valproic acid inhibits GABA transaminase, blocks thalamic T-type calcium channels, and blocks voltage-gated sodium channels. In post-pubertal female patients, it is important to counsel them on the potential teratogenic effects of this medication and also to consider alternative treatments in patients who are considering becoming pregnant or who cannot guarantee reliable birth control practices.

Kessler and McGinnis discuss the treatment of childhood absence seizures. They discuss how the treatment of choice is ethosuximide due to its favorable side effect profile. They recommend using valproic acid if there are concurrent seizure conditions.

Incorrect Answers:
Answer A: Carbamazepine would be the appropriate therapy for a partial seizure (tonic or clonic movements in addition to an absence of a post-ictal period) or for trigeminal neuralgia (sudden pain in the distribution of the trigeminal nerve). It would not address both absence seizures and juvenile myoclonic epilepsy.

Answer B: Cognitive behavioral therapy is a useful treatment that helps a patient develop coping strategies and change unhelpful patterns of thought. It could be useful if this girl was having trouble focusing in school for another reason (such as obsessive-compulsive disorder); however, it is not useful in managing seizures.

Answer C: Ethosuximide is the most appropriate initial therapy for absence seizures. In this patient, she is suffering from both absence seizures and juvenile myoclonic epilepsy which could not be treated by ethosuximide alone, in contrast to valproic acid which could treat both conditions.

Answer D: Lamotrigine is an anti-epileptic that can also be used as a mood stabilizer. It carries the potentially severe side effect of Stevens-Johnson syndrome. It is not a first-line treatment for absence seizures and juvenile myoclonic epilepsy. Stevens-Johnson syndrome will present with painful desquamation.

Bullet Summary:
Absence seizures present with periods of inattentiveness and subtle automatisms and are most appropriately treated with ethosuximide or with valproic acid if there is concurrent juvenile myoclonic epilepsy.


Question:
A 57-year-old immigrant from Nigeria presents to the emergency department for sudden, severe pain and swelling in her lower extremity. She was at a rehabilitation hospital when her symptoms started. The patient has a medical history of obesity, diabetes, bipolar disorder, and tonic-clonic seizures. Her current medications include metformin, insulin, lisinopril, and valproic acid. The patient has IV drug and alcohol use disorder and has presented to the ED many times for intoxication. Her temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. Physical exam reveals anasarca and asymmetric lower extremity swelling. Based on the results of a doppler ultrasound of her swollen lower extremity, heparin is started. The patient is then transferred to the general medicine floor for continued management. Laboratory studies are shown below.

Serum:
Na+: 137 mEq/L
K+: 5.5 mEq/L
Cl-: 100 mEq/L
HCO3-: 24 mEq/L
Urea nitrogen: 22 mg/dL
Ca2+: 5.7 mg/dL
Creatinine: 1.7 mg/dL
Glucose: 70 mg/dL

Which of the following is the most likely diagnosis?

Answer: Nephrotic syndrome

Explanation:
This patient who presents with a deep venous thrombosis (lower extremity swelling, ultrasound followed by treatment with heparin), anasarca, and asymptomatic hypocalcemia most likely has a diagnosis of nephrotic syndrome.

Nephrotic syndrome occurs when large amounts of protein are lost in the urine. This can lead to hyperlipidemia (due to loss of lipoproteins), hypercoagulable state (due to loss of antithrombin III and protein C/S), and hypoalbuminemia. Most serum calcium is bound to albumin, so when albumin is lost in the urine, it can lead to profound hypocalcemia. The ionized or free portion of calcium remains the same; therefore, these patients do not exhibit symptoms of hypocalcemia. Furthermore, the loss of albumin will reduce the oncotic pressure in the vessels resulting in massive extravasation of fluid and anasarca.

Ambler et al. report a case of a patient who presented with a DVT and was found to have nephrotic syndrome. They discuss how the patient had asymptomatic changes in lab values including calcium. They recommend performing a urine dipstick for protein in cases of suspected nephrotic syndrome.

Incorrect Answers:
Answer A: Antithrombin III deficiency is a common cause of a hypercoagulable state because antithrombin III normally helps to neutralize coagulation factors. This disease is a possible explanation for this patient's DVT as it predisposes patients to thrombotic events; however, it does not explain her profound hypocalcemia. Asymptomatic antithrombin III deficiency does not need to be treated; however, patients should have anticoagulation if they undergo surgery.

Answer B: Factor V Leiden is the most common cause of a hypercoagulable state because this mutation renders factor V unable to be inactivated by proteins C and S. This is a possible explanation for this patient's DVT; however, it does not explain her hypocalcemia. Asymptomatic factor V Leiden mutation does not need to be treated; however, patients should have anticoagulation if they undergo surgery.

Answer D: Liver dysfunction is possible given this patient's history of alcohol use and IV drug abuse, placing her at risk for chronic hepatitis C infection. Despite the anasarca, this patient has no other stigmata of liver failure such as jaundice or ascites, making nephrotic syndrome more likely.

Answer E: Prothrombin gene mutation could explain this patient's hypercoagulable state because high levels of this gene can result in unprovoked thrombotic events. This disease would not explain why the patient has anasarca or hypocalcemia. Asymptomatic patients with this mutation do not require treatment.

Bullet Summary:
Nephrotic syndrome can present with a hypercoagulable state due to loss of antithrombin III and protein C/S and hypocalcemia from loss of albumin in the urine.


Question:
A 45-year-old African American woman presents to her primary care physician for not feeling well. She states she has had a cough for the past week. She also has abdominal pain and trouble focusing that has been worsening and has lost 5 pounds recently. Her gastroesophageal reflux disease (GERD) has been poorly controlled recently. The patient is a non-smoker and has a history of GERD for which she takes antacids. Her temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. A physical exam is within normal limits. Laboratory studies are ordered and are below:

Serum:
Na+: 139 mEq/L
K+: 4.1 mEq/L
Cl-: 101 mEq/L
HCO3-: 24 mEq/L
Urea nitrogen: 12 mg/dL
Glucose: 70 mg/dL
Creatinine: 0.9 mg/dL
Ca2+: 12.5 mg/dL
Alkaline phosphatase: 150 U/L
Phosphorus: 2.0 mg/dL

Urine:
Color: amber
Nitrites: negative
Sodium: 5 mmol/24 hours
Red blood cells: 0/hpf

Which of the following is the most likely explanation of this patient's current presentation?

Answer: Increased parathyroid hormone

Explanation:
This patient is presenting with hypercalcemia and low phosphate levels suggesting a diagnosis of hyperparathyroidism.

Hyperparathyroidism can cause hypercalcemia which typically presents with "bones, stones, moans, and groans," which corresponds to symptoms of bone pain (increased bone resorption), kidney stones, GI pain, and change in mental status/trouble focusing. The increased PTH results in renal phosphate wasting, increased production of 1,25-dihydroxy vitamin D, and increased renal calcium reabsorption. It also results in increased osteoclast activity resulting in increased bone breakdown. This will lead to laboratory changes of hypercalcemia and hypophosphatemia.

Taniegra reviews the evidence regarding hyperparathyroidism. She discusses how this disease is usually discovered asymptomatically with routine blood work. She recommends surgical treatment with intractable cases as success rates are high.

Figure A is a chest radiograph that is within normal limits. No masses are seen which suggests that malignancy is a less likely diagnosis.

Incorrect Answers:
Answer A: Antacid overuse for this patient's GERD can lead to a milk-alkali syndrome which presents with hypercalcemia and metabolic alkalosis. This patient does not have metabolic alkalosis. Treatment is with the discontinuation of the offending agent.

Answer B: Increased 1,25-dihydroxy vitamin D occurs in sarcoidosis which presents with cough, fatigue, and weight loss in an African American woman. Increased vitamin D by itself will result in hypercalcemia, and hyperphosphatemia rather than hypophosphatemia. Treatment is supportive and/or with corticosteroids.

Answer D: Malignancy (lung or renal cancer) is possible given this patient's presentation. Hypercalcemia of malignancy is a common feature with PTH-related protein effects; however, the patient's normal urinalysis and normal chest radiograph as well as non-smoking history point more toward a diagnosis of increased PTH.

Answer E: Viral illness is possible given this patient's systemic symptoms; however, it does not explain this patient's hypercalcemia and hypophosphatemia. Treatment of seasonal viruses is generally supportive though specific viruses such as influenza have agents such as oseltamivir that may be helpful.

Bullet Summary:
Hyperparathyroidism presents with "bones, stones, moans, and groans," as well as laboratory findings of hypercalcemia and hypophosphatemia.


Question: A 4-year-old boy comes to the pediatrician with his mother with a 3-day history of cough and runny nose with decreased oral intake over the past 24 hours. His activity level has been normal and he has been attending school until this morning. He has no medical history or known allergies, but he has not received any of the recommended vaccines due to parental refusal. His temperature is 100.3°F (37.9°C), blood pressure is 98/62 mmHg, pulse is 85/min, respirations are 22/min, and oxygen saturation is 98% on room air. On examination, the child is fussy. Bilateral conjunctivae are injected, and there is redness on and around the tonsils. There is no lymphadenopathy. Faint rhonchi are heard at the lung bases bilaterally. Which of the following is the most appropriate next step in management?

Obtain chest radiography and respiratory virus panel

Answer: Supportive care with return precautions

Explanation:
This boy presenting with 3 days of progressive fever, rhinorrhea, cough, conjunctivitis, and tonsilar erythema most likely has viral pharyngitis. The appropriate treatment for this is supportive care with return precautions (instructing the mother to bring the child back to a physician if he becomes short of breath).

Viral pharyngitis in children is most often caused by adenovirus, though other viruses can cause the condition as well. The diagnosis is clinical, with a presentation of fever, rhinorrhea, conjunctivitis, sore throat, and cough. Features that can help distinguish viral pharyngitis from pneumonia include a lack of tachypnea, increased work of breathing, hypoxemia, or adventitial lung sounds. No additional testing or intervention is necessary. The illness is typically self-limited and resolves with supportive care.

Shapiro et al. study features predictive of group A streptococcal (GAS) pharyngitis in children. The authors found that among patients with features of viral illness, patients older than 11 years of age who did not have a fever, cervical lymphadenopathy, or tonsillar exudates were at the lowest risk for GAS. The authors recommend against testing low-risk children for GAS to avoid antibiotic treatment in patients merely colonized with GAS.

Incorrect Answers:
Answer A: Administering antitoxin and toxoid vaccine is the treatment for Corynebacterium diphtheriae infection. Although this child has not received his age-appropriate vaccinations, his presentation is not concerning for infection with diphtheria. C. diphtheriae infection presents with cervical lymphadenopathy and gray pseudomembranes on pharyngeal exam, neither of which this child has.

Answer B: Amoxicillin should be given in cases of group A streptococcal (GAS) pharyngitis. Before amoxicillin is prescribed, testing with Streptococcal rapid antigen detection testing should be performed. Additionally, rapid antigen detection testing is not indicated in this patient as she has a cough and does not have cervical lymphadenopathy, tonsillar exudates, or palatal petechiae. All of these findings are not typical of GAS pharyngitis.

Answer C: Influenza testing and oseltamivir are indicated if the child had myalgias or high fevers that would make influenza more likely and if he presented within 2 days of symptom onset. Oseltamivir is only approved for treating influenza in uncomplicated cases if started within this 2-day window from symptom onset. Exceptions to this 2-day guideline include adults greater than 65 years of age, pregnant women, residents of long-term care facilities, American Indians, persons with BMI greater than 40 kg/m^2, and those receiving immunosuppressive medications.

Answer D: Obtaining a chest radiograph and respiratory virus panel can be done when a lower respiratory infection such as bacterial pneumonia is expected. However, viral infections are much more common than bacterial pneumonia in this age group. Additionally, this patient is not tachypneic, does not have evidence of increased work of breathing, and is not hypoxemic, all of which make bacterial pneumonia less likely.

Bullet Summary:
Viral pharyngitis in children is a clinical diagnosis consisting of fever, rhinorrhea, conjunctivitis, cough, sore throat, and pharyngeal erythema, and is treated with supportive care.


Question: A 23-year-old woman presents to the emergency department with severe abdominal pain. The pain has been dull and progressive, but became suddenly worse while she was exercising. Her medical history is notable for depression, anxiety, and gonococcal urethritis that was appropriately treated. She is sexually active and does not use condoms. She admits to drinking at least 5 standard alcoholic drinks a day. The patient also recently lost a large amount of weight for a fitness show she planned on entering. The patient's current medications include oral contraceptive pills, fluoxetine, alprazolam, ibuprofen, acetaminophen, and folate. Her temperature is 99.5°F (37.5°C), blood pressure is 80/40 mmHg, pulse is 110/minute, and respirations are 15/minute with an oxygen saturation of 96% on room air. On physical exam, you note an athletic young woman with burly shoulders, a thick neck, and acne on her forehead and back. On abdominal exam you note diffuse tenderness with 10/10 pain upon palpation of the right upper quadrant. Intravenous fluids are started and labs are sent. A urinary ß-hCG has been ordered. Which of the following is the most likely the diagnosis?

Obstruction of the common bile duct by radio-opaque stones

Answer: Vascular ectasia within the liver

Explanation:
This patient is presenting with constant right upper quadrant pain in the setting of oral contraceptives (OCP) and likely anabolic steroid use, suggesting a diagnosis of a ruptured hepatic adenoma (HA). The pathophysiology of HA is vascular ectasia from exposure to OCPs and androgens.

A hepatic adenoma typically occurs secondary to exposure to OCPs and androgens (anabolic steroids) causing a generalized vascular ectasia. Both of these drugs should be discontinued in patients with hepatic adenoma. Symptoms include right upper quadrant abdominal pain. Sudden worsening of pain with unstable vitals suggests a ruptured hepatic adenoma. Patients should be managed with fluids and an ultrasound should be performed to support the diagnosis. Additional imaging tests include an abdominal contrast-enhanced CT to better characterize the pathologic anatomy in this disease. The definitive treatment in this disease is surgery in order to remove the hepatic adenoma. In cases of a ruptured hepatic adenomas, patients require transfusion and operative management.

Bioulac-Sage et al. present the current data that are available for classifying hepatic adenomas. They discuss how many of these lesions involve alterations of the HNF1A gene, the JAK/STAT3 pathway, and the CTNNB1 gene. They recommend performing molecular analysis in order to better understand which lesions are likely to undergo malignant transformation.

Incorrect Answers:
Answer A: Ectopic implantation of a blastocyst (ectopic pregnancy) that has ruptured could present similarly to what this patient is experiencing. Symptoms would include lower abdominal pain and unstable vitals. However, this patient's pain is more pronounced in the right upper quadrant and is occurring in the setting of OCP and possible anabolic steroid use.

Answer B: Inflammation of the pancreas represents a diagnosis of pancreatitis. This would present with epigastric pain after a fatty meal or alcohol consumption. Though this patient does drink more than recommended and should be counseled to reduce her consumption, the pain began after exercising at the gym and there are no other symptoms of pancreatitis.

Answer C: Obstruction of blood flow through the hepatic vein causes Budd-Chiari syndrome, which presents with a non-specific triad of abdominal pain, ascites, and hepatomegaly in the setting of a hypercoagulable state (factor V Leiden, polycythemia vera, OCP use, and paroxysmal nocturnal hemoglobinuria). Though this patient has risk factors for this pathology, they do not have any classic symptoms.

Answer D: Obstruction of the common bile duct with radio-opaque stones would present with colicky right upper quadrant pain in a patient that is "fat, female, fertile, and forty." Though the pain presented in this case is localized to the right upper quadrant, it is not colicky and there is not a positive Murphy sign described. OCP's are a risk factor for cholesterol stones as are rapid weight loss, both of which this patient has. This is a strong possibility on the differential; however, the symptoms presented suggest a diagnosis of ruptured hepatic adenoma given the demographics of the patient.

Bullet Summary:
A hepatic adenoma presents with right upper quadrant pain in the setting of OCP and/or androgen use and can rupture leading to unstable vitals.


Question: A 65-year-old man presents to his primary care physician for a routine appointment. He has no concerns. His past medical history is significant for human immunodeficiency virus (HIV) infection, diagnosed 15 years ago. The patient is intermittently compliant with his antiretroviral therapy (ART). His temperature is 99.0°F (37.2°C), blood pressure is 130/84 mmHg, pulse is 92/min, and respirations are 11/min. His most recent lab work showed a cluster of differentiation CD4 count of 150 cells/µL. He received a dose of the 13-valent pneumococcal vaccine and a dose of the 23-valent pneumococcal vaccine 15 years ago, as well as a second dose of the 23-valent pneumococcal vaccine 10 years ago. In addition to encouraging greater compliance with his ART, which of the following is indicated in this patient?

Trimethoprim-sulfamethoxazole and varicella vaccine

Trimethoprim-sulfamethoxazole and zoster vaccine

Trimethoprim-sulfamethoxazole and pneumococcal vaccine

Answer: Trimethoprim-sulfamethoxazole and pneumococcal vaccine

Explanation:
This patient with HIV and a CD4 count of 150 cells/μL requires trimethoprim-sulfamethoxazole (TMP-SMX) prophylaxis and another dose of the 23-valent pneumococcal vaccine.

All HIV patients with a CD4 count < 200 cells/μL require TMP-SMX as prophylaxis against Pneumocystis jirovecci pneumonia. Patients with CD4 counts < 100 cells/μL also require TMP-SMX as prophylaxis against toxoplasmosis. HIV patients require a dose of the 23-valent pneumococcal vaccine at age 65. Patients should initially receive the 13-valent pneumococcal vaccine, followed by the 23-valent pneumococcal vaccine 8 weeks later, 5 years later, and at age 65. Compliance with ART is also critical in patients with HIV.

Kaplan et al. discuss recommendations for the prevention of opportunistic infections in HIV-infected adults. The authors find that daily prophylaxis with TMP-SMX is an appropriate regimen for the prevention of Pneumocystis pneumonia. The authors recommend dapsone, atovaquone, or pentamidine for patients that cannot receive TMP-SMX.

Incorrect Answers:
Answer A: Azithromycin is used as prophylaxis against Mycobacterium avium complex (MAC) in patients with a CD4 count < 50 cells/μL that has not been started on ART. Since this patient has a CD4 count of 150 cells/μL, azithromycin is not indicated at this time.

Answer B: Azithromycin is used as prophylaxis against Mycobacterium avium complex (MAC) in patients with a CD4 count < 50 cells/μL that has not been started on ART. This patient should not receive the zoster vaccine as it is a live vaccine and is contraindicated in immunocompromised patients.

Answer D: Although this patient does require TMP-SMX as prophylaxis against Pneumocystis pneumonia, he should not receive the varicella vaccine as it is a live vaccine and therefore contraindicated in immunocompromised patients.

Answer E: Although this patient does require TMP-SMX as prophylaxis against Pneumocystis pneumonia, he should not receive the zoster vaccine as it is a live vaccine and therefore contraindicated in immunocompromised patients.

Bullet Summary:
Patients with a CD4 count < 200 cells/μL require prophylaxis with TMP-SMX and all patients with HIV should receive a dose of the 23-valent pneumococcal vaccine at age 65.


Question: An 82-year-old man with a history of atrial fibrillation presents to the emergency room complaining of sudden-onset, painless vision loss in his right eye starting 2 days ago. When it started, he felt like a “curtain was pulled down” over his right eye but wasn't able to seek care immediately because it was snowing outside and he didn't want to travel in inclement weather. A review of systems is significant for occasional palpitations and presyncope. The patient’s medications include aspirin and carvedilol. He reports that he is a smoker with a 50-pack-year history. The patient’s temperature is 99°F (37.2°C), blood pressure is 112/72 mmHg, pulse is 84/min, and respirations are 14/min with an oxygen saturation of 97% on room air. Physical exam reveals 20/800 vision in the right eye. A fundoscopic exam of the right eye is shown in Figure A. Physical and fundoscopic examinations of the left eye are unremarkable. Which of the following is the most appropriate next step in management?

Continue aspirin and start statin therapy

Answer: Continue aspirin and start statin therapy

Explanation:
This patient with a history of atrial fibrillation is presenting with acute-onset, painless, monocular vision loss and a fundoscopic exam positive for retinal whitening. This suggests a diagnosis of central retinal artery occlusion and management should focus on control of underlying risk factors given that he is 2 days out from the event.

Symptoms of central retinal artery occlusion (CRAO) include sudden, painless, complete, and normally unilateral vision loss. A fundoscopic exam can show pallor of the optic disc, diffuse ischemic whitening of the retina, cherry-red macula, retinal edema, and constricted/bloodless retinal arterioles. The center of the macula appears red because the photoreceptor layer at the fovea is perfused by the posterior choroidal vasculature rather than the central retinal artery. Treatment involves surgical decompression of the retinal anterior chamber, ideally within 1 hour of the occlusion. Tissue plasminogen activator should be administered if the patient presents within an acceptable time frame. If surgical intervention is not readily available, additional steps with minimal evidence to support their use include ocular massage (to dislodge the embolus) and either carbogen therapy or hyperbaric oxygen. Chronic management includes control of underlying risk factors with aspirin and statin therapy.

Dattilo et al. review the evidence regarding the treatment of central retinal artery occlusion. They discuss how treatments have not been particularly effective in the treatment of this disorder. They recommend engaging in secondary prevention of vascular events by controlling risk factors.

Figure/Illustration A is a fundoscopic photograph showing retinal pallor surrounding the superior temporal arcade (red circle). This finding is characteristically seen in central retinal artery occlusion.

Incorrect Answers:
Answer A: Administering ranibizumab is not appropriate because this medication is an anti-VEGF antibody, which can be used to treat wet age-related macular degeneration. Symptoms of wet age-related macular degeneration include acute visual distortion and loss of central vision that occurs over months. A fundoscopic exam would show neovascularization.

Answer B: Administration of tissue plasma activator has not been shown to be beneficial in central retinal artery occlusion if the event has occurred several days ago. This medication is used in patients who sustain an ischemic stroke within 4.5 hours of developing symptoms. A stroke in the posterior lobe can present with cortical hemianopsia.

Answer D: Retinal photocoagulation is used to treat diabetic retinopathy. In diabetic retinopathy, symptoms of painless vision loss present gradually. A retinal exam would show retinal hemorrhage and possibly neovascularization.

Answer E: Timolol is a beta-blocker used to treat glaucoma. Assessment of the optic disk would show an enlarged cup-to-disk ratio. Glaucoma can present with pain and pressure in the affected eye. Acute angle closure glaucoma would present with a rock-hard eye and may lead to permanent vision loss if not treated promptly.

Bullet Summary:
Chronic management of central retinal artery occlusion involves control of underlying risk factors with aspirin and statin therapy.