Question 1

A 9-year-old boy is brought to the emergency department by his babysitter after "passing out" at home.  The babysitter says, "He was playing video games and I noticed his eyes started blinking a lot.  He did not respond when I called his name and then he slumped to the floor.  He has been very sleepy since waking up."  The boy had been well, with no fevers, upper respiratory symptoms, or vomiting.  He takes carbamazepine for epilepsy and has not had a seizure in years.  He also takes loratadine as needed for allergic rhinitis.  The patient has no known drug allergies and his immunizations are up to date.  It has been a stressful time for him as his parents are going through a divorce and he has been splitting time between 2 homes.  The boy also just returned from a week-long stay at his grandparents' house.  Vital signs are normal.  On physical examination, the patient is drowsy but able to answer questions and follow directions.  Pupils are 4 mm bilaterally and equally reactive to light.  Cardiopulmonary examination is normal.  The abdomen is soft and nontender without hepatosplenomegaly.  Muscle tone and strength are normal in all extremities.  Plantar reflexes are downgoing.  There are no skin rashes or lesions.  Which of the following is the best next step in evaluation of this patient?

Accepted Answer

Explanation: This patient has had a breakthrough seizure, a seizure that occurred despite previously successful antiepileptic therapy. Subtherapeutic drug levels are a common cause of breakthrough seizures in children with known epilepsy. Common causes for subtherapeutic drug levels include altered drug metabolism (eg, phenytoin), increasing patient weight, and nonadherence to the therapeutic regimen. In this case, the most likely cause is medication nonadherence, which often occurs in patients with psychosocial stressors (eg, divorce, multiple third-party caregivers). Therefore, measuring a serum carbamazepine level would be the best next step in evaluation of this patient. If levels are subtherapeutic, adherence improves when providers discuss problem solving and sources of stress or fears and provide additional education regarding epilepsy. Risk factors for nonadherence include family communication issues, complex medical regimens, and depressed socioeconomic status (eg, inability to afford medications, unreliable transportation). If the serum carbamazepine levels are therapeutic, additional workup such as follow-up electroencephalography and serum electrolyte evaluation would be indicated. (Choice A) Carbamazepine can cause fulminant hepatic failure with resulting hyperammonemia, hepatic encephalopathy, and seizure. This patient's normal behavior prior to the seizure and lack of vomiting or asterixis make hepatic failure unlikely; therefore, serum ammonia level and liver function tests are not indicated. (Choice B) Inborn errors of metabolism often present with seizure but typically at a younger age (eg, infancy). When metabolic disorders present with seizures, the seizures typically are not controlled until the metabolic error is corrected. (Choice D) CT scan is indicated if the patient exhibits pre-seizure altered mental status or an abnormal neurologic examination. This patient, however, exhibits equal and reactive pupils, normal reflexes, and normal behavior prior to the seizure; therefore, an intracerebral process (eg, tumor, hematoma) is unlikely. (Choice E) An ECG would be appropriate evaluation for syncope but would yield little information toward a diagnosis of partial seizure with secondary generalization. This patient exhibits post-event somnolence, suggesting a seizure rather than a syncopal event. Educational objective: Nonadherence to medications is a major cause of breakthrough seizures in patients with epilepsy. Prompt measurement of serum antiepileptic drug concentrations is an important part of the initial workup for these patients. References: Behavioral predictors of medication adherence trajectories among youth with newly diagnosed epilepsy. (http://www.ncbi.nlm.nih.gov/pubmed/26209946) Patterns of nonadherence to antiepileptic drug therapy in children with newly diagnosed epilepsy. (http://www.ncbi.nlm.nih.gov/pubmed/21521848)

Question 2

A 45-year-old Caucasian man comes to see you in the office. He is accompanied by his wife. They have noticed that he has shakiness of both hands for the past six months. The shakiness is limited only to his hands and has not progressed over time. It becomes much more pronounced when he tries to pick up things or attempts to eat or drink. On physical examination, you notice a constant tremor in both his hands, which becomes much more pronounced when his arms are outstretched. There is also a very subtle head tremor present. There is no rigidity or slowing of voluntary movements in the upper or lower extremities. His father had a similar problem in his 50s or 60s, but he is not sure what treatment he received at that time. His wife is extremely anxious and wants to know his prognosis. Item 1 of 2 Which of the following is the most appropriate response?

Accepted Answer

Explanation: The patient in the above vignette is most likely suffering from a familial tremor or benign essential tremor. It is the most common cause of postural tremor, and its incidence usually increases with age. The familial cases usually have an autosomal dominant inheritance pattern and presents at a relatively younger age. The tremor in patients with familial disease is usually present in the distal upper extremities and becomes much more pronounced with outstretching of the arm. It also increases at the end of an activity or movement. All patients with essential tremor typically do not have any other neurological signs. The absence of a resting tremor (which worsens at rest but improves with voluntary activity), rigidity (increased resistance to passive movements), bradykinesia (slowing of voluntary movements), or gait difficulty differentiates this condition from an early onset of Parkinson's disease. The presence of a positive family history and a head tremor is also more suggestive of an essential tremor. Benign essential or familial tremors usually do not cause any significant disability, and the patient can expect to have a normal life expectancy. There are no associated neurological symptoms in patients with benign essential tremors. (Choice B)The tremor in patients with benign essential or familial tremors usually worsens with time and can cause difficulty in the performance of fine motor tasks. (Choice C) The tremor can remain stable for years without any significant deterioration. It can cause significant disability in some, but not all the patients. (Choice D) Medications, especially beta-blockers, only have a limited effect on symptom control. They do not cause a complete resolution of the symptoms. Educational Objective: Benign essential tremors or familial tremors generally do not cause significant disabilities or neurological problems in patients.

Question 3

A 55-year-old woman comes to the emergency department with progressively worsening severe back pain and difficulty walking for the last 2 weeks.  The pain is at the mid-thoracic level and wraps around her upper abdomen in a band-like fashion.  She also has weakness, numbness, and tingling in both lower extremities.  No bowel or bladder dysfunction is reported.  The patient completed treatment for non-Hodgkin's lymphoma 8 months ago with chemotherapy and radiation therapy.  On physical examination, her temperature is 36.7 C (98 F), blood pressure is 110/80 mm Hg, and pulse is 94/min.  Neurological examination shows motor strength of 3/5 in both lower extremities.  The patellar and Achilles reflexes are brisk and Babinski reflex is upgoing bilaterally.  Which of the following are the most appropriate next steps in management of this patient?

Accepted Answer

Explanation: Cancers that commonly metastasize to the spinal column include prostate, breast, lung, non-Hodgkin's lymphoma, and renal cell carcinoma. This patient with recent history of non-Hodgkin's lymphoma has classic thoracic radicular pain (wraps around the abdomen) accompanied by weakness and sensory changes in the lower extremities consistent with epidural spinal cord compression (ESCC). Bowel or bladder dysfunction usually develops late in the disease but may present early in those with compression at the level of conus medullaris. epidural spinal cord compression Cord compression is a medical emergency as long-term prognosis is determined by pretreatment neurologic status. High-dose corticosteroids (especially dexamethasone) should be administered immediately to decrease edema and swelling around tumor tissue. Early steroid administration has shown better neurologic outcomes. MRI of the spine should be obtained to confirm the diagnosis. It is also needed to assess spinal stability and plan definitive therapy. Generally, patients with an unstable spine and radioresistant tumors should undergo urgent surgical evaluation for decompression. Patients with a stable spine and minimal neurologic symptoms or those with highly radiosensitive tumors, such as lymphoma, may be treated with radiotherapy alone. (Choice A) Cerebrospinal fluid evaluation may be needed in patients with myelopathy of undetermined etiology. An MRI is required to diagnose structural abnormalities before a lumbar puncture is performed, particularly in patients with a history of malignancy. (Choices C, D, and E) Steroids are crucial in the early management of ESCC and should be given empirically. Intravenous antibiotics would be indicated for infection, such as an epidural abscess, that may produce similar symptoms. However, in the setting of malignancy and no signs of infection, neoplastic ESCC is more likely. The need for surgical evaluation is determined by findings on MRI and radioresistance of the tumor. Educational objective: Suspected epidural spinal cord compression should be treated as an emergency with empiric high-dose corticosteroids. An MRI should be ordered to confirm the diagnosis and assess spine stability. Patients with highly radiosensitive tumors may be treated with radiation. Patients with radioresistant tumors and those with an unstable spine should undergo surgical evaluation. References: Neoplastic myelopathy. (http://www.ncbi.nlm.nih.gov/pubmed/22961188) A 2011 updated systematic review and clinical practice guideline for the management of malignant extradural spinal cord compression. (http://www.ncbi.nlm.nih.gov/pubmed/22420969) Direct decompressive surgery followed by radiotherapy versus radiotherapy alone for metastatic epidural spinal cord compression: a meta-analysis. (http://www.ncbi.nlm.nih.gov/pubmed/24503688)

Question 4

A 32-year-old man comes to the office due to right-sided facial weakness for 3 days. He has had no fever, headache, vision disturbances, or extremity weakness or numbness but has been extremely fatigued over the past several months. The patient has no prior medical conditions and takes no medications. He drinks alcohol occasionally and does not use tobacco or illicit drugs. The patient lives in coastal California and has not traveled recently. Vital signs are within normal limits. Physical examination reveals right-sided facial droop with flattening of the nasolabial fold. He is unable to elevate the right eyebrow or close the right eye completely. Upper and lower extremity muscle strength, deep tendon reflexes, and sensation are normal on both sides. There is no neck rigidity. The lungs are clear on auscultation, and heart sounds are normal. Hepatosplenomegaly is present. The cervical, axillary, and inguinal lymph nodes are multiple, firm, and nontender. No skin rash or extremity edema is present. The joints are not swollen or tender. Item 1 of 2 Which of the following is the best initial test for this patient?

Accepted Answer

Explanation: 11ec2b69_c002_76c3_8f63_3de048ff658a_MD0005_00 This young patient's unilateral facial nerve palsy, hepatomegaly, and lymphadenopathy raise suspicion for extrapulmonary sarcoidosis. Sarcoidosis is a multisystem granulomatous disease of unknown origin that primarily affects young adults. Most cases involve the lungs, but extrapulmonary manifestations frequently (~30%) occur and can be the presenting feature. The following sites are commonly involved: Skin: papular, nodular, or plaque-like lesions or erythema nodosum Eyes: uveitis and keratoconjunctivitis sicca Joints: acute polyarthritis Nervous system: facial nerve palsy Reticuloendothelial system: hepatomegaly (~20%) and peripheral lymphadenopathy (~40%) Extrapulmonary sarcoidosis is almost always accompanied by significant fatigue. Screening for suspected cases usually begins with a chest x-ray because >90% of patients have bilateral hilar or mediastinal adenopathy. Additional testing is then typically required for confirmation. (Choice A) Herpes simplex meningitis is often associated with focal neurologic findings (eg, cranial nerve deficits) and is usually diagnosed with cerebrospinal fluid PCR. However, most patients have fever (>90%) and altered mental status. Peripheral lymphadenopathy and hepatomegaly would be atypical. (Choice C) Early disseminated Lyme disease can cause facial nerve palsy (~8%) and is usually diagnosed with Lyme serology. Flu-like symptoms and lymphadenopathy can also occur. However, this patient lives in coastal California, where Lyme disease is less prevalent, and hepatosplenomegaly is not a typical feature of Lyme disease. (Choice D) Patients with facial nerve palsy should undergo neuroimaging if they have red flag symptoms (eg, sparing of the upper face, associated hearing loss, associated facial twitching) or if their condition continues to worsen after 3 weeks or fails to resolve by 4 months. This patient with upper and lower facial weakness, peripheral lymphadenopathy, and hepatomegaly is less likely to have a CNS lesion. (Choice E) Patients with CNS tuberculosis can develop cranial nerve palsies and fatigue. However, the majority of cases are associated with 2-3 weeks of prodromal symptoms (eg, headache, low-grade fever, personality changes) and prominent meningeal signs (eg, nuchal rigidity). Educational objective: Sarcoidosis is a multisystem granulomatous disease. The lungs are most commonly affected, but extrapulmonary manifestations of the skin (eg, erythema nodosum), eyes (eg, uveitis), joints (eg, acute polyarthritis), nervous system (eg, facial nerve palsy), and reticuloendothelial system (eg, hepatosplenomegaly, lymphadenopathy) commonly occur. Chest x-ray is often the first screening test because >90% of patients have bilateral hilar or mediastinal adenopathy. References: Extrapulmonary manifestations of sarcoidosis. (http://www.ncbi.nlm.nih.gov/pubmed/23597964) Neurologic manifestations of sarcoidosis. (http://www.ncbi.nlm.nih.gov/pubmed/24365304)

Question 5

A 79-year-old man comes to the physician with his daughter for memory loss over the last 8-12 months.  She initially noticed that he had trouble with forgetfulness, followed by distractibility and difficulty with word finding and object recognition.  The patient is currently dependent on her for most independent activities of daily living, including transportation and finances.  He is able to dress, bathe, and toilet himself.  His gait has been unstable, and he sometimes seems to get "stuck in a chair."  On review of systems, he notes urinary urgency, frequency, and incontinence.  He has a history of benign prostatic hyperplasia for which he takes tamsulosin.  The patient has no tremor, problems with sleep, weakness, hallucinations, or sensory disturbances.  On examination, his vital signs are normal.  He is oriented to self only.  On mental status examination, he has impairments in language, visuospatial skills, and executive function.  His cranial nerves are intact.  There is normal bulk and tone of the musculature.  His gait is wide based and magnetic.  He has decreased stride length and a slow turn requiring several steps.  Laboratory results, including TSH and vitamin B12, are normal.  Brain imaging is shown in the image below.  Which of the following is the most appropriate next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_c000_7af0_8f63_c175a900124d_MD0005_00 This patient's presentation is consistent with a likely diagnosis of normal-pressure hydrocephalus (NPH). NPH is characterized by the classic triad of gait disturbance followed by impaired cognition and urinary incontinence. Gait is usually wide based and can be described as being magnetic due to the appearance of the feet being stuck to the floor. Cognitive impairment usually follows gait dysfunction and can include early psychomotor slowing and problems with executive function (eg, financial planning). Urinary incontinence is typically a late manifestation and should raise suspicion in this patient as he is already on treatment for benign prostatic hyperplasia. Typical findings on brain imaging include ventriculomegaly out of proportion to sulcal enlargement and the absence of an obstruction. Patients with a supportive history and ventriculomegaly on neuroimaging should first undergo lumbar drainage of cerebrospinal fluid (CSF). The lumbar tap test (Miller Fisher test) evaluates parameters such as gait speed, stride length, verbal memory, and visual attention before and after removal of 30-50 mL of CSF. Patients with improvement in any of the parameters have a high probability of responding to definitive treatment with ventriculoperitoneal (VP) shunt placement (Choice E). If there is no improvement with CSF drainage, VP shunting may not be very helpful. (Choice A) There is limited data on the efficacy of acetazolamide in the treatment of NPH and it is not currently recommended. (Choice B) Carbidopa-levodopa can be used to treat Parkinson disease, which is characterized by bradykinesia, resting tremor, and postural instability. Although this patient has a magnetic gait and slow movement, his lack of tremor or rigidity on neurologic examination and the presence of dilated ventricles on MRI make NPH more likely. (Choice C) Donepezil is an acetylcholinesterase inhibitor that can improve cognitive function and activities of daily living in patients with various forms of dementia (eg, Alzheimer disease, vascular dementia, dementia with Lewy bodies). There are no major studies evaluating the efficacy of donepezil in the treatment of NPH, the primary treatment of which is VP shunting. Educational objective: Normal-pressure hydrocephalus typically presents with the classic triad of gait disturbance followed by impaired cognition and urinary incontinence. Neuroimaging with MRI should demonstrate ventriculomegaly out of proportion to sulcal enlargement and the absence of an obstruction. Lumbar drainage of cerebrospinal fluid with pre and post assessment of gait and cognition is helpful in predicting the effectiveness of ventriculoperitoneal shunting. References: Diagnosis and management of idiopathic normal-pressure hydrocephalus. (http://www.ncbi.nlm.nih.gov/pubmed/24175154)

Question 6

A 54-year-old homeless man is brought to the emergency department by the police after he was found to be confused and disheveled.  The patient is unable to provide a medical history and has no prior hospital record.  Temperature is 36.5 C (97.7 F), blood pressure is 110/60 mm Hg, pulse is 90/min, and respirations are 16/min.  The patient is thin and appears ill, with temporal muscle wasting.  The mucous membranes are dry.  The lungs are clear to auscultation, and heart sounds are normal with no murmur.  The abdomen is soft, nontender, and nondistended.  The patient is disoriented and unable to recognize where he is or state the date.  Bilateral pupils are equal and reactive to light, but extraocular motion is restricted on leftward gaze.  No obvious motor weakness is present.  Deep tendon reflexes are reduced and plantar responses are flexor.  The patient has no neck rigidity, but his gait is markedly ataxic.  Laboratory results are as follows:  With treatment, the patient's gait abnormalities and eye movement gradually improve.  He becomes more oriented, but the nurses report that he is forgetful and tells incongruent stories that seem to have never happened.  Neuroimaging is most likely to reveal abnormalities in which of the following areas of this patient's brain?

Accepted Answer

Explanation: Korsakoff syndrome (KS) is a late-stage complication of chronic thiamine deficiency due to repeated or prolonged episodes of Wernicke encephalopathy (WE). Up to 80% of patients recovering from an acute episode of WE show signs of KS, including significant retrograde and anterograde amnesia, often with confabulation. Cognition, attention, social behavior, and long-term memory are relatively preserved. Patients with KS typically have mammillary body atrophy on MRI of the brain. Unlike WE, the neurocognitive changes of KS rarely improve; most patients require long-term supervision and social support. (Choice A) The basal ganglia is important for voluntary movement, procedural learning, and routine behaviors (eg, teeth grinding). Parkinson disease primarily affects the basal ganglia and is associated with "pill-rolling" tremor, bradykinesia, and cogwheel rigidity. (Choice B) Damage to the corpus callosum can result in problems with learning, hand-eye coordination, and visual/auditory memory. This patient, who developed memory impairment and confabulation after treatment for WE, likely has KS. (Choice C) The frontal lobes contribute to voluntary movements and higher-level cognition that integrates emotion with memory. Although patients with frontal lobe damage may occasionally confabulate, this patient, who developed symptoms shortly after recovering from WE, is far more likely to have KS. (Choice D) The internal capsule is composed largely of the corticospinal tract, which brings information from the primary motor cortex to the lower motor neurons in the spinal cord. Damage to this area typically results in contralateral hemiparesis or hemiplegia. Educational objective: Patients with Korsakoff syndrome often have MRI evidence of mammillary body atrophy, a relatively specific finding for this condition.

Question 7

A 24-year-old woman comes to the physician with 2 days of progressive leg weakness and paresthesias.  Today, she could not void urine.  She has no significant medical history, except for an upper respiratory tract infection 2 weeks ago.  The patient takes no medications and does not use illicit drugs.  Her blood pressure is 110/70 mm Hg and pulse is 80/min.  Physical examination shows bilateral lower-extremity weakness, decreased reflexes, and decreased pain sensation up to the level of the umbilicus.  Sensations are intact in the dermatomes above the umbilicus.  Bladder catheterization shows a volume of 500 mL.  Which of the following is the best next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_c009_f0dd_8f63_7111c297128b_MD0005_00 This patient has a rapidly developing myelopathy as evidenced by flaccid paralysis and paresthesias, along with a clearly demarcated sensory level at the umbilicus. Flaccid paralysis and hyporeflexia with a history of upper respiratory infection (URI) may also suggest Guillain-Barré syndrome (GBS). However, a sensory level and bowel/bladder dysfunction after a URI make transverse myelitis (TM) more likely. Flaccid paralysis and hyporeflexia are common in early stages of myelopathy (spinal shock). Guillain-Barré syndrome (GBS) MRI is necessary to diagnose the cause of acute myelopathy. In the absence of overt trauma or history of malignancy, compressive lesions such as tumor, herniated intervertebral disk, and epidural abscess are less likely but must still be ruled out. In addition, MRI will show enhancement of the affected spinal cord regions with gadolinium in TM. Three to five days of high-dose corticosteroids should be given once TM is diagnosed. Empiric steroids may be given if there is high suspicion of compressive myelopathy in the setting of malignancy. (Choice A) MRI provides better definition of neuronal tissue, disk, ligaments, epidural fat, and shape and size of the spinal canal. CT scan is better for detection of bony abnormalities. (Choice B) A lumbar puncture should be performed to identify the cause of noncompressive myelopathy. Cerebrospinal fluid (CSF) analysis may be helpful in differentiating idiopathic from secondary TM. (Choice D) Nerve conduction studies are useful in evaluating peripheral nerve disorders such as GBS or carpal tunnel syndrome. They can differentiate among axonal disease, demyelinating disorders, and conduction defects. They do not provide any definitive diagnostic or prognostic information in myelopathy. (Choice E) Plain x-ray of the vertebrae is indicated only in patients with low back pain without neurologic deficits and who are at high risk of vertebral fractures (eg, trauma, prolonged glucocorticoid use, osteoporosis, age >70). Educational objective: Acute paralysis with the presence of a sensory level or bowel/bladder dysfunction suggests acute myelopathy. These patients should be evaluated immediately with MRI. Steroids should be administered after the diagnosis is established with MRI or empirically if suspicion for compressive myelopathy is high, as in malignancy. References: Myelopathy. (http://www.ncbi.nlm.nih.gov/pubmed/18477657) Acute inflammatory myelopathies. (http://www.ncbi.nlm.nih.gov/pubmed/24507538)

Question 8

A 14-year-old girl with a history of juvenile myoclonic epilepsy is brought to the emergency department after 2 generalized tonic-clonic seizures that started approximately 1 hour ago.  She has not returned to baseline between seizures.  The patient was prescribed daily levetiracetam, but her parents suspect that she has not taken it for the past week.  On arrival, her airway is patent and she has good respiratory effort.  Her blood pressure is 123/82 mm Hg, pulse is 130/min, respirations are 16/min, and pulse oximetry is 99% on 2 L oxygen via nasal cannula.  The patient's eyes are open, but she is not responding to commands.  Neurologic examination shows pupils that are equal but minimally responsive to light.  Rhythmic non-suppressible twitching of the mouth, both arms, and both legs has persisted for 10 minutes.  Fingerstick glucose is 115 mg/dL.  Which of the following is the most appropriate next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_c004_4b86_8f63_07a1f72ab848_MD0005_00 Common factors that decrease seizure threshold include medication noncompliance, intercurrent illness, electrolyte abnormalities, sleep deprivation, and head injury. This patient's medication noncompliance is the most likely trigger for her seizure. Historically, status epilepticus has been defined as a single continuous seizure for >30 minutes or multiple seizures occurring in a short period with no return to baseline mental status; however, for treatment purposes, shorter cutoffs are used, as described in the flowchart. An actively seizing patient should undergo immediate assessment of the airway, breathing, and circulation. An intravenous line should be placed in anticipation of medication administration and laboratory testing (eg, serum glucose to rule out hypoglycemia as the etiology). Abortive therapy is recommended for seizures lasting >5 minutes, as delayed treatment can result in increased risk of complications (eg, hypoxemia, acidemia). First-line treatment is a benzodiazepine (eg, lorazepam, diazepam) and should not be delayed, even if there is difficulty obtaining intravenous access. If seizure activity persists, fosphenytoin should be administered. (Choice A) This patient's tachycardia, minimally responsive pupils, and seizure suggest that she is having an epileptic seizure rather than a psychogenic nonepileptic seizure. An electroencephalogram can be used to confirm seizure activity, but benzodiazepine abortive therapy is the time-sensitive priority. (Choice B) Barbiturates (eg, phenobarbital) should be administered if status epilepticus has persisted despite administration of benzodiazepines and phenytoin derivatives. Barbiturates are long-acting and often result in respiratory depression and sedation; therefore, other agents should be tried first. (Choice D) Naloxone is a pure opioid antagonist used to treat opioid overdoses, which classically present with pinpoint pupils, sedation, and respiratory depression. This patient's presentation is not consistent with an opioid overdose. (Choice E) Brain MRI is the preferred imaging modality to assess for underlying structural abnormalities but is not typically performed in the acute setting. A head CT is preferred to rule out intracranial hemorrhage in an actively seizing trauma patient. Neither study is indicated in this patient with a known seizure disorder. Educational objective: Benzodiazepine therapy is the first-line treatment for status epilepticus and should be administered for seizures lasting >5 minutes. Medication noncompliance, electrolyte abnormalities, sleep deprivation, and intercurrent illnesses lower the seizure threshold. References: Drug management for acute tonic-clonic convulsions including convulsive status epilepticus in children. (http://www.ncbi.nlm.nih.gov/pubmed/18646081) Treatment of community-onset, childhood convulsive status epilepticus: a prospective, population-based study. (http://www.ncbi.nlm.nih.gov/pubmed/18602345)

Question 9

An 8-year-old boy is brought to the office by his father due to concerns at school.  His father says, "He was a great student last year, but his grades have slipped this year.  His teacher says that he does not pay attention and often 'spaces out' during class.  I think he just likes to daydream and gets distracted."  At home, the father has noticed that the boy "blanks out" sometimes for a few seconds when he is in the middle of playing, and then he blinks his eyes a lot and quickly resumes his activity.  The patient says that he feels sad about his grades.  He has had no recent fever or sore throat, and there have been no recent stressors in his life.  The patient has gastroesophageal reflux that is controlled with ranitidine.  Vaccinations are up to date.  He has a 10-year-old sister who is in good health.  Vital signs are normal.  On physical examination, the patient is alert and interactive.  Heart rate is regular with normal S1 and S2.  The abdomen is soft and nondistended.  Cranial nerves II-XII are intact.  Muscle tone and strength are normal in all extremities.  There is a small bruise on his left knee but no rashes or pigmented skin lesions.  Which of the following is the most accurate statement regarding management of this patient's condition?

Accepted Answer

Explanation: 11ec2b69_c004_e7c7_8f63_81510541633f_MD0005_00 This patient presents with childhood absence epilepsy (CAE), a common seizure disorder of early school-aged children. Absence seizures typically start abruptly, are provoked by hyperventilation, occur many times a day, and last <20 seconds. Patients frequently describe classroom inattention, frequent "staring spells" interrupting play, eye fluttering, and automatisms (eg, oral tics). Comorbid psychiatric conditions are common, including attention deficit hyperactivity disorder, depression, and anxiety. Diagnosis is based on electroencephalography, which shows a characteristic ~3 Hz spike and wave discharge pattern. CAE is frequently well controlled with ethosuximide monotherapy. In contrast to their effects on other forms of epilepsy, certain common antiepileptics (eg, carbamazepine) can exacerbate CAE, and others (eg, phenytoin, phenobarbital) are ineffective. CAE often spontaneously remits by early puberty with no major long-term sequelae. When the patient has been seizure-free for 2 years, the antiepileptic medications frequently can be tapered. (Choice A) Cognitive behavioral therapy has been shown to reduce the frequency of psychogenic nonepileptic seizures, which are more common in patients with epilepsy with exacerbating psychosocial stressors. Patients with psychogenic nonepileptic seizures often have unusual motor activity (eg, pelvic thrusting, rolling from side to side, opisthotonus), which are not seen in this patient. (Choice B) Stimulant therapy is effective for inattentive symptoms accompanying attention deficit disorder, a common comorbidity in CAE. Stimulant medications carry a theoretical risk of decreasing the seizure threshold but can be used with caution in comorbid disease. This patient, however, ceases all activity with these spells, suggesting that he experiences seizures, not inattention. (Choices C and E) Periodic monitoring of transaminases is recommended for patients on valproic acid, given that it carries a risk of hepatotoxicity. Agranulocytosis has been reported with ethosuximide; however, hepatoxicity and leukocytosis have no such association. Periodic granulocyte count monitoring is recommended for patients on ethosuximide. Educational objective: Childhood absence epilepsy is characterized by brief lapses in consciousness that last <20 seconds and occur multiple times a day. Electroencephalography shows a pattern of ~3 Hz generalized spike and wave activity. Ethosuximide is the first-line treatment. References: Absence seizures in children. (http://www.ncbi.nlm.nih.gov/pubmed/24351614) Benign epilepsy in children. (http://www.ncbi.nlm.nih.gov/pubmed/21497276)

Question 10

An 8-year-old boy with hemophilia A is brought to the emergency department after his family was in a minor motor vehicle accident.  The child was restrained in the back seat on the passenger side when the car was hit on the driver's side.  The airbag deployed and the boy lost consciousness for approximately 15 seconds.  The patient has a headache but no nausea or vomiting.  His temperature is 36.7 C (98 F), blood pressure is 114/66 mm Hg, pulse is 88/min, and respirations are 16/min.  Physical examination shows a 2-cm right frontal hematoma.  No other signs of trauma are seen.  Neurological examination is within normal limits.  Which of the following is the best next step in the management of this child's condition?

Accepted Answer

Explanation: 11ec2b69_c00a_8d1e_8f63_3ba188dc7cc6_MD0005_00 In a healthy child with normal mental status, Glasgow Coma Scale (GCS) score of 15, and no evidence of basilar skull fracture on examination, the incidence of clinically significant intracranial injury after minor head trauma is low (<1%). However, this child is at increased risk for intracranial hemorrhage (ICH) due to his history of hemophilia A. basilar skull fracture ICH is a significant cause of morbidity and mortality in children with hemophilia. ICH can occur spontaneously in infants and even after minor head trauma in older children with hemophilia. Common presenting symptoms include headache,vomiting, and altered mental status. A head CT scan without contrast must be ordered to look for hemorrhage in children with bleeding diatheses who sustain more than a trivial head injury or are symptomatic. New blood appears white on CT and can be obscured by contrast (also appears white). In addition to imaging, Factor VIII or IX should be replaced as soon as possible following the traumatic injury. white on CT (Choice A) Discharging this patient home is inappropriate given the risk for intracranial hemorrhage based on history of hemophilia and current symptoms. (Choice B) Observation for 4-6 hours prior to discharge would be appropriate if this child did not have a history of hemophilia. (Choice C) Head CT scan with contrast is most useful for identifying abscesses or intracranial masses, but contrast obscures acute bleeding and can confound the workup for intracranial injury. (Choice E) Plain radiographs of the skull can identify skull fractures but cannot detect intracranial injury. If a fracture is suspected, a head CT without contrast should be ordered because risk of intracranial hemorrhage increases with a skull fracture. Educational objective: Children with hemophilia and other bleeding diatheses who are symptomatic at the time of presentation are at increased risk for intracranial hemorrhage after even minor head trauma. A head CT without contrast should be performed emergently. References: Identification of children at very low risk of clinically-important brain injuries after head trauma: a prospective cohort study. (http://www.ncbi.nlm.nih.gov/pubmed/19758692) Intracranial hemorrhage after blunt head trauma in children with bleeding disorders. (http://www.ncbi.nlm.nih.gov/pubmed/21232760) Utility of computed tomography of the head following head trauma in boys with haemophilia. (http://www.ncbi.nlm.nih.gov/pubmed/17880444) Intracranial haemorrhage in haemophilia A and B. (http://www.ncbi.nlm.nih.gov/pubmed/18081890)

Question 11

A 12-year-old boy is brought to the emergency department due to unsteadiness, weakness, and falls. The patient recently went on a week-long family camping and hiking trip to the mountains in Washington State. On returning home to California 2 days ago, he experienced a sense of fatigue and a tingling sensation in his fingers. Yesterday, the patient started having bilateral leg weakness and is now unable to walk without assistance; he sways from side to side when standing and then falls to the floor. The patient has had no fever, headache, joint pain, or skin rash and has no history of recent respiratory or gastrointestinal illness. Vital signs are within normal limits. He is alert and active but can stand only briefly before requiring assistance. There is no increased resistance to neck flexion. Cranial nerve examination is normal and sensation is not impaired. Muscle strength is 4/5 in the upper extremities and 2/5 in the lower extremities. Deep tendon reflexes are absent at the knees and ankles but are normal elsewhere. Finger-to-nose testing shows dysmetria of the arms. Item 1 of 2 Which of the following is the best next diagnostic intervention that should be performed for this patient?

Accepted Answer

Explanation: This patient with ataxia, weakness, and absent deep tendon reflexes after a camping trip to Washington State most likely has tick paralysis. Tick paralysis is a rare, potentially life-threatening disorder seen primarily in Australia and Western North America. Although more than 40 species of ticks can cause tick paralysis, most cases in North America are due to Dermacentor andersoni (Rocky Mountain wood tick) and D variabilis (American dog tick). Neurotoxins in the tick saliva are transmitted to the host over 4-7 days of tick attachment and result in: A brief prodrome of fatigue and paresthesias Gait ataxia and ascending paralysis that develop over hours Absent deep tendon reflexes Fever is typically absent and sensation is rarely affected. Laboratory and imaging studies are usually normal. Patients with suspected tick paralysis require a meticulous skin examination to identify the tick. (Choices A and C) Electrodiagnostic studies and cerebrospinal fluid analysis (elevated protein with normal leukocytes) are often obtained to support the diagnosis of Guillain-Barré syndrome. This syndrome causes ascending paralysis with absent deep tendon reflexes, but usually follows a gastrointestinal or respiratory infection and typically progresses over several days or weeks (rather than hours). This child who recently camped in the woods should first be examined meticulously for ticks to rule out tick paralysis. (Choice B) A spinal cord lesion can cause motor dysfunction and diminished deep tendon reflexes. However, sensation is likely to be affected, and most etiologies (eg, infection, tumor) result in slowly progressive symptoms over several days or weeks (not hours). (Choice E) Clostridium botulinum toxin can be found by serum analysis. Botulism usually causes an afebrile, descending paralysis starting with the cranial nerves. This patient with motor weakness of the legs and normal cranial nerves is unlikely to have botulism. Educational objective: Tick paralysis is a rare, potentially life-threatening disorder caused by neurotoxins found in tick saliva. Manifestations typically arise after 4-7 days of tick attachment and include rapidly progressive gait ataxia and ascending paralysis. Neurologic examination usually reveals absent deep tendon reflexes and normal sensation. Fever is uncommon. A meticulous skin examination to identify the tick is required. References: Tick paralysis. (http://www.ncbi.nlm.nih.gov/pubmed/20842579) Tick paralysis. (http://www.ncbi.nlm.nih.gov/pubmed/15257515) A 60-year meta-analysis of tick paralysis in the United States: a predictable, preventable, and often misdiagnosed poisoning. (http://www.ncbi.nlm.nih.gov/pubmed/20186584)

Question 12

A 35-year-old man comes to the office due to worsening daytime sleepiness over the last 6 months. He says, "I keep falling asleep during inappropriate times of the day. I regularly fall asleep in staff meetings and even fell asleep while talking to a customer." The patient has not fallen asleep while driving and has had no episodes of sudden weakness, inability to move, or restless legs. Although he has no difficulty falling asleep and sleeps 7-8 hours a night, he rarely feels rested. The patient lives alone and does not know if he snores. He does not report gasping for air. He is not depressed but says he is "under stress" at work after receiving a negative performance evaluation, and he worries he will be fired for falling asleep. His medical history is significant for hypertension treated with lisinopril. He smokes a pack of cigarettes a day and consumes an average of 2-5 alcoholic drinks a week. He does not use illicit drugs. Temperature is 36.7 C (98 F), blood pressure is 129/88 mm Hg, pulse is 84/min, and respirations are 14/min. BMI is 36 kg/m². Physical examination is within normal limits. Which of the following is the best next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_c001_1731_8f63_9fb6374bb850_MD0005_00 This patient's history of excessive daytime sleepiness is suspicious for obstructive sleep apnea (OSA), given his risk factors of gender, hypertension, and obesity. The STOP-Bang survey can help quantify risk for OSA; in this case, even without clarification regarding snoring or witnessed apneas, the patient has a STOP-Bang score of 4 and would be considered at intermediate risk. Other common causes of excessive daytime sleepiness include narcolepsy, periodic limb movements of sleep, insufficient sleep, depressive disorders, general medical conditions, and the effects of prescription or recreational drug use. The next step in evaluation of excessive daytime sleepiness with suspected OSA is polysomnography (PSG). PSG evaluates the quality of sleep, and the recorded tracings of physiologic variables can assist in the diagnosis of obstructive sleep apnea, periodic limb movements, and narcolepsy. (Choice A) Continuous positive airway pressure is the appropriate treatment once the diagnosis of OSA is confirmed. (Choice B) For patients with suspected narcolepsy, management also involves an initial evaluation with PSG to rule out other sleep disorders. In such cases PSG is followed by a multiple sleep latency test to confirm shortened sleep latency and sleep-onset rapid eye movement periods. Although narcolepsy is part of the differential diagnosis, it is less likely in this patient without episodes of sudden weakness (cataplexy) or inability to move (sleep paralysis). (Choice D) A psychiatric evaluation would be the next step if there were strong suspicion that the patient's excessive daytime sleepiness is a symptom of depression or other psychiatric disorder. This patient has denied depression; his stress is related to impairment due to his sleep disorder. (Choice E) The stimulant modafinil would be appropriate for a confirmed diagnosis of narcolepsy. Educational objective: Common causes of excessive daytime sleepiness include obstructive sleep apnea (OSA), narcolepsy, periodic limb movements, and depressive disorders. The classic presentation for OSA is an overweight, hypertensive male with excessive daytime sleepiness, snoring, and apneic spells. Polysomnography is indicated in the diagnostic evaluation of patients with suspected OSA and can assist in the diagnosis of other sleep disorders. References: Office evaluation of the "tired" or "sleepy" patient. (http://www.ncbi.nlm.nih.gov/pubmed/21321832) Management of common sleep disorders. (http://www.ncbi.nlm.nih.gov/pubmed/23944726)

Question 13

A 12-year-old boy is brought to the emergency department due to unsteadiness, weakness, and falls.  The patient recently went on a week-long family camping and hiking trip to the mountains in Washington State.  On returning home to California 2 days ago, he experienced a sense of fatigue and a tingling sensation in his fingers.  Yesterday, the patient started having bilateral leg weakness and is now unable to walk without assistance; he sways from side to side when standing and then falls to the floor.  The patient has had no fever, headache, joint pain, or skin rash and has no history of recent respiratory or gastrointestinal illness.  Vital signs are within normal limits.  He is alert and active but can stand only briefly before requiring assistance.  There is no increased resistance to neck flexion.  Cranial nerve examination is normal and sensation is not impaired.  Muscle strength is 4/5 in the upper extremities and 2/5 in the lower extremities.  Deep tendon reflexes are absent at the knees and ankles but are normal elsewhere.  Finger-to-nose testing shows dysmetria of the arms. Diagnostic intervention reveals significant findings.  Which of the following is most likely to improve this patient's condition?

Accepted Answer

Explanation: Patients with tick paralysis require a meticulous skin examination to identify the causative tick. Tick removal eliminates the precipitating neurotoxin and usually results in significant clinical improvement within a few hours. Most patients recover completely without further intervention or medications. (Choice A) Equine serum heptavalent antitoxin is used to treat botulism. Botulism typically causes a descending paralysis starting in the cranial nerves. (Choice B) High-dose intravenous glucocorticoids are used to treat transverse myelitis. Transverse myelitis typically causes a sensory level and altered sensation in the lower extremities. (Choice D) Tick removal is usually curative and the treatment of choice. (Choice E) Therapeutic plasma exchange reduces time to recovery in patients with Guillain-Barré syndrome. This syndrome usually develops over several days or weeks (not hours) and is frequently preceded by a gastrointestinal or respiratory illness. Educational objective: Patients with tick paralysis require a meticulous skin examination to identify the causative tick. Tick removal usually significantly improves symptoms within several hours. Most patients recover completely without further intervention.

Question 14

A 57-year-old woman comes to the emergency department due to difficulty speaking.  Two hours ago, the patient was on the phone, at work, arguing with her daughter when she suddenly could not find her words.  She had to end the call and then could not explain to co-workers what was wrong.  The patient also noticed some weakness in her right arm.  Her symptoms resolved on the way to the emergency department.  She has not had similar symptoms before.  Medical history includes hypertension and type 2 diabetes mellitus.  Temperature is 37.2 C (99 F), blood pressure is 155/90 mm Hg, pulse is 89/min, and respirations are 14/min.  Complete neurologic examination shows no abnormalities.  Blood glucose is normal.  Noncontrast head CT scan is normal.  The patient feels "back to normal" and requests to be discharged home.  Which of the following is the most appropriate response by the physician?

Accepted Answer

Explanation: This patient with stroke risk factors (eg, hypertension, type 2 diabetes mellitus) and transient deficits (eg, aphasia, right arm weakness) localizable to a cerebral vascular distribution (eg, left middle cerebral artery) likely experienced a transient ischemic attack (TIA). A TIA-defined as a transient episode of neurologic dysfunction caused by ischemia but without acute infarction-is considered a neurologic emergency because it signals an acutely increased risk of stroke (eg, up to 5% within the first 48 hr, up to 12% within the first 30 days). The clinical diagnosis of TIA should prompt an urgent, comprehensive diagnostic evaluation that includes the following: Advanced brain imaging, including MRI, which is more sensitive than CT scan for detecting ischemia Neurovascular imaging, including CT scan or MR angiography of the head and neck, to evaluate cerebral vasculature patency, and carotid duplex ultrasonography to evaluate for carotid stenosis Investigations for potential sources of embolism, including prolonged cardiac rhythm monitoring and echocardiography, to evaluate for a cardiac source (eg, atrial fibrillation, intracardiac thrombus) These studies are typically done in the inpatient setting to expedite the workup, which should be completed within 24-48 hours (Choice A). Immediate identification of a stroke source (eg, carotid stenosis, mural thrombus) and timely intervention (eg, antiplatelet therapy, anticoagulation) can reduce the risk of disabling stroke compared to delayed intervention. (Choice B) Although patients under stress may experience neurologic symptoms (eg, paresthesia, dizziness), focal neurologic deficits localizable to a vascular distribution in a patient with multiple stroke risk factors (eg, hypertension, type 2 diabetes mellitus) are concerning for TIA; these should not be attributed to stress without further evaluation. (Choice C) A repeat CT scan may be used to sequentially track brain hemorrhage (eg, hemorrhagic stroke, traumatic hematoma) and assess the need for intervention. However, it is not routinely used to follow ischemia 6 hours later because ischemic infarcts may take days to be evident on CT scan, and smaller infarcts may not be visible at all (in contrast to MRI, which is more sensitive). (Choice D) Thrombolysis may be appropriate for patients with ischemic stroke who present within 4.5 hours of symptom onset. However, these patients have ongoing, disabling neurologic symptoms, in contrast to this patient whose TIA symptoms have resolved. Educational objective: Patients with transient ischemic attack have an acutely increased risk of stroke and should undergo urgent evaluation and initiation of appropriate preventive therapy (eg, antiplatelet therapy) within 24 hours. References: Can I send this patient with stroke home? strategies managing transient ischemic attack and minor stroke in the emergency department. (http://www.ncbi.nlm.nih.gov/pubmed/29321107) Implementation of a rapid, protocol-based TIA management pathway. (http://www.ncbi.nlm.nih.gov/pubmed/29560046)

Question 15

A 46-year-old African-American male has a large pituitary mass, which was seen on a CT scan of his head following a head injury.  He denies any symptoms, except for mild erectile impotence.  Physical examination reveals a right, upper, temporal, quadrantic field defect.  His hormonal profile shows a prolactin level of 5,000 ng/mL (normal 5-20 ng/ml).  His IGF-1, TSH, and free T4 levels are normal, while his LH and FSH levels are low.  MRI reveals a 2.5 cm pituitary mass abutting, but not elevating, the optic chiasm.  What is the most appropriate management for this patient?

Accepted Answer

Explanation: The patient has a large macroadenoma that is likely to respond to oral dopaminergic receptor agonists. Dopaminergic receptor agonists (bromocriptine and cabergoline) are the first-line treatment for prolactinomas, including large prolactinomas. With such treatment, the tumor's size decreases within days, and the patient's vision improves even before the decrease in tumor size is appreciated on MRI. Once treatment with an agent is started, the patient will require close monitoring of his clinical symptoms, prolactin levels, visual field assessments, and MRI of his pituitary gland. (Choices A and B) Transphenoidal and transcranial surgery is rarely required for patients with prolactinomas. Even patients with visual defects or other compressive symptoms may respond to treatment with dopaminergic receptor agonists. (Choice C) Hypogonadism secondary to hyperprolactinemia is likely to respond to dopaminergic receptor agonists. Treatment with testosterone is not warranted at this time. (Choice D)Gamma knife radiation is contraindicated in this patient because the tumor is within 5 mm of the optic chiasm. Furthermore, gamma knife is not indicated as a primary mode of treatment in majority of patients with pituitary tumor because of the lack of immediate effects and an increased risk of hypopituitarism in the long term. Educational Objective: Dopaminergic receptor agonists are first-line treatment for prolactinomas, including large prolactinomas. Treatment with dopaminergic receptor agonists generally leads to a decrease in tumor size within a few days. Visual symptoms usually improve before the tumor's decrease in size is seen on MRI.

Question 16

A 54-year-old homeless man is brought to the emergency department by the police after he was found to be confused and disheveled. The patient is unable to provide a medical history and has no prior hospital record. Temperature is 36.5 C (97.7 F), blood pressure is 110/60 mm Hg, pulse is 90/min, and respirations are 16/min. The patient is thin and appears ill, with temporal muscle wasting. The mucous membranes are dry. The lungs are clear to auscultation, and heart sounds are normal with no murmur. The abdomen is soft, nontender, and nondistended. The patient is disoriented and unable to recognize where he is or state the date. Bilateral pupils are equal and reactive to light, but extraocular motion is restricted on leftward gaze. No obvious motor weakness is present. Deep tendon reflexes are reduced and plantar responses are flexor. The patient has no neck rigidity, but his gait is markedly ataxic. Laboratory results are as follows: Item 1 of 2 Which of the following is the best next step in management of this patient's confusion?

Accepted Answer

Explanation: Wernicke encephalopathy (WE) is a life-threatening disorder of thiamine deficiency seen most commonly in patients with heavy alcohol use and/or severe malnutrition. The classic features of WE include: Encephalopathy - disorientation and confusion Oculomotor dysfunction - lateral rectus palsy Gait ataxia - wide-based gait or complete gait impairment No specific testing for thiamine deficiency is available, but laboratory evidence of alcoholic hepatitis (2:1 ratio of aspartate to alanine aminotransferase) or synthetic hepatic dysfunction (prolonged INR, low albumin) often supports the diagnosis. Treatment with intravenous thiamine, a well-tolerated and low-risk medication, is required with urgency. Because all 3 clinical features of WE are present in <10% of affected patients, treatment is generally given to those in whom the diagnosis is possible (eg, unclear cause of gait ataxia or confusion). Intravenous thiamine usually improves ocular abnormalities within hours, but confusion and gait ataxia may persist for days or weeks; many patients never fully recover. (Choice A) Delirium can be a sign of acute infection, but this patient has no fever or leukocytosis and has classic findings for WE. (Choice B) Although this patient has dry mucous membranes and evidence of malnutrition, providing fluids with glucose (dextrose) prior to thiamine is associated with a worsening of WE symptoms and is not recommended. (Choice C) Lorazepam is a benzodiazepine often used to treat acute alcohol withdrawal. Manifestations typically begin with central nervous system hyperactivity, including insomnia, tremulousness, anxiety, and diaphoresis. This patient may require treatment for alcohol withdrawal, but he is manifesting signs of WE and requires thiamine. (Choice E) Lactulose is commonly used to treat hepatic encephalopathy, which can cause confusion. However, hepatic encephalopathy is not typically associated with ophthalmoplegia. Educational objective: Wernicke encephalopathy is due to thiamine deficiency, which is common in patients with chronic alcoholism. Classic manifestations include the triad of encephalopathy, oculomotor dysfunction, and gait ataxia. Treatment with intravenous thiamine is usually lifesaving and should be considered in any patient with unexplained delirium or ataxia.

Question 17

A 28-year-old previously healthy man comes to the office with his girlfriend after having intermittent headaches, dizziness, and fatigue for the last several weeks.  He has also had insomnia and trouble concentrating.  She reports that he is irritable, argumentative, and very sensitive to noise.  The patient was involved in a fight 6 weeks ago during which he was hit on the head and "blacked out" for a few minutes.  According to his girlfriend, he consumes several hard drinks over the weekend but rarely drinks "in excess."  His blood pressure is 130/80 mm Hg and pulse is 76/min.  Cardiopulmonary examination is unremarkable.  Muscle power is 5/5 in the bilateral upper and lower extremities, sensation is intact, and reflexes are 2+ throughout.  The patient is oriented and has normal memory but is anxious.  His hemoglobin is 14.2 gm/dL, creatinine is 0.8 mg/dL, and liver function tests are normal.  MRI of the head is normal.  Urine toxicology screen is positive for cannabinoids but negative for cocaine and opioids.  Which of the following is the most likely diagnosis?

Accepted Answer

Explanation: This patient is showing signs of postconcussive syndrome following a mild traumatic brain injury (TBI). TBI of any severity can lead to (a few hours to days later) postconcussive syndrome, which includes the following constellation of symptoms: headache, confusion, amnesia, difficulty in concentrating or with multitasking, vertigo, hypersensitivity to various stimuli, mood alteration, sleep disturbance, and anxiety. These symptoms typically resolve with symptomatic treatment within a few weeks to months following TBI; however, some patients may have persistent symptoms lasting >6 months. (Choice A) This patient does not appear to drink alcohol excessively based on his social history and laboratory studies. Given his relatively recent history of head trauma, postconcussive syndrome is a more likely diagnosis than alcohol intoxication. (Choice B) Cannabis withdrawal involves at least 1 physiological symptom (eg, abdominal pain, sweating, shakiness, fever, chills, headache) and 2 or more of the following symptoms developing after cessation of heavy and prolonged use: Irritability, anxiety, insomnia, decreased appetite, restlessness, depressed mood. Given that cannabis was found on this patient's urine toxicology screen, withdrawal from this drug is less likely. (Choice C) Generalized anxiety disorder is characterized by excessive anxiety and worry about multiple issues for >6 months. Although difficulty in concentrating, irritability, sleep disturbance, and fatigue are symptoms of generalized anxiety disorder, this patient's history of head trauma and shorter duration of symptoms make postconcussive syndrome more likely. (Choice E) Post-traumatic stress disorder (PTSD) develops after exposure to psychologically traumatic events (eg, threatened death, serious injury, sexual violence) with characteristic symptoms of recurrent flashbacks, nightmares, hyperarousal, avoidance, and numbing. PTSD can also be associated with insomnia, irritability, and difficulty in concentrating, but this patient's history of head trauma and physical symptoms make TBI more likely. Educational objective: Traumatic brain injury (TBI) of any severity can lead to (a few hours to days later) postconcussive syndrome, which is characterized by headache, confusion, amnesia, difficulty in concentrating or with multitasking, vertigo, mood alteration, sleep disturbance, and anxiety. These symptoms typically resolve with symptomatic treatment within a few weeks to months following TBI; however, some patients may have persistent symptoms lasting >6 months. References: Diagnosis of mild head injury and the postconcussion syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/10739967)

Question 18

A 45-year-old Caucasian man comes to see you in the office.  He is accompanied by his wife.  They have noticed that he has shakiness of both hands for the past six months.  The shakiness is limited only to his hands and has not progressed over time.  It becomes much more pronounced when he tries to pick up things or attempts to eat or drink.  On physical examination, you notice a constant tremor in both his hands, which becomes much more pronounced when his arms are outstretched.  There is also a very subtle head tremor present.  There is no rigidity or slowing of voluntary movements in the upper or lower extremities.  His father had a similar problem in his 50s or 60s, but he is not sure what treatment he received at that time.  His wife is extremely anxious and wants to know his prognosis.
The patient works as a carpenter and feels that he is unable to perform the fine motor tasks due to the tremor. Which of the following is the most appropriate next step in the management of this patient?

Accepted Answer

Explanation: The patient is suffering from a familial or benign essential tremor. Treatment for familial or benign essential tremor is not necessary in all patients and should be delayed as long as possible due to the need for indefinite treatment. The patient, however, has a significant disability due to the tremor that is affecting his ability to perform fine motor tasks with his hands. Beta-blockers are the most common first-line agents used in the treatment of benign essential tremors. Several studies have evaluated the efficacy of propranolol in reducing tremors and the disability associated with the tremor. (Choices A and B) The patient has symptoms that are affecting his work as a carpenter. He should be treated with pharmacotherapy at this point. Physical therapy does not improve the tremor in this condition and is not recommended. (Choices D and E) The patient does not have any signs or symptoms of Parkinson's disease; therefore, a carbidopa-levodopa combination or pramipexole is not indicated in this patient. Educational Objective: Beta-blockers are the first-line agents for symptomatic treatment of patients with benign essential or familial tremor.

Question 19

A 44-year-old woman comes to the emergency department complaining of several hours of severe occipital headache.  The headache is described as intense and began suddenly when she was climbing up the stairs at the local subway station.  She rested at home for 6-7 hours after the onset of symptoms, but the headache remains persistent and unbearable.  The patient has mild nausea but no vomiting, blurry vision, muscle weakness, or syncope.  She has a history of moderate-intensity tension headaches that concentrate around the temple.  The patient has been taking over-the-counter pain killers for these headaches recently but states that this current headache is worse and differs from her usual pattern.  She also has a history of mild intermittent asthma and dysmenorrhea.  She has a 15-pack-year smoking history.  Her temperature is 98 F (36.7 C), blood pressure is 142/90 mm Hg, and pulse is 95/min.  She is in significant distress due to pain but is cooperative.  There are no focal neurologic deficits.  Non-contrast CT scan of the head shows no intracranial abnormality.  Which of the following is the best next step in managing this patient?

Accepted Answer

Explanation: 11ec2b69_c006_bc88_8f63_0b86ced6d173_MD0005_00 This patient's presentation is concerning for subarachnoid hemorrhage (SAH), which is most often due to a ruptured saccular aneurysm. SAH typically presents with sudden onset of a severe headache and possible nausea, vomiting, brief loss of consciousness, focal neurologic deficits, or meningismus. Patients usually develop a suddenly worsening headache different from their typical headache pattern, or experience an intense new headache described as "the worst headache of my life." The headache is typically lateralized to the side of the aneurysm and can correlate to rapid spread of bleeding. Even though this patient has a history of chronic tension headaches, her current headache is more intense and differs in character and location (occipital area) from her usual headaches. Noncontrast head CT is the preferred initial test to establish the diagnosis of SAH. Head CT performed within 6-12 hours of SAH onset is nearly 100% sensitive for diagnosing SAH, but this rapidly decreases with increased time from SAH onset and smaller hemorrhages. As a result, lumbar puncture (LP) is required to completely exclude suspected SAH in patients with a negative noncontrast head CT. The typical cerebrospinal fluid (CSF) findings in SAH include an elevated opening pressure, xanthochromia (pink/yellow tint due to hemoglobin degradation products), or consistently elevated red blood cell (RBCs) count in the 4 CSF tubes. An elevated RBC count in the first tube, followed by declining numbers of RBCs with each successive tube suggests a traumatic tap. Retained CSF blood can take up to 6 hours to degrade and cause xanthochromia; for this reason, LP is most sensitive >6 hours from SAH onset. (Choice A) Acetaminophen combined with aspirin and caffeine can effectively treat tension headaches refractory to initial nonsteroidal anti-inflammatory drugs. However, this patient first needs LP to exclude SAH before being treated for tension headaches. (Choice B) This patient's headache could be due to recent use of over-the-counter pain killers causing rebound headache. However, this is a diagnosis of exclusion, and LP should be performed first to rule out SAH. (Choice D) An MRI of the head has similar sensitivity to a head CT for diagnosing SAH. This patient's head CT is negative; as a result, she requires LP to definitively exclude SAH. (Choice E) Triptan medications are generally more effective for migraines than for tension headaches. Educational objective: Subarachnoid hemorrhage typically presents with sudden onset of severe headache and possible nausea, vomiting, brief loss of consciousness, focal neurologic deficits, or meningismus. Patients usually develop a suddenly worsening headache different from their typical headache pattern, or an intense new headache described as "the worst headache of my life." Noncontrast head CT is the initial preferred test for diagnosis, followed by lumbar puncture to document xanthochromia if the head CT is negative. References: Guidelines for the management of aneurysmal subarachnoid hemorrhage: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. (http://www.ncbi.nlm.nih.gov/pubmed/22556195) National guidelines for analysis of cerebrospinal fluid for bilirubin in suspected subarachnoid hemorrhage. (http://www.ncbi.nlm.nih.gov/pubmed/14503985)

Question 20

A 32-year-old man comes to the office due to right-sided facial weakness for 3 days.  He has had no fever, headache, vision disturbances, or extremity weakness or numbness but has been extremely fatigued over the past several months.  The patient has no prior medical conditions and takes no medications.  He drinks alcohol occasionally and does not use tobacco or illicit drugs.  The patient lives in coastal California and has not traveled recently.  Vital signs are within normal limits.  Physical examination reveals right-sided facial droop with flattening of the nasolabial fold.  He is unable to elevate the right eyebrow or close the right eye completely.  Upper and lower extremity muscle strength, deep tendon reflexes, and sensation are normal on both sides.  There is no neck rigidity.  The lungs are clear on auscultation, and heart sounds are normal.  Hepatosplenomegaly is present.  The cervical, axillary, and inguinal lymph nodes are multiple, firm, and nontender.  No skin rash or extremity edema is present.  The joints are not swollen or tender. A week later, the patient returns to the office for follow-up.  His facial weakness is improving with oral glucocorticoids, and he has had no new symptoms.  Laboratory results from the previous visit are as follows:  Chest x-ray reveals bilateral hilar adenopathy and interstitial infiltrates.  Which of the following is most appropriate to confirm the diagnosis?

Accepted Answer

Explanation: This patient's chest x-ray evidence of bilateral hilar lymphadenopathy and interstitial infiltrates supports the diagnosis of sarcoidosis. Other common findings include elevated calcium, erythrocyte sedimentation rate, and alkaline phosphatase (if the liver is involved). Angiotensin-converting enzyme levels are also increased in approximately 75% of cases, but this test has poor diagnostic sensitivity/specificity and cannot be used for confirmation (Choice A). Suspected cases are typically confirmed when the following occurs: Diseases causing similar symptoms (eg, tuberculosis) are excluded. Biopsy reveals noncaseating granulomas. Biopsy should be performed on the involved location that is easiest to assess, which is usually a palpable lymph node or skin lesion. If no superficial lesion is present or the diagnosis remains uncertain after initial biopsy, bronchoscopy with transbronchial biopsy is often performed (Choice B). This patient with peripheral lymphadenopathy (eg, cervical, axillary, inguinal) should undergo excisional lymph node biopsy for confirmation. (Choice D) All patients with pulmonary sarcoidosis should undergo pulmonary function testing to evaluate for respiratory impairment, but this test cannot confirm the diagnosis. (Choice E) Liver biopsy cannot easily distinguish noncaseating granulomas due to sarcoidosis from other hepatic granulomatous diseases. As such, liver biopsy is usually reserved for cases in which other biopsies have failed to establish a diagnosis. Educational objective: Patients with clinical and radiographic features consistent with sarcoidosis are usually diagnosed after other diseases with similar symptoms (eg, tuberculosis) have been excluded and biopsy demonstrates noncaseating granulomas. Biopsy should be performed on the involved location easiest to assess, which is usually a palpable lymph node or skin lesion. When there is no easily accessible lesion, fiberoptic bronchoscopy with transbronchial lung biopsy is often performed. References: The clinical and radiologic features of nodular pulmonary sarcoidosis. (http://www.ncbi.nlm.nih.gov/pubmed/18843518)

Exam 1: Advanced Clinical Medicine