Question 1

A 62-year-old Caucasian man comes to the physician because of progressive weight loss and right lower extremity weakness for the last two months. On further questioning, he tells you that he has been having frequent leg cramps in the right leg. These cramps are most prominent in the morning, after he wakes up. He also has difficulty with swallowing and chewing. He denies any problems with bowel or bladder function. His other medical problems include hypertension and hypercholesterolemia. He does not use tobacco. His family history is not significant. His vital signs are within normal limits. The physical examination reveals weakness in his right lower leg, atrophy and fasciculation of the thigh and calf muscles, a hyperactive knee jerk, and tongue fasciculations. The rest of his neurological examination, including the sensory examination, is normal. Item 1 of 2 Which of the following is the most likely diagnosis?

Accepted Answer

Explanation: Amyotrophic lateral sclerosis (ALS) is the most common form of progressive motor neuron disease. It is a relentlessly progressive disorder that involves both the lower motor neurons (consisting of anterior horn cells in the spinal cord and brainstem neurons innervating the bulbar muscles) and upper or corticospinal motor neurons. At its onset, ALS may involve selective loss of function of only upper or lower motor neurons, but it ultimately causes progressive loss of both upper and lower motor neurons. The initial sign of the disease with lower motor neuron involvement is an insidiously developing asymmetric weakness, usually first evident distally in one of the limbs. Patients may disclose a history of recent development of cramping with volitional movements that typically occur in the early morning hours (e.g. while stretching in bed). Weakness is associated with progressive wasting, atrophy of muscles, and spontaneous twitching or fasciculations of motor units. Involvement of the bulbar muscles leads to difficulty with chewing and swallowing, as well as fasciculations of the face and tongue. ALS with prominent corticospinal involvement is characterized by hyperactivity of muscle-stretch reflexes (tendon jerks) and frequent spastic resistance to passive movements of the affected limbs. Ocular motility, sensory, bowel, bladder, and cognitive functions are preserved, even with advanced disease. (Choice B) Vascular dementia is characterized by the presence of behavioral disturbances and cognitive deficits associated with clinical or radiographic evidence of a stroke. The decline in the level of cognition is relatively abrupt and progresses in a stepwise fashion. (Choice A) Binswanger's disease is a type of vascular dementia that involves white matter infarcts. Patients with this disease usually present with apathy, agitation, and bilateral corticospinal or bulbar signs. (Choice C) Multiple sclerosis is usually seen in younger females with two or more clinically distinct episodes of CNS dysfunction. Patients typically have sensory, visual, or bladder and bowel dysfunction. (Choice E) Brain stem tumors may compress the cervical cord and produce weakness, fasciculations in the upper limbs, and spasticity in the legs. The presentation may closely resemble ALS; however, absence of pain or sensory changes and normal bowel and bladder function in the patient favor ALS. Educational Objective: ALS should be suspected in patients who present with progressive weakness accompanied by both upper and lower motor neuron deficits. Ocular motility, sensory, bowel, bladder, and cognitive functions are preserved, even with advanced disease.

Question 2

A 79-year-old man is brought to the office by his daughter.  The patient has been having increasing difficulty caring for himself over the last 2 years.  He is now dependent on his daughter for meal preparation, shopping, and cleaning.  He will frequently go a week without bathing and often has difficulty dressing himself appropriately.  The patient sometimes grows frustrated and shouts at his daughter but has never been physically violent.  On direct questioning, he states that he feels fine, does not see any reason for coming to the office, and is quite suspicious of his daughter's motives.  The patient has lived alone since his wife died 5 years ago.  He has hypertension, osteoarthritis, and a history of remitted major depressive disorder.  Medications include hydrochlorothiazide and acetaminophen as needed.  Neurologic examination is nonfocal. The patient scores 20 out of 30 on the Montreal Cognitive Assessment.  Neuroimaging studies reveal diffuse cortical atrophy.  The laboratory workup is unremarkable.  Which of the following is the most appropriate next step in pharmacotherapy?

Accepted Answer

Explanation: Acetylcholinesterase inhibitors (eg, donepezil, rivastigmine, galantamine) are first-line pharmacotherapy for dementia-related cognitive impairment. These medications inhibit acetylcholinesterase at the synaptic cleft, thereby increasing cholinergic transmission and resulting in modest symptomatic improvement in some patients. The NMDA receptor antagonist memantine is another medication that has been shown to be modestly beneficial for symptomatic management of moderate to severe dementia. Importantly, there is no evidence to suggest that any medication is disease-modifying; as such, pharmacotherapy for dementia should be considered symptomatic treatment. (Choice A) Bromocriptine, a dopamine agonist, may address parkinsonian symptoms in Parkinson disease dementia or dementia with Lewy bodies; however, it has no indication for treatment of non-parkinsonian dementia. (Choice B) Antipsychotics are associated with an increased risk of death in patients with dementia and should be used only as a last resort in the treatment of severe behavioral disturbance. When this class of medications is indicated, second-generation antipsychotics (eg, quetiapine) are generally preferred because of their favorable side effect profile. (Choice D) Selegiline, a monoamine oxidase inhibitor, has been studied as a treatment for dementia due to its antioxidant properties; however, results have been mixed and it is currently not considered a first-line treatment. (Choice E) There is limited evidence that selective serotonin reuptake inhibitors (eg, sertraline) are effective in the treatment of dementia-related neuropsychiatric symptoms other than depression. This patient does not currently meet criteria for a depressive disorder, and his prominent cognitive impairment would be better targeted with an acetylcholinesterase inhibitor. Educational objective: Acetylcholinesterase inhibitors are first-line pharmacotherapy for cognitive symptoms associated with dementia. Memantine, an NMDA receptor antagonist, may also be beneficial for patients with dementia. References: Donepezil and memantine for moderate-to-severe Alzheimer's disease. (http://www.ncbi.nlm.nih.gov/pubmed/22397651) Nursing home placement in the Donepezil and Memantine in Moderate to Severe Alzheimer's Disease (DOMINO-AD) trial: secondary and post-hoc analyses. (http://www.ncbi.nlm.nih.gov/pubmed/26515660)

Question 3

A 71-year-old man is brought to the emergency department by his daughter for evaluation of an episode of slurred speech and right upper extremity weakness. The symptoms began approximately 3 hours ago and lasted an hour. He had no associated headache, chest pain, palpitations, shortness of breath, or syncope. The patient has had no similar episodes in the past. He now feels well. Medical history is significant for hypertension. He has a 60-pack-year smoking history but quit 8 years ago. The patient's mother had atrial fibrillation. Blood pressure is 162/96 mm Hg and pulse is 92/min. BMI is 33 kg/m². Auscultation of the upper anterior cervical triangle on the left side of the neck reveals a bruit. Neurologic examination is normal. No lower extremity edema is present. Low-density lipoprotein cholesterol is 110 mg/dL. ECG shows normal sinus rhythm and nonspecific ST-segment and T-wave changes. MRI of the brain shows mild age-related changes but no areas of restricted diffusion. MR angiogram reveals 75% stenosis of the proximal left internal carotid artery and normal intracerebral vessels. Echocardiography shows left ventricular hypertrophy, normal left ventricular function, and mild left atrial enlargement. A patent foramen ovale is detected via agitated saline study. Lower extremity ultrasound is negative for deep vein thrombosis. He is started on aspirin and rosuvastatin. Which of the following is the best management strategy for this patient?

Accepted Answer

Explanation: This patient developed transient right-sided motor weakness and slurred speech with no evidence of infarction on MRI, suggesting a transient ischemic attack (TIA). Right-sided motor symptoms usually indicate a lesion in the vascular distribution of the left middle cerebral artery, a branch of the left internal carotid artery. MR angiogram of this vessel demonstrated significant stenosis, indicating symptomatic carotid artery disease. Symptomatic carotid artery disease is diagnosed when a patient with carotid stenosis develops a TIA or nondisabling stroke in the vascular distribution of the diseased vessel. Manifestations typically arise due to either low-flow states (obstructing lesions) or embolic disease. Patients with symptomatic carotid artery disease who have high-grade (eg, 70%-99%) stenosis and a life expectancy ≥5 years should undergo carotid endarterectomy (CEA) to reduce future risk of stroke. However, the risks of CEA likely outweigh any potential benefits for patients who are poor surgical candidates (eg, multiple medical comorbidities), those with an ipsilateral stroke with persistent disabling neurologic deficits, and those with 100% occlusion of the internal carotid artery. Surgical management of lower-grade (<70%) lesions is debated among expert panels and depends on sex of the patient and the presence or absence of symptoms. In many cases, patients with such lesions are managed medically only. For all patients with carotid artery disease, medical management includes aspirin, statin, and the control of atherosclerotic risk factors (eg, hypertension, hyperglycemia, smoking). (Choice A) Clopidogrel can be used in the medical management of patients with carotid stenosis who cannot tolerate aspirin (first-line therapy). This patient requires aspirin and surgical treatment with CEA. (Choice C) Extended ambulatory ECG monitoring can determine whether an intermittent arrhythmia (eg, atrial fibrillation) may be the underlying cause of TIA or stroke. However, patients do not typically require this test if there is a clear cause of TIA or stroke during initial evaluation (eg, brain imaging, carotid vessel imaging, ECG). (Choice D) Anticoagulation (eg, warfarin) is not generally recommended for patients with carotid artery stenosis. Anticoagulation is often used in patients with left ventricular thrombus or atrial fibrillation. (Choice E) Patent foramen ovale closure is recommended for patients with embolic-appearing cryptogenic strokes. However, closure is not required in patients who have a clear inciting cause (eg, symptomatic carotid artery disease) of TIA. Furthermore, this patient does not have evidence of deep vein thrombosis, and patent foramen ovale closure is currently recommended only in patients age <60. Educational objective: Symptomatic carotid artery stenosis is a common cause of stroke and transient ischemic attack. Patients with symptomatic high-grade (70%-99%) carotid lesions and life expectancy ≥5 years should be evaluated for carotid endarterectomy. Lower-grade (<70%) lesions may be managed either medically (eg, aspirin, statins, antihypertensives) or surgically, depending on the degree of stenosis and sex of the patient. References: Results of the Stent-Protected Angioplasty versus Carotid Endarterectomy (SPACE) study to treat symptomatic stenoses at 2 years: a multinational, prospective, randomised trial. (http://www.ncbi.nlm.nih.gov/pubmed/18774746) Best evidence for medical therapy for carotid artery stenosis. (http://www.ncbi.nlm.nih.gov/pubmed/24075112)

Question 4

An 18-hour-old girl is undergoing a routine examination in the newborn nursery.  The patient has urinated 4 times and passed meconium.  She was born via uncomplicated vaginal delivery at 41 weeks to a 23-year-old primipara.  Apgar scores were 8 and 9 at 1 and 5 minutes, respectively.  Birth weight and length are at the 25th percentile for gestation.  Temperature is 37.2 C (99 F).  The patient is initially asleep but easily roused.  Examination shows open and soft fontanelles, posteriorly rotated ears, and a small maxilla.  The soft palate has a high arch, but no submucosal cleft is felt on palpation.  The neck is short and webbed, without masses.  Auscultation reveals a regular heart rate and rhythm with no murmurs.  There is good air entry in all fields with no grunting, retractions, or nasal flaring.  External genitalia are normal.  The hands and feet are moderately edematous.  Crease lines on both hands are normal.  Pulses are 2+ in all extremities. The mother is concerned about the recurrence risk of this condition in subsequent pregnancies.  Which of the following is the best response?

Accepted Answer

Explanation: 11ec2b69_bfe4_4042_8f63_9990a1c8cb8f_MD0005_00 Turner syndrome occurs in approximately 1 in 2,500 live female births due to a complete or partial loss of an X chromosome. The majority of cases are caused by a random error in cell division, known as nondisjunction. If sex chromosomes fail to separate during egg or sperm formation, one gamete will lack an X chromosome, and fertilization will result in a 45,X zygote (monosomy X). Nondisjunction later during fetal development can result in 45,X mosaicism. Turner syndrome is considered a sporadic event, so the likelihood of recurrence in subsequent pregnancies is similar to that of the general population. nondisjunction (Choices A and D) The recurrence risk is approximately 50% in autosomal dominant disorders in which one parent has the disorder and 25% in autosomal recessive disorders in which both parents are carriers. Because Turner syndrome is not inherited, the recurrence risk for this mother is the same as in the general population. (Choice C) A mother who is a carrier of a chromosome 21 Robertsonian translocation has about a 10% risk of having a child with Down syndrome. Robertsonian translocations, which occur when the long arms of 2 chromosomes fuse, do not typically involve X chromosomes. (Choice E) The risk of nondisjunction resulting in trisomy 21 or Klinefelter syndrome (XXY) increases with maternal age; however, advanced maternal age is not a risk factor for Turner syndrome as the paternal X chromosome is missing in most cases. Educational objective: Turner syndrome is typically due to a random error in cell division, resulting in loss of an X chromosome. No increased recurrence risk is present after giving birth to an infant with Turner syndrome, and unlike other chromosomal nondisjunctions, the risk of 45,X does not increase with maternal age. References: Genetic considerations in the patient with Turner syndrome-45,X with or without mosaicism. (http://www.ncbi.nlm.nih.gov/pubmed/23020909)

Question 5

An 18-hour-old girl is undergoing a routine examination in the newborn nursery. The patient has urinated 4 times and passed meconium. She was born via uncomplicated vaginal delivery at 41 weeks to a 23-year-old primipara. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. Birth weight and length are at the 25th percentile for gestation. Temperature is 37.2 C (99 F). The patient is initially asleep but easily roused. Examination shows open and soft fontanelles, posteriorly rotated ears, and a small maxilla. The soft palate has a high arch, but no submucosal cleft is felt on palpation. The neck is short and webbed, without masses. Auscultation reveals a regular heart rate and rhythm with no murmurs. There is good air entry in all fields with no grunting, retractions, or nasal flaring. External genitalia are normal. The hands and feet are moderately edematous. Crease lines on both hands are normal. Pulses are 2+ in all extremities. Item 1 of 2 Which of the following is the most likely long-term outcome for patients with this condition?

Accepted Answer

Explanation: 11ec2b69_bfe3_cb10_8f63_7760e2b62931_MD0005_00 This newborn's webbed neck and congenital lymphedema of the hands and feet are concerning for Turner syndrome. Other distinctive features include a broad chest with widely spaced nipples, high-arched palate, and low posterior hairline. These key physical findings warrant urgent diagnostic confirmation with karyotype analysis. Once diagnosed, patients require 4-extremity blood pressure measurements and echocardiography to look for aortic coarctation, which, if unrecognized, can cause life-threatening cardiogenic shock in the newborn. Ultrasound should be ordered to screen for horseshoe kidney, which can predispose to urinary tract infections. Every few years during childhood, screening for hypothyroidism and celiac disease should occur due to an increased risk of these autoimmune conditions. In addition, because of an increased risk of learning disabilities, neuropsychological testing is performed prior to school entry to ensure that appropriate therapies can be provided if needed. However, normal intelligence is expected. (Choice A) Autism spectrum disorder often occurs in patients with fragile X syndrome. This disorder is more common in boys and classically causes long, narrow faces, large ears, and large testicles. (Choice B) Developmental regression, as well as microcephaly, epilepsy, and stereotypic hand movements, typically develops in Rett syndrome. Girls with this disorder appear normal at birth and have no dysmorphic features. (Choice C) Early-onset Alzheimer dementia is associated with trisomy 21, which is characterized by upslanting palpebral fissures, epicanthal folds, and a transverse palmar crease, none of which are seen in this patient. (Choice D) Epilepsy is common in many syndromes, such as trisomies 13 and 18 and Rett syndrome. However, it is not a typical comorbidity in Turner syndrome. Educational objective: Characteristic features of Turner syndrome include a webbed neck and congenital lymphedema. Normal intelligence is expected, but patients should be monitored for increased risk of learning disabilities. References: Cognition and behavior in Turner syndrome: a brief review. (http://www.ncbi.nlm.nih.gov/pubmed/22946281)

Question 6

A 56-year-old man comes to the physician with fatigue, bilateral knee and ankle pain, and left-sided chest pain.  He has awakened in an uncomfortable sweat several nights during the last 4 weeks.  He experienced exertional shortness of breath and was diagnosed with non-ischemic cardiomyopathy a year ago.  During his initial evaluation, his left ventricular ejection fraction was 20%, and cardiac catheterization showed no significant coronary disease.  The patient's medications include carvedilol, hydralazine, isosorbide dinitrate, furosemide, and spironolactone.  He received an automatic implantable cardioverter-defibrillator for primary prevention of cardiac death 6 months ago.  He is allergic to angiotensin-converting enzyme inhibitors, which caused facial angioedema.  His blood pressure is 142/90 mm Hg and his heart rate is 65/min and regular.  There is no jugular venous distension.  Breath sounds are decreased at the left base due to splinting.  There is no splenomegaly.  There is a small effusion in the right knee with mild tenderness.  There are no skin rashes.  Chest x-ray shows cardiomegaly and a small left pleural effusion.  Which of the following is the best next step in management of this patient?

Accepted Answer

Explanation: This patient's presentation - fatigue, night sweats, arthralgia/arthritis, and pleuritis/pleural effusion - is suggestive of drug-induced lupus due to hydralazine. It is seen in approximately 5%-10% of patients receiving hydralazine and occurs due to development of autoantibodies that trigger an autoimmune response and a clinical syndrome similar to systemic lupus erythematosus. Other autoantibodies, with the exception of anti-histone antibodies, are uncommon in this disorder. Drug-induced lupus causes multisystem involvement, and clinical findings can include fever, malaise, myalgia, arthralgia/arthritis, serositis (pleuritis, pericarditis), and/or hepatomegaly/splenomegaly. The diagnosis is made by a history of one or more offending drugs and a positive test for antinuclear antibodies (ANAs). Some common drugs associated with risk of lupus include procainamide, hydralazine, minocycline, and anti-tumor necrosis factor (TNF) alpha therapy (etanercept, infliximab). Treatment is generally symptomatic, and discontinuation of the offending drug typically leads to resolution of symptoms within weeks to months. (Choice B) The patient is tolerating diuretics quite well without side effects and has compensated nonischemic cardiomyopathy. Discontinuation of diuretics can lead to worsening congestive heart failure. (Choice C) The patient had no significant coronary disease on cardiac catheterization and has no symptoms of progressive coronary artery disease. Exercise echocardiography is not indicated at this point. (Choice D) Oral doxycycline is used for the treatment of Lyme disease. Lyme disease is a tick-borne illness that typically presents with a characteristic skin rash and erythema migrans at the site of the bite in the early-localized phase. The next phase, early-disseminated disease, is characterized by multiple erythema migrans lesions. In addition, neurologic (eg, peripheral neuropathy, cranial nerve palsies, lymphocytic meningitis) and/or cardiac involvement (eg, myopericarditis, atrioventricular block) occurs weeks to months after the initial infection. Late disease (months to years) is associated with arthritis of the large joints and mild neurological manifestations (encephalopathy or polyneuropathy). This patient's clinical presentation is not consistent with Lyme disease. (Choice E) Troponin I levels are typically elevated in patients with acute or recent myocardial infarction. Measurement of brain natriuretic peptide (BNP) levels is useful in the evaluation of patients with symptomatic left ventricular dysfunction and heart failure. This patient's presentation is suggestive of drug-induced lupus, and measurement of troponin or BNP levels is not useful. Educational objective: Drug-induced lupus causes multisystem involvement; clinical findings include fever, malaise, myalgia, arthralgia/arthritis, serositis (pleuritis, pericarditis), and/or hepatomegaly/splenomegaly. The diagnosis is made by typical symptoms, history of taking an offending drug, and a positive test for antinuclear and anti-histone antibodies. References: Drug-induced lupus erythematosus. (http://www.ncbi.nlm.nih.gov/pubmed/16373256) Drug-induced arthritic and connective tissue disorders. (http://www.ncbi.nlm.nih.gov/pubmed/18166218)

Question 7

A 78-year-old healthy, Chinese-American man is brought to the emergency department because of hallucinations, delusions, and aggressive behavior, according to his nephew.  He has had these symptoms for the past six days.  He denies fever, chills, decreased vision, headaches, or abdominal pain.  His other medical problems include hypertension, coronary artery disease, and osteoporosis.  He does not use tobacco or drink alcohol.  He is a vegan.  He lives alone.  His medications include aspirin, hydrochlorothiazide, enalapril, acetaminophen, and diclofenac.  He has no known drug allergies.  His blood pressure is 141/88 mmHg, pulse is 100/min, temperature is 37.2 C (99 F), and respirations are 14/min.  Examination shows dry mucus membranes and a loud second heart sound.  The abdomen is soft, nontender, and non-distended.  Bowel sounds are mildly increased.  There is no rebound tenderness or rigidity.  The range of motion of the spine is normal and without pain.  There is no scoliosis or excessive kyphosis of the back.  There is no tenderness to palpation of the lower back.  The mental status examination reveals an agitated, elderly man with delusional ideation and bizarre behavior.  There are no motor or sensory focal deficits, and no meningeal signs.  The patient's labs reveal:   His chest-x ray and urinalysis results are normal.  Which of the following is the most appropriate pharmacotherapy?

Accepted Answer

Explanation: Vitamin B12 deficiency can present in a subtle manner in the elderly patient. Some patients do not even have anemia, but the majority have an increase in the mean corpuscular volume (MCV). Megaloblastic anemia can be secondary to folic acid or vitamin B12 deficiency, and is characterized by an increase in indirect bilirubin due to ineffective erythropoiesis, as well as pancytopenia or bicytopenia, as in this case. The neurologic presentation includes ataxia, dementia, and occasionally, delirium. (Choice A)Folic acid deficiency is not related to any neurologic abnormalities. (Choice D)Clinically, this is not a case of thiamine deficiency. Thiamine deficiency presents with a classical triad of encephalopathy, oculomotor dysfunction, and gait ataxia, and is usually seen in alcoholics. Furthermore, anemia and elevated MCV are not seen in thiamine deficiency. (Choice E)Even though infection is one of the most common causes of delirium in the elderly, this patient has no other signs or symptoms of infection. He is afebrile. He has no left shift, and his white count is not elevated. (Choice B)Although the patient has pancytopenia, there is no evidence of malignancy. Even if there were malignancy, aggressive chemotherapy could be especially dangerous for a patient of this age. Educational Objective: It is important to recognize vitamin B12 deficiency as a potential cause of delirium or dementia in the elderly patient. Subtle laboratory findings, such as increased MCV and reticulocytes, or pancytopenia, can help to identify this condition.

Question 8

A 45-year-old man suffers multiple injuries in a motor vehicle accident.  He undergoes several surgeries and has a long stay in the intensive care unit (ICU) complicated by hospital-acquired pneumonia, gastrointestinal bleeding, and deep venous thrombosis.  After 30 days in the ICU, he is deemed ready to be transferred to the general medical ward.  Which of the following is the most important aspect of patient handoff from the ICU team to medical team?

Accepted Answer

Explanation: 11ec2b69_bfe3_07be_8f63_5769b2c61a51_MD0005_00 A number of standardized procedures have been developed for safe and effective patient handoffs between providers (on the same clinical team or on different clinical services). All handoffs should contain pertinent information about the patient's demographics and clinical status, care plan, anticipated problems, and pending actions or results that require follow-up. Listing anticipated problems (and possible courses of action) is especially important as it allows the receiving providers to take faster, more effective action if any of these complications arise. (Choice A) Although avoiding redundancy can make handoffs quicker, including the proper information (eg, anticipated problems) is more important for high-quality patient care. For some high-risk processes (eg, drug allergies), incorporating redundancy into the system may in fact be preferable. (Choice B) Briefly relaying the patient's history and hospital course is important, but a detailed description of surgical procedures is excessive information that complicates effective patient handoffs, especially between non-surgical caregivers. (Choice C) Limiting verbal communication harms patient handoff as it does not give the receiving providers an opportunity to clarify and ask questions about the patient. Studies have shown that most effective handoffs occur when the various providers are face-to-face and have an opportunity to address any questions or concerns in real time. (Choice E) Ideally, handoffs should occur using a common standardized handoff tool. Changing the structure and flow of handoffs creates more errors due to the increased likelihood that important information will be excluded. Educational objective: Standardized procedures have been developed for safe and effective patient handoffs. All handoffs should contain pertinent information about the patient's demographics and clinical status, care plan, expected/anticipated problems, and pending actions or results that require follow-up. References: The Veterans Affairs shift change physician-to-physician handoff project. (http://www.ncbi.nlm.nih.gov/pubmed/20180438)

Question 9

An 85-year-old man is evaluated at a nursing home due to intermittent agitation.  The patient has dementia and has been residing in the facility for several years.  He can communicate his needs but does not recognize his caregivers and requires assistance with all activities of daily living.  According to the staff, the patient has had intermittent agitation and aggression; these have become somewhat more frequent over time.  The patient is unable to provide meaningful history, but there is no report of fever, cough, abdominal pain, urinary symptoms, or change in appetite.  Records indicate a history of hypertension but no cerebrovascular accidents or Parkinson disease.  His only medications are olanzapine and haloperidol as needed for agitation.  Temperature is 37 C (98.6 F), blood pressure is 130/70 mm Hg, and pulse is 78/min.  The patient is comfortable but confused and oriented to self only.  Lungs are clear to auscultation and heart sounds are normal.  The abdomen is soft and nontender with normoactive bowel sounds.  Neurological examination reveals mild resting hand tremors and rigidity of both arms.  There is no extremity edema or rash.  Which of the following is the most appropriate next step in management of this patient?

Accepted Answer

Explanation: This elderly man with dementia and intermittent agitation, for which he is on multiple antipsychotics, now has resting tremors and upper extremity rigidity, suggestive of drug-induced parkinsonism due to antipsychotics (haloperidol, olanzapine). In patients with dementia, neuropsychiatric symptoms (eg, agitation, aggression, hallucinations) are common and may become more pronounced, particularly at night (sundowning), as the disease progresses. Treatment options include behavioral interventions (eg, activities, music therapy, communication skills training for caregivers), antidementia drugs, and antidepressants. Although antipsychotics are sometimes used (particularly in severe or refractory cases), they are associated with increased mortality and multiple side effects, including extrapyramidal symptoms (eg, secondary parkinsonism, akathisia, dystonias). Secondary parkinsonism causes cogwheel rigidity, "pill-rolling" resting tremors, masked facies, shuffling gait, and bradykinesia, many of which are seen in this patient. Because this patient has significant side effects and only intermittent agitation (likely due to dementia progression), his antipsychotic regimen should be stopped, and behavior interventions to treat his agitation should be implemented. Antipsychotics are typically reserved for patients in whom the safety of the patient or caretaker is threatened; their use should be minimized. (Choice A) Increasing this patient's antipsychotic regimen will likely worsen his secondary parkinsonism. In patients with suspected primary Parkinson disease, haloperidol should be avoided as it can exacerbate symptoms. (Choice B) Levodopa and carbidopa are used to treat motor disturbances in primary Parkinson disease. However, Parkinson disease begins with motor symptoms, and patients develop dementia late in the disease, unlike this patient who developed parkinsonian features years after dementia. Levodopa and carbidopa are not indicated in secondary parkinsonism caused by antipsychotics. (Choice C) Lewy body dementia causes parkinsonian symptoms, dementia, and increased sensitivity to neuroleptic medications (eg, exacerbation of parkinsonism, confusion with neuroleptic agents). However, these patients develop visual hallucinations, autonomic dysfunction (eg, orthostatic hypotension, urinary incontinence), and fluctuations in levels of cognition (eg, episodes of "blanking out," bizarre behavior, excessive somnolence), which are not noted in this patient. Haloperidol is contraindicated in patients with Lewy body dementia due to neuroleptic sensitivity. (Choice D) In patients who are elderly or have dementia, urinary tract infections can cause worsening agitation with minimal urinary symptoms. However, they would not be expected to cause parkinsonian symptoms, and it is unlikely that an untreated urinary tract infection would last 2 months without the patient experiencing marked clinical deterioration. Educational objective: Neuropsychiatric symptoms are common among patients with dementia; treatment includes behavioral interventions, antidementia drugs, and antidepressants. Antipsychotics are sometimes used but are associated with increased mortality and multiple side effects, including extrapyramidal symptoms. If possible, antipsychotics should be discontinued in patients who develop side effects.

Question 10

A 62-year-old Caucasian man comes to the physician because of progressive weight loss and right lower extremity weakness for the last two months.  On further questioning, he tells you that he has been having frequent leg cramps in the right leg.  These cramps are most prominent in the morning, after he wakes up.  He also has difficulty with swallowing and chewing.  He denies any problems with bowel or bladder function.  His other medical problems include hypertension and hypercholesterolemia.  He does not use tobacco.  His family history is not significant.  His vital signs are within normal limits.  The physical examination reveals weakness in his right lower leg, atrophy and fasciculation of the thigh and calf muscles, a hyperactive knee jerk, and tongue fasciculations.  The rest of his neurological examination, including the sensory examination, is normal.
Which of the following treatment has been shown to be beneficial in these patients?

Accepted Answer

Explanation: Riluzole is a glutamate inhibitor that is currently approved for the management of amyotrophic lateral sclerosis. Although it cannot arrest the underlying pathological process, it may prolong survival and delay the need for a tracheostomy. (Choices B, C, and D)Amyotrophic lateral sclerosis is a neurodegenerative disease. Agents such as corticosteroids, intravenous immunoglobulins, and cyclophosphamide, have no role in its management. These agents may be useful in immunologically-mediated neurological diseases such as multiple sclerosis and Guillain-Barre syndrome. (Corticosteroids are used to treat acute exacerbations of multiple sclerosis, while plasmapheresis and intravenous immunoglobulins are the main treatment modalities of Guillain-Barre syndrome.) Educational Objective: Riluzole is a glutamate inhibitor that is currently approved for the management of amyotrophic lateral sclerosis.

Question 11

A 73-year-old woman is brought to the office by her daughter due to worsening memory. The patient began having difficulty with her memory 2 years ago but was mostly independent until recently. Over the past 2 months, she has gotten lost several times while driving locally. She can no longer be allowed to cook for herself because she has frequently forgotten to turn off the stove. The patient has hypertension, gastroesophageal reflux disease, and alcohol use disorder. Her medications include lisinopril and ranitidine. Family history is negative for dementia. Vital signs and physical examination are within normal limits. TSH and vitamin B₁₂ levels are normal. Montreal Cognitive Assessment is 22 out of 30 (normal: ≥26). Which of the following is the most appropriate next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_bfe5_0394_8f63_51fdcb7df770_MD0005_00 This patient's progressive cognitive decline and resultant functional impairment (ie, deficits in activities of daily living) are concerning for a diagnosis of dementia (also known as major neurocognitive disorder). Before a diagnosis of a specific dementing syndrome can be made, reversible causes of dementia must be ruled out. Routine laboratory testing includes complete blood count, complete metabolic panel, TSH, and vitamin B₁₂ levels; specific at-risk populations should receive additional testing. This patient's workup should include a folate level as her alcohol use disorder places her at risk for folate deficiency and resultant cognitive impairment. Although thiamine deficiency and subsequent Wernicke-Korsakoff syndrome may also lead to cognitive decline in patients with alcoholism, suspected cases are generally treated empirically as the utility of laboratory tests for thiamine is low. (Choice A) Apolipoprotein E polymporphisms are associated with Alzheimer dementia. However, genetic testing is currently not recommended for the routine evaluation of dementia as it exposes patients to the psychological harms of false positives and false negatives without providing significant clinical utility. (Choice C) The most common neurological manifestations of Lyme disease are meningitis, radiculoneuritis, and cranial neuropathy. Testing for Lyme disease would be low yield in this patient without the presence of a symptom constellation consistent with the illness or a specific history (eg, tick bite). (Choices D and E) Testing for syphilis and vitamin D deficiency is currently recommended in only specific at-risk populations, such as patients with a known syphilis exposure or those suffering from nephrotic syndrome, respectively. Educational objective: The initial laboratory workup to rule out reversible causes of cognitive impairment includes complete blood count, complete metabolic panel, TSH, and vitamin B₁₂ levels. Selective testing of folate and for syphilis or vitamin D deficiency is indicated for patients with specific risk factors; patients with alcoholism are at increased risk for folate deficiency. ₁₂ References: Evaluation of suspected dementia. (http://www.ncbi.nlm.nih.gov/pubmed/22010769) Dementia for the primary care provider. (http://www.ncbi.nlm.nih.gov/pubmed/28797371) Modifiable predictors of dementia in mild cognitive impairment: a systematic review and meta-analysis. (http://www.ncbi.nlm.nih.gov/pubmed/25698435)

Question 12

A 32-year-old Caucasian man comes to the office for a routine follow-up visit.  He was diagnosed with epilepsy three years ago, and has been taking carbamazepine 800 mg/day ever since.  He does not use tobacco, alcohol or illicit drugs.  His family history is not significant.  Physical examination shows no abnormalities.  The patient is concerned about the potential side effects of his medication.  Which of the following complications is this patient at greatest risk for?

Accepted Answer

Explanation: Carbamazepine can cause bone marrow suppression. Patients should be made aware of early symptoms such as fever, mouth ulcers, easy bruising or petechiae, which can be markers of the development of neutropenia, aplastic anemia or thrombocytopenia. (Choice A) There is a risk of thrombocytopenia, not thrombosis. (Choice B) Pulmonary fibrosis or interstitial lung disease has not been associated with the use of this drug. (Choice D) Urinary complications include renal failure or urinary retention, not the development of kidney stones. (Choice E) Carbamazepine use has been associated with hyponatremia and SIADH, not hypernatremia. Educational objective: Although the frequency is not clear, neutropenia and bone marrow suppression due to carbamazepine use are significant adverse reactions. Elderly patients are also at risk of SIADH. Because of some mild anticholinergic effects, there is a risk of glaucoma, urinary retention or constipation.

Question 13

An 81-year-old woman is brought to the office for assessment of behavioral changes over the past 10 months. The patient moved to a nursing home a year ago, and initially enjoyed playing simple card games there with other residents. Now she either seems uninterested in playing or quickly becomes upset during games, repeating, "I can't, I can't, I can't." She also becomes easily irritable and pushes staff away when they try to help her dress or comb her hair. The patient has had increasing difficulty recalling recent events and has started accusing staff of stealing her jewelry. Sleep and appetite are unchanged. The patient has a history of hypertension and noninsulin-dependent diabetes mellitus, as well as postpartum depression in her 20s. Temperature is 37 C (98.6 F), blood pressure is 140/90 mm Hg, and pulse is 86/min. Physical examination is unremarkable. She is oriented to person and place but not to time or day. Short-term recall is very poor. Her mood is "OK." Laboratory results, including electrolytes, complete blood count, thyroid function tests, and vitamin B₁₂, are normal. Rapid plasma reagin is negative; fluorescent treponemal antibody absorption test is nonreactive. MRI of the brain reveals mild generalized cortical atrophy and slightly reduced hippocampal volume. Which of the following is the most appropriate statement regarding this patient's prognosis?

Accepted Answer

Explanation: 11ec2b69_bfe5_78c5_8f63_514c80149a33_MD0005_00 This patient has gradually progressive symptoms (eg, poor memory, apraxia, language difficulties, executive dysfunction) that suggest a diagnosis of Alzheimer disease (AD). Onset occurs at age >60 and initially affects memory and language predominantly. Later in the course of the illness, behavioral symptoms become more prominent. Patients typically become more easily irritable, especially when frustrated or overwhelmed. Paranoid delusions are common and usually due to misattribution. Cholinesterase inhibitors can sometimes slow the progression of AD, but there is no cure. The disease is progressive and the patient may eventually die from complications of an infection, dehydration, or malnutrition. The differential diagnosis for AD includes other dementing illnesses and cognitive changes due to delirium or psychiatric illness (eg, depression). This patient's lack of other depressive symptoms (eg, sleep and appetite are unchanged, mood is not depressed), stable medical condition, and progressive decline over 10 months make AD more likely. dementing illnesses (Choice A) This patient's agitated behavior and delusions of items being stolen are common in moderate to severe dementia (often due to patients forgetting where an item was placed). Behavioral symptoms in AD are ideally managed with nonpharmacological interventions, with antipsychotics reserved for situations in which agitated behaviors pose a risk to self or others. (Choice C) In the absence of syndromal depression, antidepressants are unlikely to improve this patient's condition. When cognitive impairments are due to depression, antidepressants can help to reverse these deficits. (Choice D) Stepwise decline occurs with vascular dementia, which is characterized by stepwise cognitive decline as well as focal neurological symptoms. Brain imaging may reveal multiple small cortical and subcortical infarcts. The patient has hypertension and diabetes as risk factors but no findings consistent with vascular dementia. (Choice E) Delirium is always secondary to an underlying medical process or drug effect; the patient's mental status improves once it resolves. Delirium can also manifest with delusions, memory impairment, or agitation. The hallmark of delirium is acute onset of altered level of consciousness with difficulty in sustaining attention, which is not present in this patient. Educational objective: Alzheimer disease is a progressive illness characterized by memory loss and functional decline across several cognitive domains. Medications may slow progression, but there is no cure. References: Neurocognitive disorders in DSM-5: pervasive changes in the diagnostics of dementia. (http://www.ncbi.nlm.nih.gov/pubmed/24744097) Differential diagnosis of dementia. (http://www.ncbi.nlm.nih.gov/pubmed/19351220)

Question 14

A 54-year-old Caucasian chef presents to clinic complaining about "a new problem with really bad dizziness."  She says that when she first sat up in bed this morning, she suddenly felt very unstable and as if her body was spinning in space.  She was overcome by nausea but did not vomit, and then the episode passed after approximately one minute.  She had similar episode when she was working in the restaurant kitchen.  She has not been ill recently or in contact with any ill people to her knowledge.  Her past medical history is significant for mild chronic obstructive pulmonary disease and ulcerative colitis.  Current medications include albuterol, prednisone, mesalamine, and ibuprofen.  She has a remote thirty pack-year history of cigarette smoking and drinks alcohol only on social occasions.  Her temperature is 36.8C (98F), blood pressure is 124/72 mm Hg (sitting) and 120/68 mm Hg (standing), pulse is 75/min, and respirations are 14/min.  Positional nystagmus is noted on physical examination.  Which of the following measures is the most appropriate course of action?

Accepted Answer

Explanation: Benign paroxysmal positional vertigo (BPPV) is defined as an abnormal feeling of motion triggered by certain provocative positions. The condition is most often attributed to canalithiasis, or the presence of calcium "rocks" within the posterior semicircular canal. If there is substantial debris in the canal, then linear accelerations (eg, gravity) may cause the endolymph to move inappropriately. This results in the inaccurate sensation of spinning subsequent to movement of the head. Although the condition is often idiopathic, it can also be associated with head trauma or vestibular pathologies. BPPV should first be treated with the canalith repositioning procedure (Choice C), which is a series of maneuvers that move particles out of the posterior semicircular canal and into the utricle. BPPV is a clinical diagnosis. Further testing is not indicated with typical posterior canal BPPV presentation. Imaging of the head (Choices A and B) is indicated if a mass lesion, hemorrhage, stroke, or Meniere's disease is thought responsible for the patient's vertigo. Promethazine (Choice D) is a nonselective antihistamine used in the treatment of nausea or vomiting. It may be of help in patients with vertigo but addresses only this particular symptom and not the cause. Plugging of the posterior canal (Choice E) is a surgical procedure used in patients with intractable symptoms of BPPV. In most cases, the procedure reduces the functionality of the posterior canal without affecting hearing. Educational Objective: Benign paroxysmal positional vertigo should first be treated with the canalith repositioning procedure, which is a series of maneuvers that moves particles out of the posterior semicircular canal and into the utricle. References: Management of benign paroxysmal positional vertigo with the canalith repositioning maneuver in the emergency department setting. (http://www.ncbi.nlm.nih.gov/pubmed/21068225) Benign paroxysmal positional vertigo. (http://www.ncbi.nlm.nih.gov/pubmed/20607044)

Question 15

A seven-year-old female is being evaluated in the pediatric clinic for short stature.  Her medical records reveal that she was small at birth.  When she was approximately two years of age, her parents noticed that she was not growing as much as her peers.  Her height and weight are below the 5th percentile on the normal growth chart.  Her projected height is more than 2 standard deviations below her mid-parental height.  She has short fourth metacarpal bones bilaterally and multiple nuchal folds.  Her carrying angles in the upper extremities are increased.  A diagnosis of Turner syndrome is suspected.  Karyotyping is performed and the result is 45, XO.  At this time, which of the following is necessary in the management of this patient?

Accepted Answer

Explanation: The patient has classic Turner syndrome with 45,XO karyotype. This disease is characterized by short stature and hypogonadism. All patients who are initially diagnosed must be screened for the presence of other associated somatic abnormalities. The most important of these are the cardiac defects, which include coarctation of the aorta, bicuspid aortic valve, mitral valve prolapse, and hypoplastic heart. Since some of these defects cannot be picked up by clinical examination, an echocardiogram is necessary. The other associated defects are: visual and hearing deficits, kidney malformation (including horseshoe shaped kidney), and an increased predisposition for autoimmune endocrinopathy (especially primary hypothyroidism); therefore, in addition to an echocardiogram, all patients initially diagnosed with Turner syndrome require visual and hearing assessment, renal ultrasound, and TSH level measurement. (Choice B) Streak gonads in Turner's syndrome increase the patient's chances for malignancy when there is an associated mosaicism with a Y chromosome. In this case, there is no evidence of Y chromosome mosaicism; therefore, removal of the streak gonads is not necessary. (Choice C)Patients with Turner syndrome develop moderate to severe insulin resistance and diabetes when they are older. Blood sugar screening is performed based on the patient's clinical manifestations. Since this patient is very young and has no suggestive manifestations, screening for diabetes mellitus is not required at this time. (Choice D)Patients with Turner syndrome have hypogonadism, and eventually require estrogen replacement; however, if estrogen therapy is given at an early age, there may be premature fusion of the epiphysis, which will potentially decrease the patient's final height. Most physicians begin prescribing hormone replacement therapy when the patient reaches 14 years of age. Growth hormone is approved for use in patients with Turner's syndrome to improve their final height. (Choice E)Majority of patients with Turner's syndrome does not have significant pulmonary abnormalities. Pulmonary function tests are not required unless clinically indicated. Educational Objective: In addition to short stature and hypogonadism, patients with Turner syndrome may have several other somatic abnormalities, such as cardiac defects, visual impairment, hearing disability, hypothyroidism, and kidney malformations. Screening for these disorders is necessary at the time of diagnosis.

Question 16

A 79-year-old man is brought to the office by his daughter. The patient has been having increasing difficulty caring for himself over the last 2 years. He is now dependent on his daughter for meal preparation, shopping, and cleaning. He will frequently go a week without bathing and often has difficulty dressing himself appropriately. The patient sometimes grows frustrated and shouts at his daughter but has never been physically violent. On direct questioning, he states that he feels fine, does not see any reason for coming to the office, and is quite suspicious of his daughter's motives. The patient has lived alone since his wife died 5 years ago. He has hypertension, osteoarthritis, and a history of remitted major depressive disorder. Medications include hydrochlorothiazide and acetaminophen as needed. Neurologic examination is nonfocal. Item 1 of 2 Which of the following is the most appropriate next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_bfe6_1506_8f63_75ae76ebeac1_MD0005_00 This patient's presentation is concerning for dementia (ie, major neurocognitive disorder). Dementia should be suspected in patients with evidence of progressive cognitive decline that leads to functional impairment in activities of daily living. All patients with suspected dementia should undergo routine cognitive testing (eg, Montreal Cognitive Assessment) to establish an objective measure of cognitive deficit. Although <1% of dementia cases are considered reversible, workup should include routine labs-complete blood count, complete metabolic panel, TSH, and vitamin B₁₂-to rule out reversible causes. Selected cases may warrant additional labs, including folate, syphilis antibodies, vitamin D, and/or cerebrospinal fluid (CSF) studies. Most clinicians also routinely obtain an MRI of the brain as part of the workup. Thiamine deficiency and subsequent Wernicke-Korsakoff syndrome may also lead to cognitive decline; however, suspected cases are generally treated empirically as the utility of lab tests for thiamine is low. (Choice A) Genetic testing is currently not indicated for evaluation of Alzheimer disease. (Choices B and C) Patients with atypical features of dementia (eg, age <60, rapidly progressive dementia) may benefit from an electroencephalogram and/or lumbar puncture for CSF studies. (Choice D) Serum folate testing is recommended only in patients with known risk factors (eg, those taking the antifolate drug methotrexate). Educational objective: Although cases of reversible dementia are rare (ie, <1%), clinicians must rule out reversible causes prior to making a diagnosis of a specific dementia syndrome. The initial laboratory workup should include TSH, complete blood count, complete metabolic panel, and vitamin B₁₂ levels. ₁₂ References: Dementia for the primary care provider. (http://www.ncbi.nlm.nih.gov/pubmed/28797371) Rapidly progressive dementia. (http://www.ncbi.nlm.nih.gov/pubmed/27042906) The use of MRI, CT and lumbar puncture in dementia diagnostics: data from the SveDem Registry. (http://www.ncbi.nlm.nih.gov/pubmed/25358376)

Question 17

A 62-year-old woman collapses at home after a period of shortness of breath and foaming at the mouth.  She has no respirations or pulse when the emergency response team arrives 30 minutes later.  The electrocardiogram (ECG) shows asystole.  Cardiopulmonary resuscitation with advanced cardiac life support is initiated.  The patient is intubated and given epinephrine with restoration of a pulse.  She is transferred to the emergency department in a comatose state.  ECG shows Q waves in the inferior leads, first-degree atrioventricular block, and a right bundle branch block.  Initial laboratory results are as follows:  The patient is admitted to the intensive care unit.  She remains comatose 24 hours later.  Her blood pressure is 108/60 mm Hg, and core temperature is 36.7 C (98 F).  There is no motor response to firm supraorbital pressure.  Her pupils are fixed and dilated at 8 mm.  Corneal reflexes are absent.  Caloric testing produces no conjugate eye deviation.  Deep tracheal suctioning produces no cough reflex.  A brain CT scan demonstrates loss of the gray/white junction, loss of sulci, and obliterated ventricles.  Which of the following is the most appropriate next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_bfea_5ac9_8f63_c54cff2ec0d9_MD0005_00 Clinical criteria suggesting brain death include clinical/brain imaging evidence of a devastating cause, absence of confounding factors (eg, major endocrine or electrolyte disturbances, drug intoxication), and hemodynamic stability (core body temperature >36 C [97 F], systolic blood pressure >100 mm Hg). Patients with positive clinical criteria should undergo neurologic examination to document absent cerebral and brainstem reflexes, including coma, absent motor response to pain, absent pupillary light reflex, absent corneal and oculocephalic reflexes, and absent cough reflex with tracheal suctioning. Patients who may not meet all the clinical criteria but who have suspected brain death should undergo ancillary testing to document absent brainstem activity (eg, electroencephalography) or cerebral blood flow (eg, CT angiography). Patients with positive clinical criteria and abnormal neurologic findings should undergo apnea testing to confirm brainstem failure. Baseline normal PaCO₂ and PaO₂ are required; apnea tests should be avoided in chronic CO₂ retainers. Patients should be pre-oxygenated and then disconnected from the ventilator. Absence of a respiratory response (in a typical observation period of 8−10 minutes) with a PaCO₂ >60 mm Hg (or >20 mm Hg from baseline) and a final arterial pH of <7.28 are considered a positive apnea test. Brain death is diagnosed in patients with a positive apnea test if they also meet local legal requirements (eg, repeat neurologic examination in 24 hours, another neurologist's assessment to confirm brain death). Patients with apnea testing that is difficult to interpret or inconclusive should also undergo ancillary testing. Patients with positive ancillary testing and within local legal regulations are also considered brain dead. This patient has clinical criteria for brain death, including CT findings consistent with sequelae of severe global hypoperfusion likely due to prolonged hypotension. She also has positive neurologic findings (coma; absent corneal, caloric, and gag reflexes; fixed and dilated pupils). The next step is an apnea test. (Choice A) An apnea test is required before the diagnosis of brain death can be made. (Choices B and C) If the apnea testing is inconclusive, the patient may need further ancillary testing, including brain imaging (to exclude cerebral blood flow) or electroencephalography. (Choice E) After positive apnea testing or ancillary testing, there may be local legal requirements (eg, repeat neurological examination in 24 hours) to satisfy criteria for brain death. Educational objective: Brain death diagnosis requires a central nervous system catastrophe of known etiology with an absence of confounding factors (eg, endocrine or electrolyte disturbances, drug intoxication) or hypothermia. Patients meeting clinical criteria should undergo neurologic examination to document absent cortical and brainstem function (eg, coma, absent motor response to pain, absent pupillary light reflex, absent corneal and oculocephalic reflexes, absent gag reflex). Apnea testing is used to confirm the diagnosis of brain death in patients meeting these criteria. References: Diagnosis of brain death. (http://www.ncbi.nlm.nih.gov/pubmed/21577338)

Question 18

A 24-year-old female is brought to the emergency department (ED) after an episode of witnessed seizure activity in her home.  Her mother says that she has a history of generalized tonic-clonic seizures.  She has been seizure-free for the past six years.  She stopped taking all her medications two years ago.  Her only current daily medication includes a multivitamin and an oral contraceptive pill.  At the ED, she is given a loading dose of IV fosphenytoin and started on maintenance doses of oral phenytoin.  Over the next three days, she does not have any further seizures.  On the fourth day, she has developed a horizontal nystagmus.  Her total phenytoin level on the same day is 20 mcg/mL (normal range is10-20 mcg/mL).  Repeat blood work returns with the same value.  Which of the following is the most appropriate next step?

Accepted Answer

Explanation: It is important to recognize the early signs and symptoms of phenytoin toxicity. The earliest sign is the presence of nystagmus on far lateral gaze. Some other effects include blurred vision, diplopia, ataxia, slurred speech, dizziness, drowsiness, lethargy, and decreased mentation, which progresses to coma. Systemic side effects and neurotoxicity is one of the major limitations to the use of phenytoin. Usual therapeutic range of phenytoin is between 10-20 mcg/mL and most patients will experience adverse dose related neurotoxic effects with levels greater than 20 mcg/mL. However, the serum levels associated with neurotoxicity vary from patient to patient. Some patients can experience side effects even when the measured levels are within the normal therapeutic range. The first step in the management of side effects due to higher drug levels is to reduce the dose or alter the treatment schedule to minimize the peak drug levels. Therefore, in this vignette, the dose of phenytoin should be reduced and patient should be observed for resolution of nystagmus. (Choices A and B) Abrupt cessation of an antiepileptic drug is not recommended. Any antiepileptic drug that needs to be withdrawn due to intolerable side effects should be tapered gradually (over days to weeks) to prevent seizure relapse. (Choice C) The dosage of the drug should be reduced to prevent further toxicity. Observation alone is not appropriate. (Choice D) Oral contraceptive pills do not affect the drug level of phenytoin. Conversely, chronic phenytoin therapy can cause failure in contraception by enhancing the metabolism of oral contraceptive pills through the induction of hepatic enzymes. Educational objective: Patients with signs and symptoms of phenytoin toxicity should be initially managed by altering the drug dosage or treatment schedule to minimize drug peak levels. References: Clinical features and management of poisoning due to phenytoin. (http://www.ncbi.nlm.nih.gov/pubmed/2671594)

Question 19

A 42-year-old woman comes to the office due to 6 months of worsening headaches. The patient has had occasional headaches in the past, but lately "they seem to be getting out of control" and are often throbbing. She also has constant, pressure-like headaches localized behind the forehead and eyes. The patient takes acetaminophen and ibuprofen and recently began taking them on a daily basis. The medications often make her nauseated, and she has had several episodes of vomiting. The patient also has blurry vision but no muscle weakness, syncope, or seizures. She experiences a considerable amount of stress at work and says that she "cannot afford to slow down." Medical history is significant for irritable bowel syndrome and chronic back pain. She smokes a pack of cigarettes daily. The patient's mother suffered from migraines and had surgery for a meningioma. Her father died of a drug overdose. Blood pressure is 155/98 mm Hg and pulse is 83/min. BMI is 27 kg/m². Chest examination is unremarkable. Abdominal examination shows mild periumbilical tenderness. There is no muscle weakness, and the deep tendon reflexes are symmetric. There is no hand tremor. Romberg test is negative. Funduscopic findings are shown in the image below. Which of the following is the most appropriate next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_bfeb_1e1b_8f63_ef8ca2fe9832_MD0005_00 Headache can be divided into primary (eg, migraine, tension) and secondary (eg, tumor, infection) causes. Patients without red flag features may be treated for primary causes, but those with such features (eg, fevers, papilledema) should be evaluated for secondary causes. This patient's worsening headaches, nausea, vomiting, blurry vision, and papilledema on funduscopy are all highly concerning for raised intracranial pressure (ICP). Her periumbilical tenderness could be from irritable bowel syndrome, nonsteroidal anti-inflammatory drug (eg, ibuprofen) use, or another intra-abdominal pathology (eg, primary mass lesion). Papilledema occurs when elevated ICP is transmitted to the optic nerve. Common causes include the following: Papilledema Mass lesions Increased cerebrospinal fluid production Decreased cerebrospinal fluid outflow (venous sinus thrombosis) Idiopathic intracranial hypertension (pseudotumor cerebri) Patients such as this one with a chronic daily headache and evidence of raised ICP should undergo neuroimaging, preferably an MRI with contrast. If there is a concern for acute subarachnoid hemorrhage (eg, acute onset, "worst headache of life"), noncontrast CT scan is preferred. (Choice A) Hypertensive emergencies may also present similarly, but the chronicity of this patient's presentation in the absence of significantly elevated blood pressure makes undiagnosed hypertension unlikely. (Choice C) A lumbar puncture to diagnose idiopathic intracranial hypertension may eventually be necessary. However, performing a lumbar puncture in the presence of raised ICP and a mass lesion places the patient at risk for brainstem herniation. Therefore, the first step in management is neuroimaging to exclude the presence of a mass lesion. (Choice D) Migraine or medication overuse headache can be severe and associated with nausea and vomiting, but papilledema is not seen with these diagnoses. (Choice E) Although cocaine intoxication may cause headache, the remainder of this patient's clinical features (eg, papilledema) are not explained by drug intoxication. Therefore, a urine toxicology screen would not be the best next step. Educational objective: Adults with headaches and red flag features should be evaluated for secondary causes. Headache and papilledema should prompt consideration of neuroimaging, preferably a brain MRI with contrast to evaluate for etiologies such as mass lesions and cerebral venous sinus thrombosis. References: Approach to acute headache in adults. (http://www.ncbi.nlm.nih.gov/pubmed/23939446) Primary brain tumors in adults. (http://www.ncbi.nlm.nih.gov/pubmed/18533376)

Question 20

A 46-year-old African American man comes to the physician with a "funny feeling" in his face that started yesterday.  He has difficulty smiling and chewing on the right side of his mouth.  Cigarette smoking has also been difficult because he is unable to "puff."  He has "cold sores" every few months that correlate with stressful deadlines at his computer programming job.  He smokes 1 pack of cigarettes daily.  He lives in Arizona and has not traveled outside of the state in at least 3 years.  Physical examination shows wrinkling of the left forehead with ipsilateral eyebrow elevation.  He is unable to raise his right eyebrow, and his right forehead remains smooth.  When the patient is asked to close his eyes, his right eye remains partially open.  The right side of his mouth appears droopy.  The remainder of his examination is normal.  Which of the following is the best next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_bfe9_2247_8f63_4b72111ee624_MD0005_00 The rapid onset of unilateral upper and lower facial weakness is consistent with Bell's palsy, an idiopathic neuropathy of cranial nerve VII. Common findings of the afflicted side include inability to raise the eyebrow or close the eye, drooping of the mouth corner, and disappearance of the nasolabial fold. The "funny feeling" described by this patient is due to the perception of stiffness or pulling of the face to 1 side. It is very important that patients be assessed for symmetry by raising their eyebrows due to bilateral upper-motor-neuron innervation to the forehead. Forehead muscle sparing is suggestive of an intracranial lesion and would warrant brain imaging (Choice E) to evaluate for ischemia or tumors. No diagnostic workup is necessary unless the patient presents with bilateral facial palsy, lives or has traveled to a Lyme-endemic area, or has other concerning symptoms. If there is a history of travel to the northeast or upper midwestern United States, erythema migrans, or bilateral facial palsy, serum enzyme-linked immunosorbent assay testing (Choice D)should be obtained. This patient lives in an area with a low incidence of Lyme disease and has no other symptoms, making this test superfluous. Lyme-endemic area Corticosteroids are the treatment of choice for Bell's palsy. They should be started within 3 days to improve chances of complete recovery. Eye care is extremely important for patients with poor eyelid closure as the cornea is at risk of dryness and abrasions. Therefore, artificial tears and eye patching should be prescribed. Most patients recover within 1-6 months of onset. (Choice B) Although reactivation of herpes simplex virus is thought to cause most cases of Bell's palsy, clinical trials have failed to demonstrate any benefit of antiviral monotherapy. Data regarding combination glucocorticoid and antiviral therapy remain controversial. (Choice C) Although uncommon, bilateral facial palsy can also occur in sarcoidosis. Chest x-ray should be performed if there is a high suspicion for sarcoidosis as almost all patients have lung involvement that classically presents as bilateral hilar adenopathy. However, this patient has no cough, dyspnea, or chest pain that typically occurs in sarcoidosis and should not undergo unnecessary radiation. Educational objective: Unilateral upper and lower facial weakness is consistent with cranial nerve VII neuropathy. No further workup is warranted unless the patient has traveled to a Lyme-endemic area or presents with other concerning symptoms. Corticosteroids and supportive eye care are the mainstays of treatment. References: Combined corticosteroid and antiviral treatment for Bell palsy: a systematic review and meta-analysis. (http://www.ncbi.nlm.nih.gov/pubmed/19724046) Ophthalmic management of facial nerve palsy: a review. (http://www.ncbi.nlm.nih.gov/pubmed/17355853) Prednisolone and valaciclovir in Bell's palsy: a randomised, double-blind, placebo-controlled, multicentre trial. (http://www.ncbi.nlm.nih.gov/pubmed/18849193) Early treatment with prednisolone or acyclovir in Bell's palsy. (http://www.ncbi.nlm.nih.gov/pubmed/17942873)

Exam 1: Advanced Clinical Medicine