Question 1

A 74-year-old man comes to the office due to memory problems over the past several months. He is accompanied by his wife. The patient says, "I am worried about my memory. I feel like I am losing my mind. I can't remember what I've read in the newspaper, and I even have difficulty following the plot on TV shows." His wife says, "He frequently misplaces things like his keys and wallet and can't recall names of individuals he should know. She adds, "He used to like to cook and watch TV, but he doesn't seem to enjoy these things anymore. When he attempted to make a favorite dish recently, he left out half the ingredients and burned the meal." Over the past 2 months, the patient has been involved in 2 minor car accidents in which he was "not paying attention." He recently told his wife that they are "ruined and going bankrupt," despite her multiple assurances that this is not so. She says, "I show him the bank statements, but a few days later, it's as if he doesn't remember and he asks to see them again." They went through a difficult time over the past year due to his suspicions that she had an affair, which were recently confirmed. The patient's medical history is significant for gastroesophageal reflux disease, rheumatoid arthritis, and hypertension. He has increased soreness and pain in his joints and neck that he attributes to "my body breaking down." The patient has a history of thyroid cancer that was successfully treated 15 years earlier. His medications include lisinopril, levothyroxine, hydrochlorothiazide, aspirin, and antacids. Physical examination is unremarkable. Laboratory studies, including vitamin B₁₂ and thyroid function, are normal. The patient is diagnosed with major depressive disorder, and antidepressant medication is started. Three months later, his condition has significantly improved. Examination of memory and concentration demonstrates clear improvement. The patient's wife says that he is "back to his normal self and sharp as a whistle." He has been driving without accidents. They have started socializing again and have attended couples counseling to address issues with infidelity. At a recent clinic visit, the patient and his wife ask about his prognosis, as they are planning their advance directive documents. Which of the following disorders is the patient at increased risk of developing?

Accepted Answer

Explanation: Late-life depression (occurring age >65) has been found to be a significant risk factor for the development of major neurocognitive disorder (dementia) in the years that follow. This is in contrast to individuals who have had major depressive episodes in their earlier years. Although depression with cognitive impairment has historically been referred to as "pseudodementia" due to the quick improvement of cognitive symptoms with effective depression treatment, it is associated with an increased risk of subsequent vascular or Alzheimer dementia. Physicians should remain vigilant for signs of dementia in patients with a history of late-life depression. (Choice A) Delusional disorder involves one or more nonbizarre delusions lasting >1 month in the absence of any mood or other psychotic symptoms. It is not associated with a prior resolved depressive episode. (Choice B) Depression is often comorbid with anxiety; however, late-onset depression is not a known risk factor for future development of generalized anxiety disorder. (Choice C) Late-onset bipolar disorder refers to bipolar disorder presenting after age 50. It is fairly uncommon, and a previous late-life depressive episode does not increase the risk of developing this disorder. (Choice D) Pick disease, or major frontotemporal neurocognitive disorder, is a neurodegenerative disorder of the frontal and temporal lobes that involves early onset (in the 50s and 60s), personality changes, and executive dysfunction progressing to dementia. There is no indication that late-life depression leads to a higher risk of developing this condition. Educational objective: Patients with late-life depression (a major depressive episode occurring age >65) are at higher risk for developing Alzheimer disease and vascular dementia than those with depressive episodes earlier in life. Physicians should remain vigilant for cognitive symptoms in this patient population. References: Shared genetic risk factors for late-life depression and Alzheimer's disease. (http://www.ncbi.nlm.nih.gov/pubmed/27060956) Long-term outcome of depressive pseudodementia in the elderly. (http://www.ncbi.nlm.nih.gov/pubmed/17184844) Late-life depression and risk of vascular dementia and Alzheimer's disease: systematic review and meta-analysis of community-based cohort studies. (http://www.ncbi.nlm.nih.gov/pubmed/23637108)

Question 2

A 72-year-old Caucasian female is brought to the emergency room because of a sudden onset of confusion and clumsiness.  Her daughter tells you that she was watching television when the symptoms started.  She has a past history of hypertension and atrial fibrillation.  On physical examination, there is no evidence of any focal weakness of her extremities.  Her speech is clear, and she is able to repeat the sentences after you.  She is able to engage in an intelligent conversation, and provide her own past medical history.  When you ask her to copy a picture of a matchstick, she is unable to do it.  Which of the lobes of the brain is most likely affected in this patient?

Accepted Answer

Explanation: Almost 90% of right-handed patients and 60% of left-handed patients have a left hemispheric dominance for speech and language functions. The patient in the above scenario appears to be suffering from construction apraxia. This is seen with the involvement of parietal lobes, and is much more pronounced with lesions of the right parietal hemisphere (nondominant parietal lobe). Patients have marked difficulty in copying simple line drawings. They may also experience difficulty in wearing clothes, and appear to struggle while attempting to get into a coat or pants. This is known as dressing apraxia, which is seen with bilateral or right-sided (nondominant) parietal lobe lesions. Confusion is also seen in patients with lesions of the nondominant parietal lobe. (Choice B) Damage to the dominant parietal lobe, especially the inferior parietal lobe, presents as Gerstmann syndrome. The patients have difficulty in performing simple arithmetic tasks (acalculia), inability to name individual fingers (finger agnosia), impaired writing (agraphia) and right/left confusion (difficulty in identifying or distinguishing the right or left side of the body). (Choice C) Lesions of the nondominant temporal lobe can cause visual disorders (homonymous upper quadrantanopia) and impaired perception of complex sounds (auditory agnosia). (Choice D) Dominant temporal lobe lesions, in addition to having homonymous upper quadrantanopia, almost always involve the language functions, and lead to aphasia. Wernicke's aphasia is usually seen in patients with dominant temporal lobe lesions. It is characterized by the impairment in comprehension of spoken or written language. Patients have difficulty in expressing their thoughts in a meaningful manner. Apraxia is not seen in temporal lobe lesions. Educational Objective: Nondominant parietal lobe lesions present with constructional and dressing apraxia. Aphasia is typically seen in dominant temporal lobe lesions.

Question 3

A 20-year-old woman comes to the office for follow-up of a head injury.  She plays soccer for her college, and yesterday she was treated in the emergency department after a brief episode of loss of consciousness while using her head to redirect the soccer ball.  In the emergency department, the patient was unable to remember details of the game, but her neurologic examination was otherwise normal.  A noncontrast CT scan of the head was also normal.  Today, she reports some pain associated with a bump on the top of her head, but otherwise feels well.  The patient has had no dizziness, nausea, or vomiting.  Neurologic examination is normal.  She would like to know if she can play in a soccer tournament in 2 days.  Which of the following is the most appropriate recommendation?

Accepted Answer

Explanation: 11ec2b69_c189_8b93_8f63_fd88117bbb24_MD0005_00 This young woman with a loss of consciousness and amnesia after head trauma has a concussion, characterized by a transient neurologic disturbance (eg, disorientation, amnesia) that results from mild traumatic brain injury. Other common signs and symptoms include headache, dizziness, slurred or delayed speech, and incoordination; nausea/vomiting and brief loss of consciousness may also occur. Initial management includes removal from sports play and a thorough neurologic examination; a CT scan of the head is often performed to rule out other etiologies or complications (eg, skull fracture). Patients should be monitored closely for 24 hours (usually as an outpatient), but in the absence of risk factors (eg, anticoagulation, neurologic deterioration), further imaging is usually not required (Choice C). Premature return to sports play increases the risk of both recurrent concussion and long-term sequelae (eg, chronic traumatic encephalopathy, second impact syndrome); therefore, a graduated return to play protocol has been recommended to reduce this risk. Average-risk adults should rest for ≥24 hours before gradually increasing activity level each 24-hour period they remain asymptomatic, potentially returning to full contact activities no sooner than in 1 week (Choice E). Initial activity should begin with light aerobic exercise, transition to noncontact sports (eg, running), and progress to contact sports (eg, lacrosse); those who develop symptoms should return to the activity level at which they were last asymptomatic for 24 hours prior to activity progression. (Choices A and B) In the absence of recurrent symptoms, patients on a graduated return to play protocol can typically advance through progressively more strenuous activity and return to contact sports in 1 week. Although patients with significant sequelae (eg, posttraumatic epilepsy) should generally avoid all contact sports, this patient with a single, uncomplicated concussion does not require extended activity restriction. Educational objective: Concussion is characterized by a transient neurologic disturbance (eg, disorientation, amnesia) that results from mild traumatic brain injury; headaches, dizziness, slurred or delayed speech, and incoordination are also common. After a concussion, average-risk adults should rest for ≥24 hours before gradually increasing activity level each 24-hour period they remain asymptomatic, potentially returning to full contact sports in 1 week.

Question 4

A 63-year-old man comes to the emergency department due to sharp, stabbing pain on the right side of the face and hiccups that started 6 hours ago.  The patient is right-handed and has been unable to feed himself since his symptoms started.  He has a history of stable angina, hypertension, and hyperlipidemia.  Medications include atenolol, simvastatin, and aspirin.  Vital signs are within normal limits.  The patient has horizontal and vertical nystagmus and ptosis of the right eye.  Gag reflex is diminished.  He reports loss of pain and temperature sensation on the right side of the face and left trunk and limbs, as well as severe dizziness when attempting to stand.  Which of the following is the most likely location of this patient's brain lesion?

Accepted Answer

Explanation: 11ec2b69_c18f_57f7_8f63_5bcae7a7c87e_MD0005_00 This patient has had a stroke that resulted in lower cranial nerve nuclei deficits (eg, vertigo, decreased gag) and loss of pain and temperature sensation of the right face (trigeminal nucleus) and left body (spinothalamic tract). Because the brainstem is where most cranial nerves originate and many motor and sensory fibers cross the midline, brainstem strokes characteristically lead to ipsilateral cranial nerve deficits and contralateral deficits of the body (ie, crossed signs). In addition, this patient has the following: Vertigo and nystagmus, suggesting a vestibulocochlear nerve (CN VIII) deficit, and bulbar weakness (eg, weak gag, dysphagia), suggesting glossopharyngeal nerve (CN IX) and/or vagus nerve (CN X) dysfunction. Each of these cranial nerves has nuclei in the lateral medulla (vestibular nucleus, nucleus ambiguus). Loss of pain and temperature sensation in the ipsilateral face and contralateral body due to damage to the spinal trigeminal nucleus and spinothalamic tract Ipsilateral Horner syndrome due to damage of descending sympathetic nervous system fibers that travel near the spinothalamic tract This constellation of findings is consistent with lateral medullary (Wallenberg) syndrome. Because the corticospinal tract travels medially in the entire brainstem before crossing at the lower medulla, most lateral brainstem lesions do not have dense contralateral hemiplegia. (Choice A) Although lesions of the lateral cerebellar hemisphere present with dizziness and ipsilateral ataxia, they do not cause Horner syndrome or loss of pain and temperature sensation. (Choices C and E) Pontine lesions can also lead to ataxia due to disruption of the cerebellar peduncles and vestibular nuclei. However, this patient's dysphagia, hoarseness, and diminished gag reflex are due to CN IX and/or CN X involvement, suggesting damage to the nucleus ambiguus, located in the medulla. The nuclei for cranial nerves V-VIII originate in the pons. (Choice D) Unlike the lateral medulla, which contains many tracts, the medial medulla mainly contains the hypoglossal nerve (CN XII) and the corticospinal tract. Patients with medial medullary syndrome develop contralateral hemiparalysis and ipsilateral CN XII palsy (tongue deviation toward the lesion). medial medullary syndrome Educational objective: A lateral medullary infarct (Wallenberg syndrome) leads to vertigo/nystagmus (vestibular nucleus), loss of pain/temperature sensation in the ipsilateral face (trigeminal) and contralateral body (spinothalamic), bulbar weakness (lower cranial nerves), and ipsilateral Horner syndrome (descending sympathetic). References: Traumatic vertebral artery dissection and Wallenberg syndrome after a motorcycle collision. (http://www.ncbi.nlm.nih.gov/pubmed/19041559) Dysphagia caused by a lateral medullary infarction syndrome (Wallenberg's syndrome). (http://www.ncbi.nlm.nih.gov/pubmed/23077713)

Question 5

A 22-year-old Caucasian female is brought to your office by her boyfriend after he found her unconscious on the bed.  He says that he is unaware of her past medical history, but remembers one similar episode that resolved completely.  Her temperature is 36.7 C (98 F), blood pressure is 130/80 mm Hg, pulse is 90/min, and respirations are 18/min.  The patient is unresponsive to verbal and tactile stimuli.  Physical examination reveals a symmetric decrease in muscular tone and normal deep tendon reflexes.  Pupils are symmetric, 5mm, and reactive to light.  Irrigation of the right external auditory canal with cold water reveals a transient, conjugate, slow deviation of gaze to the right, followed by saccadic correction to the midline.  Which of the following is the most likely cause of this patient's condition?

Accepted Answer

Explanation: The clinical scenario described above illustrates the importance of oculovestibular testing in the differential diagnosis of coma. Caloric stimulation of the vestibular apparatus is performed by irrigation of the external auditory canal with cold water. A normal response is characterized by a transient, conjugate, slow deviation of gaze to the side of the stimulus (brainstem-mediated), followed by saccadic correction to the midline (cortical correction). A caloric response cannot be voluntarily suppressed; therefore, the normal oculovestibular reaction strongly suggests psychogenic coma. (Choices A and D)Metabolic or supratentorial/infratentorial causes of coma usually disrupt brainstem-mediated or cortical components of the caloric response. (Choices B and C)Botulism and Guillain-Barre syndrome lead to the paralysis of skeletal muscles, but rarely result in a true coma. Educational Objective: A normal reaction to caloric stimulation of the external auditory canal strongly suggests psychogenic coma.

Question 6

A 77-year-old woman is brought to the office by her son due to frequent falls and confusion.  The patient began to fall without clear provocation 2 years ago.  Her family didn't think anything of the falls until she began having episodes of confusion a year ago.  The patient's son says, "She has good and bad days.  On good days she seems almost like her old self, but on bad days she appears confused and is difficult to understand."  He adds that she sometimes appears to be distracted and agitated by people or things that he cannot see.  The patient's medical history includes hypertension and coronary artery disease; her mother died of a stroke and her father had several heart attacks.  Temperature is 37.2 C (98.9 F), blood pressure is 140/86 mm Hg, pulse is 84/min, and respirations are 16/min.  Her movements and speech are slow, she has a short-stepped gait and rigidity in her upper extremities.  Montreal Cognitive Assessment is 20 on a scale of 0-30 (normal: ≥26). 
Which of the following is the most likely diagnosis?

Accepted Answer

Explanation: 11ec2b69_c18a_27d4_8f63_6114d3f6874e_MD0005_00 This patient's symptoms are consistent with dementia with Lewy bodies (DLB). DLB may be diagnosed in the setting of dementia (ie, progressive cognitive decline that causes functional impairment) when ≥2 of the following 4 clinical features are present: Fluctuating cognition (eg, alertness, attention) Visual hallucinations Parkinsonism Rapid eye movement sleep behavior disorder (ie, loss of normal rapid eye movement sleep atonia). DLB typically presents around age 75 and is 4 times more common in men. DLB is the second leading cause of dementia behind Alzheimer disease and may account for up to a third of cases of dementia. The survival time after initial diagnosis of DLB is approximately 8 years. (Choice A) Parkinsonism and visual hallucinations are not consistent with Alzheimer disease. Early, prominent, and relatively isolated amnestic symptoms are characteristic of the disease. (Choice C) Although some variants of frontotemporal dementia may involve motor syndromes, frontotemporal dementia typically presents with early personality changes (eg, disinhibition) that initially predominate over cognitive dysfunction. (Choice D) The classic triad of normal-pressure hydrocephalus (NPH) consists of cognitive impairment, urinary incontinence, and abnormal gait. Gait dysfunction in patients with NPH (eg, magnetic gait) can appear similar to a parkinsonian gait (shuffling); however, rigidity and bradykinesia are not present in NPH. (Choice E) Vascular dementia typically presents with early executive dysfunction, focal neurological findings, and evidence of cerebrovascular disease on imaging of the head. It would not explain this patient's fluctuating symptoms, parkinsonism, and visual hallucinations. Educational objective: Dementia with Lewy bodies presents with dementia and ≥2 of the following: Fluctuating cognition (eg, alertness, attention), visual hallucinations, parkinsonism, and/or rapid eye movement sleep behavior disorder (ie, loss of normal rapid eye movement sleep atonia). References: Diagnosis and management of dementia with Lewy bodies: fourth consensus report of the DLB Consortium. (http://www.ncbi.nlm.nih.gov/pubmed/28592453) Cholinesterase inhibitors for dementia with Lewy bodies, Parkinson's disease dementia and cognitive impairment in Parkinson's disease. (http://www.ncbi.nlm.nih.gov/pubmed/22419314)

Question 7

A 65-year-old man was admitted to the intensive care unit after going into cardiac arrest when alone at home.  Cardiopulmonary resuscitation and advanced cardiac life support were performed extensively in the field, and return of spontaneous circulation was eventually achieved.  Two days later, he remains intubated and is on full ventilator support.  The patient is unresponsive to verbal and tactile stimuli.  His temperature is 37.2 C (99 F), blood pressure is 110/70 mm Hg, and pulse is 70/min.  The treating physician discusses with the patient's family the possibility of brain death. The patient is diagnosed with brain death.  The physician explains this finding to the patient's family and discusses withdrawing of mechanical ventilator support.  Everyone in the family appears to understand and agrees to proceed with withdrawing ventilatory support.  A relative then walks into the patient's room and notices some movements in the patient's legs and toes.  The relative believes the patient is not dead and gets angry with the treating physician.  Which of the following is the most appropriate response to the relative's reaction?

Accepted Answer

Explanation: Brain death is the irreversible absence of cerebral and brainstem reflexes including pupillary, oculocephalic, oculovestibular (caloric), corneal, gag, sucking, swallowing, and extensor posturing. Patients with brain death can have spontaneous movements, but these originate from peripheral nerves or the spinal cord. Possible reflexes include finger flexion, truncal movements (eg, superficial and deep abdominal muscles), triple flexion response (flexion at the hip, knee, and ankle with foot stimulation), plantar reflexes (Babinski sign), limb movements to painful stimuli, and alternating flexion-extension of the toes. Explaining this in a calm manner is the appropriate response to help dissipate any anger and anxiety among family members. (Choices A, C, D, and E) This patient does not require any further testing or consultations based on limb movements alone as the criteria for brain death have already been fulfilled. Educational objective: Patients with brain death can have spontaneous movements originating from peripheral nerves or the spinal cord. References: Fasciculations in brain death. (http://www.ncbi.nlm.nih.gov/pubmed/20890193) Undulating toe movements in brain death. (http://www.ncbi.nlm.nih.gov/pubmed/15525292)

Question 8

A 30-year-old Caucasian male comes to your office and complains of pain and numbness in his right wrist and palmar surface of his first three fingers.  These symptoms woke him up last night.  He is a baker, and rolls dough using repetitive movements, including hyperextension of the wrist and pronation of the forearm.  He remembers having several similar episodes of finger numbness before, which resolved completely after a period of rest.  His past medical history is otherwise insignificant.  Which of the following is the most likely localization of the pathologic process in this patient?

Accepted Answer

Explanation: The clinical scenario described is suggestive of carpal tunnel syndrome - pain and numbness in the wrist and palmar surface of the first three fingers associated with repetitive movements of the wrist. (Choice A) Median nerve entrapment between the two heads of pronator teres is less common, and usually manifests as pain in the volar forearm, in addition to the symptoms described in this scenario. (Choices C and D) Ulnar nerve involvement is indicated by tingling and numbness of the one-and-half fingers on the ulnar aspect of the hand. (Choice E) The radial nerve is known as 'the great extensor nerve.' Sensory abnormalities during radial nerve involvement are located on the dorsal aspect of the forearm and hand. Educational Objective: Pain and numbness in the wrist and palmar surface of the first three fingers associated with repetitive movements of the wrist is typical for carpal tunnel syndrome.

Question 9

A 77-year-old woman is brought to the office by her son due to frequent falls and confusion.  The patient began to fall without clear provocation 2 years ago.  Her family didn't think anything of the falls until she began having episodes of confusion a year ago.  The patient's son says, "She has good and bad days.  On good days she seems almost like her old self, but on bad days she appears confused and is difficult to understand."  He adds that she sometimes appears to be distracted and agitated by people or things that he cannot see.  The patient's medical history includes hypertension and coronary artery disease; her mother died of a stroke and her father had several heart attacks.  Temperature is 37.2 C (98.9 F), blood pressure is 140/86 mm Hg, pulse is 84/min, and respirations are 16/min.  Her movements and speech are slow, she has a short-stepped gait and rigidity in her upper extremities.  Montreal Cognitive Assessment is 20 on a scale of 0-30 (normal: ≥26). The patient is prescribed donepezil, carbidopa levodopa, and risperidone.  Two weeks after the initial office visit, she is brought to the emergency department by her son due to increasing confusion and falls.  Supine blood pressure and pulse are 124/82 mm Hg and 65/min, respectively; standing blood pressure and pulse are 98/74 mm Hg and 87/min.  The patient is not oriented to person, place, or time.  Examination shows cranial nerves are within normal limits.  Rigidity is present in both upper and lower extremities on passive movement.  Upper and lower limb deep tendon reflexes are 2+.  Which of the following is the most likely explanation for this patient's symptoms?

Accepted Answer

Explanation: Pharmacotherapy for dementia with Lewy bodies (DLB) includes cholinesterase inhibitors for cognitive impairment, carbidopa-levodopa for parkinsonism, and melatonin for REM sleep behavior disorder. Antipsychotics may be used to treat functionally impairing visual hallucinations or delusions in patients with DLB; however, physicians should be aware that patients with DLB are extremely sensitive to antipsychotics. The use of antipsychotics in DLB may be associated with a significant worsening of confusion, parkinsonism, or autonomic dysfunction. This patient's acute exacerbation of symptoms is most likely due to recent initiation of risperidone, a drug with stronger dopamine receptor antagonism compared to other second-generation antipsychotics. When antipsychotics are necessary, evidence favors low-potency second-generation antipsychotics (eg, quetiapine) to reduce the risk of severe reactions. First-generation antipsychotics (eg, haloperidol) should be avoided entirely. In addition to severe antipsychotic sensitivity associated with DLB, antipsychotic drugs are associated with increased mortality in elderly patients with dementia. (Choice A) Anti-Parkinson therapy would not explain this patient's worsening confusion and parkinsonian symptoms. Anti-Parkinson drugs have been associated with exacerbation of psychotic symptoms in DLB, but this risk can be minimized by careful dose titration and judicious, as-needed use of low-dose antipsychotics. (Choice C) The most clinically significant side effects of cholinesterase inhibitors are diarrhea, vomiting, and bradycardia. (Choice D) Patients with postural tachycardia syndrome do not exhibit associated confusion and typically do not have arterial hypotension with standing. This patient's autonomic dysfunction is a feature of her DLB. (Choice E) Although cognitive impairment and/or parkinsonism may worsen as DLB progresses, the rapidity of the patient's deterioration combined with the recent prescription of new medication suggest that the best explanation for her worsened symptoms is the antipsychotic. Educational objective: Patients with dementia with Lewy bodies (DLB) are extremely sensitive to antipsychotics, and their use may be associated with an exacerbation of confusion, parkinsonism, and/or autonomic dysfunction. For DLB patients with functionally impairing visual hallucinations, low-dose, low-potency, second-generation antipsychotics (eg, quetiapine) are preferred over first-generation antipsychotics. References: Diagnosis and management of cognitive and behavioral changes in dementia with Lewy bodies. (http://www.ncbi.nlm.nih.gov/pubmed/28990131)

Question 10

A 32-year-old woman comes to the clinic due to persistent thoughts about killing her 3 children.  She says, "This is so upsetting because I love my children and would never harm them.  I have had these thoughts for the past 3 months and cannot get them out of my head.  It happens when I'm in the kitchen preparing dinner.  As I reach for a knife in the drawer, I get this sudden thought about stabbing all three of them.  The thoughts are senseless and ridiculous, but I worry that I'm going crazy.  I wish I never had these thoughts but can't make them stop."  The patient is happily married and says that all 3 children are well behaved.  Her medical problems include hypothyroidism and microcytic anemia.  She has no previous psychiatric history, but recalls being very anxious as a child.  The patient says, "I was always worried that something bad was going to happen to my parents if I didn't do the right thing and would get really upset if anyone touched my possessions."  Current medications include levothyroxine and iron.  Temperature is 37 C (98.6 F), blood pressure is 131/78 mm Hg, and pulse is 87/min.  On examination, the pupils are equal and reactive to light and mucous membranes are moist.  There is no thyromegaly.  Auscultation of the lungs reveals normal breath sounds.  Heart sounds are normal.  Neurologic examination reveals no focal deficits. At his 4-week follow-up, the patient continues to complain of shaking and claims that "it is starting to become bothersome."  Which of the following is the best treatment for this patient?

Accepted Answer

Explanation: Levodopa or dopamine agonists (eg, pramipexole, bromocriptine) are typically used for the initial treatment of Parkinson disease (PD) symptoms. Levodopa is the most effective in controlling symptoms, but there are potential concerns regarding its prolonged use. Levodopa may hasten destruction of substantia nigra cells and worsen symptoms in the long-term. There might also be a limited period of response to levodopa and a higher incidence of levodopa-related dyskinesia in patients with early-onset PD. Consequently, some experts favor levodopa in patients age >65 who have severe symptoms affecting their quality of life. Dopamine agonists such as pramipexole can help to save levodopa treatment for a later time when the patient's symptoms are more severe, and it might be preferred as the initial treatment in patients age <65. This patient is 60 years old with relatively mild symptoms of PD and would likely benefit from pramipexole. (Choices A, D, and E) Essential tremor (ET) usually involves both hands/forearms, worsens with fine motor activity, and the tremor is typically the only neurologic finding on examination. Propranolol and primidone have similar efficacy in the treatment of ET and are considered first-line medications. Clonazepam is a benzodiazepine that might improve symptoms of ET; however, it has a potential for abuse and dependence and can be associated with increased falls in the elderly. ET is unlikely in this patient as his tremor is unilateral in onset, improves with movement, and is associated with rigidity. (Choice B) Entacapone is a catechol-O-methyl transferase (COMT) inhibitor. It has no effect on PD by itself but it can help to prolong the effect of levodopa. Educational objective: Levodopa or dopamine agonists (eg, pramipexole) can be used as the initial treatment of Parkinson disease symptoms. Dopamine agonists might be preferred in younger patients with less severe symptoms due to concerns about the long-term efficacy and side effects of levodopa. References: Role of pramipexole in the management of Parkinson's disease. (http://www.ncbi.nlm.nih.gov/pubmed/20839895)

Question 11

A 19-year-old Caucasian male comes to clinic complaining of frequent headaches that "make it impossible to do well at work or school."  He cannot pinpoint when the headaches started.  Recently, he has also noticed a rhythmic pulsating sound in his ears when he bends over.  When he straightens, his vision dims in both eyes.  He has been diagnosed with depression, attention deficit disorder, and acne vulgaris, and is currently taking fluoxetine, atomoxetine, and isotretinoin.  Vital signs are normal.  Ophthalmologic examination is remarkable for peripapillary flame hemorrhages, venous engorgement, and hard exudates.  There is some constriction of the visual field in the inferior nasal quadrant.  Visual acuity is normal.  What is the most likely diagnosis?

Accepted Answer

Explanation: Pseudotumor cerebri (Choice D), also known as idiopathic intracranial hypertension, arises from chronically elevated intracranial pressure. Papilledema is evident on examination and may lead to progressive optic atrophy and blindness. The condition is most common in obese premenopausal women, but is also thought to occur secondary to endocrine disorders (e.g., hypoparathyroidism, hypothyroidism, adrenal insufficiency, Cushing disease) and the usage of some medications (e.g., isotretinoin, all-trans-retinoic acid, minocycline, tetracycline, cimetidine, corticosteroids, danazol, tamoxifen, levothyroxine, lithium, nitrofurantoin). The patient in this case used isotretinoin, which was the likely cause of his pseudotumor cerebri. trans Tension headache (Choice A) is usually categorized as episodic or chronic. Episodic tension headaches are mild to moderate, often associated with a specific stressful event, and usually improve after treatment with over-the-counter medications. Chronic tension headaches are linked with contraction of scalp and neck muscles and may recur daily. Visual symptoms other than occasional photophobia are not common. Cluster headaches (Choice B) are sudden, severe headaches centered in the temporal and periorbital regions, and are often associated with lacrimation, nasal congestion, or nausea. Patients typically experience 1-2 cluster periods lasting for 2-3 months every year. Young and middle-aged men are most prone to cluster headaches. Visual symptoms are not common. Migraine headaches (Choice C) are recurrent throbbing or pulsatile headaches often associated with a prodrome, nausea, vomiting, photophobia, and phonophobia. When they occur, the prodromes are characterized by visual scintillations, scotomas, dizziness, or tinnitus. Subarachnoid hemorrhages (Choice E) most commonly result from head injury or the leakage of an aneurysm or arteriovenous malformation. These headaches are associated with significant pain, sudden onset, nausea and vomiting, meningeal irritation, photophobia and visual changes, and sometimes loss of consciousness or seizures. Educational Objective: Pseudotumor cerebri most commonly occurs in obese women of childbearing age. It is also thought to occur secondary to endocrine disorders and the usage of some medications, including the acne treatments isotretinoin and minocycline.

Question 12

A 74-year-old man comes to the office due to memory problems over the past several months. He is accompanied by his wife. The patient says, "I am worried about my memory. I feel like I am losing my mind. I can't remember what I've read in the newspaper, and I even have difficulty following the plot on TV shows." His wife says, "He frequently misplaces things like his keys and wallet and can't recall names of individuals he should know. She adds, "He used to like to cook and watch TV, but he doesn't seem to enjoy these things anymore. When he attempted to make a favorite dish recently, he left out half the ingredients and burned the meal." Over the past 2 months, the patient has been involved in 2 minor car accidents in which he was "not paying attention." He recently told his wife that they are "ruined and going bankrupt," despite her multiple assurances that this is not so. She says, "I show him the bank statements, but a few days later, it's as if he doesn't remember and he asks to see them again." They went through a difficult time over the past year due to his suspicions that she had an affair, which were recently confirmed. The patient's medical history is significant for gastroesophageal reflux disease, rheumatoid arthritis, and hypertension. He has increased soreness and pain in his joints and neck that he attributes to "my body breaking down." The patient has a history of thyroid cancer that was successfully treated 15 years earlier. His medications include lisinopril, levothyroxine, hydrochlorothiazide, aspirin, and antacids. Physical examination is unremarkable. Laboratory studies, including vitamin B₁₂ and thyroid function, are normal. Which of the following is the best response to this patient's concerns?

Accepted Answer

Explanation: Late-life depression frequently presents with reversible cognitive impairment (pseudodementia). Patients often experience impaired executive function (planning and carrying out tasks, processing new information), impaired memory and recall, and difficulties with focus and concentration. When the underlying depression is treated, the cognitive impairment often fully resolves. When older adults with memory deficits are assessed, mood and affect should be closely examined to determine whether underlying depression could be causing these symptoms. Due to concerns about stigma or judgment, many older adults do not overtly discuss depressive feelings, which are frequently misdiagnosed. In addition to impaired memory and concentration, this patient has multiple factors that should raise clinical suspicion for depression, including his psychosocial situation, loss of interest and enjoyment, somatic symptoms, and anxious ruminations. (Choice A) A brain MRI scan is most useful for ruling out other reversible causes of dementia (eg, subdural hematoma, normal pressure hydrocephalus, treatable cancer) and would not confirm the diagnosis of Alzheimer disease or assist in diagnosing depression. Although structural neuroimaging should be considered in the evaluation of all patients with dementia, this patient's cognitive deficits are likely secondary to depression, which should be ruled out first. (Choice B) The Mini-Mental State Examination is a useful test for dementia, with a score <24 points suggestive of dementia or delirium. However, it would not be helpful in identifying depression, and a score of <24 in this patient may be due to depression-related impairment in motivation and/or concentration rather than true dementia. (Choice C) Although normal aging is associated with some memory loss, this patient's cognitive impairment is causing clear functional impairment and requires further evaluation and treatment. (Choice E) It would be premature to suggest that this patient may have Alzheimer disease before assessing him for major depression. Educational objective: Cognitive impairment is a common symptom of late-life depression and is reversible with treatment. Older adults with new-onset cognitive impairment should be assessed for depression. References: Diagnosis and treatment of depression and cognitive impairment in late life. (http://www.ncbi.nlm.nih.gov/pubmed/25655026) Depression and cognition in the elderly. (http://www.ncbi.nlm.nih.gov/pubmed/25581234)

Question 13

A 58-year-old, obese, Caucasian male presents to the emergency department immediately after a motor vehicle accident in which he was a restrained passenger.  His medical history is significant for diabetes mellitus type 2, hypertension, gastroesophageal reflux disease, erectile dysfunction, and gout.  His current medications include metformin, rosiglitazone, lisinopril, and omeprazole.  He drinks 1-2 beers daily and denies the use of tobacco or recreational drugs.  His temperature is 36.9 C (98.4 F), blood pressure is 134/88 mmHg, pulse is 86/min, and respirations are 15/min.  Normal S1 and S2 heart sounds are present, with no murmurs or rubs.  The lung fields are clear.  The abdomen is obese, nontender and nondistended; bowel sounds are present.  Brown stool in the rectal vault is guaiac negative.  The rectal sphincter tone is normal.  There is no evidence of clubbing, cyanosis, or edema in the extremities.  Dorsalis pedis and posterior tibial pulses are present and equal bilaterally.  On neurologic exam, the patient is awake, alert, and oriented.  Cranial nerves II-XII are intact.  Motor strength is 5/5 in all extremities.  Finger to nose cerebellar exam is well done.  Reflexes are 1+ at the ankles and 2+ at the knees, biceps, and triceps.  The cremasteric reflex is absent.  Sensation is intact throughout, except for a bilateral symmetric reduction in perception of vibration, pain, and temperature in both feet and hands.  Plantar flexion and dorsiflexion are normal.  Ambulation is normal, and the Romberg sign is negative.  What is the most likely etiology of this individual's absent cremasteric reflex?

Accepted Answer

Explanation: The loss of the cremasteric reflex in this patient is secondary to his diabetes mellitus. The presence of distal symmetric polyneuropathy (in the classic "stocking-glove" pattern) and history of erectile dysfunction are suggestive of significant diabetic neuropathy. Other possible presentations of neurogenically induced erectile dysfunction in the diabetic male include diminished testicular sensation, bladder dysfunction, and inability to masturbate. (Choice A)The symptoms of cauda equina syndrome include severe lower back pain, urinary or bowel incontinence, motor weakness or sensory loss in the legs bilaterally, and saddle anesthesia. (Choice B)The cremasteric reflex corresponds to the L1-L2 level of the spinal cord, a region also responsible for hip flexion and adduction. The patient's normal ambulation and motor strength indicate that an injury to the spinal cord at L1-L2 is unlikely. (Choice C)The normal dorsiflexion and plantar flexion in this patient signify functionality of the L5-S2 region of the spinal cord. (Choice D)The normal anal sphincter tone in this patient signifies functionality of the S2-S4 region of the spinal cord. Educational Objective: The cremasteric reflex is regulated at the L1-L2 level of the spinal cord. This reflex can be diminished or lost secondary to diabetic autonomic neuropathy.

Question 14

A 62-year-old Caucasian male complains of occasional episodes of double vision over the last two months.  He also has intermittent difficulty swallowing.  The symptoms typically occur at the end of the day and go away after a short nap.  He denies any headaches, nausea, vomiting, imbalance, or falls.  Past medical history is significant for hypertension, hyperlipidemia, type 2 diabetes mellitus, and mild intermittent asthma.  He also had a transient ischemic attack five years ago and underwent a left carotid endarterectomy.  Current medications include metformin, glipizide, lisinopril, atorvastatin, hydrochlorothiazide, and aspirin.  He also uses an albuterol inhaler as needed for wheezing and shortness of breath.  He is a current smoker with a 35 pack-year smoking history.  His blood pressure is 134/78 mmHg and heart rate is 86/min.  On examination, there is mild left ptosis.  Which of the following is the most likely cause of this patient's complaints?

Accepted Answer

Explanation: This patient's presentation is most consistent with myasthenia gravis, which is a disease of the neuromuscular junction. The classic feature of myasthenia gravis is fluctuating muscle weakness that worsens throughout the course of the day. Most patients first notice double vision and ptosis that worsens at the end of the day, which is caused by weakness in the extraocular muscles. The next most common muscles involved are the bulbar muscles resulting in dysphagia and dysarthria. Proximal limb weakness is found less frequently. Fatigability of muscle function is the key to differentiating myasthenia gravis from other similar disorders. Myasthenia gravis is an autoimmune disease caused by autoantibodies against the acetylcholine receptor. There is a known association between myasthenia gravis and thymoma. (Choice A) Statin medications are infamous for leading to myopathy. However, it would be atypical for statin-induced myopathy to only involve the ocular and bulbar muscles, and the worsening of symptoms throughout the course of the day would be atypical. Rarely, statin medications can lead to new or worsening myasthenic symptoms. (Choice C) This patient's diabetes places him at risk for developing peripheral neuropathy. However, peripheral neuropathy typically first affects the nerves in the feet since they are the longest in the body. (Choice D)Lower motor neuron disease generally does not worsen throughout the course of the day and improve with rest. (Choice E) This patient is at risk of recurrent transient ischemic attacks given his prior history and numerous cardiovascular risk factors. However, the fact that he is having daily symptoms is not typical for transient ischemic attacks. Educational objective: Myasthenia gravis is an autoimmune disease caused by autoantibodies that attack the acetylcholine receptors in the neuromuscular junction. The ocular and bulbar muscles are the most commonly involved muscles groups, and the classic presentation consists of progressive muscular weakness throughout the day that improves with rest.

Question 15

A 64-year-old man is found unresponsive, pulseless, and with asystole when the ambulance arrives.  He is intubated, resuscitated, and transferred to the hospital.  Initial workup is consistent with subarachnoid bleeding.  The patient has a history of coronary artery disease, multiple percutaneous coronary interventions, and congestive heart failure with low left ventricular ejection fraction.  On repeat evaluation 24 hours later, he is comatose and does not respond to any verbal stimuli.  The patient's temperature is 36.7 C (98.1 F), blood pressure is 123/82 mm Hg, and pulse is 78/min.  Pupils are dilated and fixed at 8 mm.  Which of the following findings would most likely suggest brain death in this patient?

Accepted Answer

Explanation: 11ec2b69_c188_5312_8f63_291b106b82ef_MD0005_00 Brain death is the irreversible absence of cerebral and brain stem function. Clinical criteria suggestive of brain death include clinical or 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 [96.8 F], systolic blood pressure >100 mm Hg). Neurologic examination typically shows irreversible absence of cerebral and brainstem reflexes including pupillary, oculocephalic, oculovestibular (caloric), corneal, gag, sucking, swallowing, and extensor posturing. This patient is comatose with no response to verbal stimuli and an absent pupillary reflex, all consistent with brain death. An absent cough reflex on tracheal suctioning is also highly suggestive of brain death. (Choices B, C, D, and E) Patients with brain death can have spontaneous movements, but these originate from peripheral nerves or the spinal cord. Possible reflexes include finger flexion, truncal movements (eg, superficial and deep abdominal muscles), triple flexion response (flexion at the hip, knee, and ankle with foot stimulation), plantar reflexes (Babinski sign), limb movements to painful stimuli, and alternating flexion-extension of the toes. Educational objective: Patients with brain death have absence of reflexes associated with cortical and brainstem function (pupillary, oculocephalic, oculovestibular [caloric], corneal, gag, sucking, swallowing, and extensor posturing). Patients with brain death can have reflexes and movements, but these originate from peripheral nerves or the spinal cord. References: Diagnosis of brain death. (http://www.ncbi.nlm.nih.gov/pubmed/21577338)

Question 16

An 81-year-old man is brought to the physician due to a recent fall.  Three days ago, he stumbled on a rug in his living room and fell to the floor.  He had no dizziness or loss of consciousness prior to the event and did not hit his head.  He says that he only slipped, but his wife is concerned about the possibility of his falling again.  The patient's other medical problems include hypertension and a history of stroke 10 years ago.  His medications include aspirin, atorvastatin, and lisinopril.  He lives independently with his wife.  The patient's vital signs, including orthostatics, are within normal limits.  Physical examination, including vision, cranial nerves, sensation, and reflexes, shows no abnormalities.  No bruises or other superficial lesions are present.  After evaluating medication use, alcohol use, appropriate footwear, and home environmental factors, which of the following is the most appropriate next step in management?

Accepted Answer

Explanation: 11ec2b69_c18d_5c25_8f63_c395e8650e73_MD0005_00 This 81-year-old man most likely experienced a mechanical fall and has no evidence of trauma. His examination is reassuring without signs of neurologic or cardiac compromise. Approximately 30%−40% of elderly patients living in the community fall each year; those in long-term care facilities have rates almost twice as high. Although it is reasonable to obtain brain imaging studies in elderly patients with evidence of head trauma, ordering imaging on all elderly patients who fall would result in >1/3 of the senior population receiving head CT scans every year. In the absence of trauma, elderly patients who require medical attention and report multiple falls or have gait abnormalities should be evaluated with a comprehensive and multifactorial approach that includes evaluation of medication use, alcohol use, chronic diseases, environmental factors, blood pressure, and postural stability. According to guidelines published by the American Geriatric Society, British Geriatric Society, and American Academy of Orthopedic Surgeons, all elderly patients should be asked annually about any episode of falls. If a patient reports an episode, the evaluation should include at least one postural stability test. Assessment of gait and balance is important as it determines the need for further evaluation and intervention. The "Get Up and Go" test is commonly used to assess postural stability. In this test, the physician instructs the patient to stand up from an armless chair without assistance, walk a short distance, turn around, return, and sit down again. If the patient is unsteady or has difficulties during the test, further evaluation is necessary. (Choice A) Holter monitoring, electrocardiogram (ECG), and echocardiogram are recommended if a cardiac cause (eg, arrhythmias, cardiac syncope, myocardial ischemia) is suspected. (Choice B) The risk of intracranial bleeding due to a fall while on antiplatelet therapy is very low and would not warrant discontinuation of therapy. Even in average-risk patients with atrial fibrillation on anticoagulation for stroke prevention, the risk of intracranial bleeding is so minimal that a patient would need to fall almost 300 times a year to outweigh the benefits of anticoagulation. (Choice C) Serum chemistries or complete blood count are indicated when a systemic condition is suspected. (Choice E) Head imaging is needed when a neurologic problem is suggested by the history or physical examination. If there is evidence of significant head trauma, a head CT scan without contrast should be performed. Educational objective: Elderly patients should be asked annually about a history of falls. Patients who report a fall should undergo a comprehensive evaluation including postural stability (eg, "Get Up and Go" test). Patients who perform poorly or have recurrent falls may need a more extensive workup. References: Falls in the elderly. (http://www.ncbi.nlm.nih.gov/pubmed/10779256) Management of falls in older persons: a prescription for prevention. (http://www.ncbi.nlm.nih.gov/pubmed/22150660)

Question 17

An 83-year-old Caucasian man comes to the emergency department because of a sudden onset, transient visual loss in his right eye.  He is currently symptom-free, and denies any other new symptoms.  His other medical problems include hypertension and peripheral vascular disease.  His medications include aspirin, hydrochlorothiazide, and enalapril.  His temperature is 36.7 C (98 F), blood pressure is 160/90 mmHg, pulse is 80/min., and respirations are 12/min.  Which of the following signs is most likely to be associated with his symptoms/condition?

Accepted Answer

Explanation: This patient has developed amaurosis fugax, an acute ischemic event involving the retinal artery. This condition is sudden and usually reversible. It is a marker of carotid artery atherosclerotic disease. A carotid bruit on auscultation of the neck is a very frequent finding; this indicates a significant carotid obstruction. (Choice B)A diastolic heart murmur can be a marker of aortic insufficiency or mitral stenosis. Both conditions are related to ischemic cerebrovascular accidents (CVA). Although these could occur, this elderly patient's transient symptom is more likely to be concurrent with findings suggestive of carotid artery disease. (Choice A)Conditions producing systolic heart murmurs such as mitral regurgitation, aortic stenosis, or tricuspid regurgitation, are more related to heart failure symptoms. Even though aortic stenosis can be related to syncope, it does not cause amaurosis fugax . (Choice D)Acute retinal vein thrombosis can be diagnosed by fundoscopy,when extensive hemorrhage is seen in the retina. It will not present as amaurosis fugax. (Choice C)Papilledema is seen as a result of increased intracranial pressure. Pseudopapilledema, on the other hand, may be seen in an established nerve damage or in optic neuritis, but not in amaurosis fugax. Ischemia will manifest as disc paleness, not papilledema, especially in a transient event. Educational objective: Amaurosis fugax is a sudden and transient monocular blindness. Even though it is reversible, it is a marker of carotid artery atherosclerotic disease that is usually advanced. Physical examination will often reveal a carotid bruit. Carotid Doppler evaluation is necessary to evaluate the extent of the disease and to assess the need for a carotid endarterectomy.

Question 18

A 27-year-old male presents with progressively worsening headaches for the past three months.  For the past two weeks, he has felt that he is losing his balance.  He denies any motor weakness or sensory symptoms.  He does not have any history suggestive of cranial nerve paralysis.  On physical examination, his pupils do not react to light bilaterally.  He has an impaired upward gaze and loss of optokinetic nystagmus.  The rest of his eye movements are within normal limits.  On tandem walking, he tends to fall on both sides.  The rest of the physical examination is unremarkable.  What is the most likely diagnosis of this patient?

Accepted Answer

Explanation: A tumor of the pineal gland characteristically causes Parinaud's syndrome, which is characterized by the loss of pupillary reaction, vertical gaze paralysis, the loss of optokinetic nystagmus, and ataxia. The headache is a prominent feature, and is due to obstructive hydrocephalus. Some pineal tumors are germinomas and secrete HCG, which can cause precocious puberty in prepubertal males. (Choice A) Although craniopharyngioma can cause a headache, it cannot explain the other signs and symptoms of the patient. Craniopharyngiomas are classically associated with diabetes insipidus and a deficiency of one or more anterior pituitary hormones. (Choice B) Frontal lobe tumors can be silent. When symptomatic, these tumors present with headaches, focal neurological deficits, or seizures. Loss of inhibition from the frontal lobe can cause the release of primitive reflexes such as glabellar tap, grasp and palmomental reflexes. Frontal lobe tumors can be associated with Foster Kennedy syndrome (optic atrophy on the side of the tumor and papilledema on the contralateral side). (Choice C) A pituitary tumor with suprasellar extension can cause bitemporal hemianopsia. Patients with pituitary tumors can present with hormonal dysfunction, depending on the secretory state and size of the tumor. (Choice E) Eye findings in multiple sclerosis are due to involvement of the medial longitudinal fasciculus, which leads to internuclear ophthalmoplegia. Bilateral or unilateral optic neuritis can also occur. Educational Objective: Pineal tumor classically presents with Parinaud's syndrome, which is characterized by the loss of pupillary reaction, vertical gaze paralysis, the loss of optokinetic nystagmus, and ataxia. Some pineal tumors are germinomas that can secrete HCG.

Question 19

A 70-year-old man presents to the emergency department due to right-sided arm and leg weakness that occurred several hours ago.  The episode lasted 15 minutes and resolved spontaneously.  He had a similar episode of right arm weakness two days ago.  He was diagnosed with hypercholesterolemia on routine check-up three months ago, and has been treated with pravastatin.  He does not smoke or consume alcohol.  His blood pressure is 140/90 mmHg and his heart rate is 70/min.  Doppler studies shows 89% stenosis in his left carotid artery and 40% stenosis in his right carotid artery.  He undergoes left carotid endarterectomy.  The surgery goes well and he recovers from the anesthesia without any complications.  In the recovery room, upon protrusion of the tongue, the tongue deviates to the left.  Which of the following structures is most likely damaged?

Accepted Answer

Explanation: A number of nerve injuries can occur during carotid endarterectomy. Inadvertent retraction or transection of the hypoglossal nerve causes tongue deviation to the site of injury, as observed in this patient. (Choice C)Recurrent laryngeal nerve injury leads to unilateral vocal cord paralysis, with a resultant change in voice quality. This nerve lies distal to the area of carotid dissection. (Choice D)The facial nerve can be damaged after it exits from the stylomastoid foramen and courses along the inferior portion of the ear. Its marginal mandibular branch (which supplies the orbicularis oris muscle) is most commonly injured, with a resultant asymmetric smile. The vagus nerve lies posterolaterally in the carotid sheath and is also at risk for injury during the procedure (Choice A). The ansa hypoglossus nerve innervates the strap muscles of the neck; unlike the other nerves, this nerve may be sacrificed with impunity (Choice E). Educational objective: A number of nerve injuries can occur during carotid endarterectomy. Inadvertent retraction or transection of the hypoglossal nerve causes tongue deviation to the side of injury. *Extremely important question for USMLE step-3

Question 20

A 56-year-old man is brought to the office by his wife due to increasingly odd behavior.  The patient's wife says that her husband started to become emotionally "cold" toward her about a year ago and that he has since received a number of complaints from women at work, who report that he has been making unwanted advances.  She says, "He used to be a very loving and caring husband, but lately he doesn't appear to care about anything."  Over the past 6 months the patient's sleep has worsened and he has begun to exhibit memory difficulties; he has gotten lost a few times while driving home.  His wife also notes that he never used to have a sweet tooth, but she now cannot buy sweets because he will eat the entire box.  The patient has a history of hypertension and hyperlipidemia.  Which of the following is the most likely diagnosis?

Accepted Answer

Explanation: 11ec2b69_c18e_1f76_8f63_89d26b61f26e_MD0005_00 This patient's symptoms are consistent with a diagnosis of behavioral variant frontotemporal dementia (bvFTD). bvFTD frequently leads to early-onset dementia and usually presents around age 60 with ≥3 of the following clinical features: Disinhibited behavior, apathy, loss of empathy, perseverative behavior, and hyperorality. Early and marked personality and behavioral changes are typical and can help differentiate bvFTD from other dementias. Memory function is typically spared in the early stages of bvFTD, and neuropsychological testing may be in the normal range. Two-thirds of patients with bvFTD have evidence of frontotemporal atrophy on neuroimaging. Half of patients with bvFTD have an autosomal dominant inheritance pattern evident in family history. bvFTD is typically fatal within 8 years of symptom onset. Other variants of FTD include primary progressive aphasia (PPA), which presents with prominent and relatively isolated language impairment that may progress to impairment of other cognitive domains. (Choices A, B, and E) The differential diagnosis of bvFTD includes Alzheimer disease, dementia with Lewy bodies, and vascular dementia. Alzheimer disease typically presents with early, prominent memory impairment. Dementia with Lewy bodies is characterized by visual hallucinations, fluctuating attention/alertness, rapid-eye movement sleep behavior disorder, and/or parkinsonism. Vascular dementia typically presents in older patients with stepwise decline in cognitive function (temporally related to cerebrovascular events) and evidence of cerebrovascular disease on neuroimaging; early executive dysfunction is classic, and behavioral changes make bvFTD more likely. (Choice D) Huntington disease is an autosomal dominant disorder that usually presents around age 40 with chorea, psychiatric symptoms (eg, depression, anxiety), and abnormal eye movements (eg, abnormal saccades). Symptoms of dementia typically begin later in the course of Huntington disease. Educational objective: Behavioral variant frontotemporal dementia (bvFTD) typically presents with prominent personality and behavioral changes (eg, disinhibition, apathy, loss of empathy, perseverative behavior, and/or hyperorality). Memory may be preserved in early bvFTD, in contrast to the prominent early memory impairment that characterizes Alzheimer disease. References: Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. (http://www.ncbi.nlm.nih.gov/pubmed/21810890) Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. (http://www.ncbi.nlm.nih.gov/pubmed/21147039) Progression in behavioral variant frontotemporal dementia: a longitudinal study. (http://www.ncbi.nlm.nih.gov/pubmed/26501846)

Exam 2: Foundations of Independent Practice