All questions
Question 1
A 32-year-old woman presents to the emergency department with a 2-day history of blurry vision and pain with movement in her right eye. She also reports that colors appear 'washed out' with that eye. She has a history of an episode of tingling and numbness in her left leg that resolved spontaneously 18 months ago. On examination, her visual acuity is 20/100 in the right eye and 20/20 in the left. She has a right afferent pupillary defect. The remainder of her neurologic examination is normal. An MRI of the brain and orbits is ordered.
Pending the MRI results, which of the following is the most appropriate next step in management for her current symptoms?
- High-dose intravenous corticosteroids (correct answer)
- Initiation of interferon beta-1a
- Plasma exchange
- Oral prednisone taper
Explanation: This patient's presentation of painful monocular vision loss with an afferent pupillary defect is classic for optic neuritis, a common presenting symptom of multiple sclerosis (MS). Her previous episode of neurologic symptoms suggests dissemination in time. An acute MS exacerbation causing significant functional disability (in this case, vision loss) is treated with high-dose intravenous corticosteroids (e.g., methylprednisolone) to shorten the duration of the relapse. Oral prednisone is less effective and may increase the risk of recurrent optic neuritis. Interferon beta-1a is a disease-modifying therapy used for long-term management, not acute flares. Plasma exchange is reserved for severe, steroid-refractory relapses.
Question 2
A 65-year-old man presents with a 9-month history of progressive weakness. It began as difficulty with fine motor tasks in his right hand, such as buttoning his shirt. Now, he notes tripping frequently and has weakness in his left foot. He also reports muscle twitching in his arms and cramping in his calves. On examination, there is asymmetric weakness and atrophy of the intrinsic hand muscles and tibialis anterior. Fasciculations are noted in his biceps and quadriceps. Deep tendon reflexes are 3+ in the upper and lower extremities, and Babinski sign is present bilaterally. Sensation is intact.
Which of the following is the most likely diagnosis?
- Amyotrophic lateral sclerosis (correct answer)
- Cervical spondylotic myelopathy
- Guillain-Barré syndrome
- Myasthenia gravis
Explanation: This patient's clinical presentation demonstrates a combination of both upper motor neuron (UMN) signs (hyperreflexia, Babinski sign) and lower motor neuron (LMN) signs (asymmetric weakness, atrophy, fasciculations). This constellation of findings in a progressive, painless pattern is classic for amyotrophic lateral sclerosis (ALS). Cervical myelopathy can cause mixed UMN/LMN signs but typically involves the neck and may have sensory deficits. Guillain-Barré syndrome is an acute, ascending paralysis, often with areflexia. Myasthenia gravis presents with fluctuating, fatigable weakness, typically affecting ocular and bulbar muscles first, and does not cause UMN signs.
Question 3
A 75-year-old man with a recent diagnosis of dementia develops vivid visual hallucinations of small children playing in his room. He has fluctuating levels of attention and alertness throughout the day. On examination, he has mild resting tremor and bradykinesia. When his physician attempted to treat the hallucinations with a low dose of haloperidol, the patient developed severe rigidity, confusion, and fever. His symptoms improved after the medication was stopped.
Which of the following is the most likely diagnosis?
- Alzheimer's disease with psychosis
- Dementia with Lewy bodies (correct answer)
- Frontotemporal dementia
- Vascular dementia
Explanation: This patient's presentation includes the core features of dementia with Lewy bodies (DLB): fluctuating cognition, visual hallucinations, and parkinsonism. Furthermore, his severe adverse reaction to haloperidol, a typical antipsychotic, demonstrates the classic neuroleptic sensitivity seen in DLB. While Alzheimer's disease can have psychosis, the combination of all these features, especially the fluctuating cognition and profound antipsychotic sensitivity, makes DLB the most likely diagnosis.
Question 4
A 45-year-old woman with a 10-year history of relapsing-remitting multiple sclerosis presents for a routine follow-up. She reports increasing difficulty walking due to leg stiffness and frequent, painful muscle spasms, especially at night. On examination, she has increased tone and hyperreflexia in both lower extremities. Her gait is stiff and slow.
Which of the following is the most appropriate initial medication to manage this patient's primary complaint?
- Baclofen (correct answer)
- Gabapentin
- Amantadine
- Modafinil
Explanation: The patient's symptoms of leg stiffness, increased tone, and painful spasms are characteristic of spasticity, a common and disabling symptom in multiple sclerosis resulting from upper motor neuron damage. Baclofen, a GABA-B agonist, is a first-line pharmacologic treatment for MS-related spasticity. Tizanidine is another first-line option. Gabapentin is primarily used for neuropathic pain. Amantadine and modafinil are used to treat MS-related fatigue.
Question 5
A 62-year-old woman is diagnosed with amyotrophic lateral sclerosis after a comprehensive workup. She has moderate limb weakness but her respiratory function is currently preserved. She and her family want to pursue all available treatments to slow the disease progression. She has no significant liver dysfunction.
Which of the following medications has been shown to modestly prolong survival in patients with this condition?
- Edaravone
- Prednisone
- Riluzole (correct answer)
- Intravenous immunoglobulin
Explanation: Riluzole, a glutamate inhibitor, is the first medication approved for amyotrophic lateral sclerosis (ALS) and has been shown in clinical trials to provide a modest survival benefit, typically extending survival by 2-3 months. It is considered a standard of care for patients with ALS. Edaravone is another approved medication that may slow functional decline in a subset of patients, but riluzole is the classic agent with proven survival benefit. Corticosteroids like prednisone and IVIG are not effective in treating ALS.
Question 6
A 55-year-old man with a recent diagnosis of amyotrophic lateral sclerosis (ALS) is having a discussion about long-term management with his neurologist. The patient is concerned about future breathing difficulties. His forced vital capacity (FVC) is currently 85% of predicted.
Which of the following interventions is most critical to monitor for and implement as his disease progresses to manage respiratory complications?
- Prophylactic tracheostomy
- Routine incentive spirometry
- Noninvasive positive pressure ventilation (NPPV) (correct answer)
- Long-term oxygen therapy
Explanation: Respiratory failure is the most common cause of death in ALS due to progressive weakness of the diaphragm and other respiratory muscles. Noninvasive positive pressure ventilation (NPPV), typically initiated at night when FVC drops below 50% or when symptoms of nocturnal hypoventilation develop, is the standard of care. NPPV has been shown to improve quality of life and prolong survival. Prophylactic tracheostomy is an invasive option considered later. Incentive spirometry is not effective for neuromuscular weakness. Oxygen therapy is not indicated unless hypoxemia is present, and it can worsen hypercapnia from hypoventilation.
Question 7
A 79-year-old woman is evaluated for a 1-year history of cognitive decline. Her husband reports that she has significant trouble remembering recent events and often repeats questions. However, what he finds most concerning is her tendency to get lost while driving in their familiar neighborhood. She also has difficulty dressing herself and copying simple diagrams. On examination, she has poor short-term memory and notable difficulty with drawing a clock face.
This patient's difficulty with navigation and dressing suggests impairment in which of the following cognitive domains?
- Executive function
- Language
- Visuospatial function (correct answer)
- Attention
Explanation: The patient's symptoms of getting lost in familiar places, apraxia (difficulty with motor planning for tasks like dressing), and inability to copy diagrams or draw a clock are classic manifestations of impaired visuospatial function. While Alzheimer's disease (the likely diagnosis) affects multiple domains, these specific symptoms point to significant parietal lobe dysfunction, which is responsible for visuospatial processing. Executive dysfunction would manifest as problems with planning and problem-solving. Language impairment would involve aphasia. Attention deficits would present as distractibility.
Question 8
A 40-year-old woman with multiple sclerosis complains of a recurrent, brief, stabbing pain in the right side of her face, often triggered by chewing or touching her cheek. The pain is electric-shock like and lasts for a few seconds. Neurological examination is normal between episodes.
Which of the following is the most appropriate first-line medication for this patient's condition?
- Amitriptyline
- Carbamazepine (correct answer)
- Sumatriptan
- Propranolol
Explanation: The patient's symptoms are characteristic of trigeminal neuralgia, which is a common and often debilitating symptom in patients with multiple sclerosis due to a demyelinating plaque in the trigeminal nerve root entry zone. The first-line treatment for trigeminal neuralgia is carbamazepine, an anticonvulsant that stabilizes neuronal membranes. Oxcarbazepine is also a first-line option. Amitriptyline is a second-line agent. Sumatriptan is for migraine, and propranolol is for migraine prophylaxis or tremor.
Question 9
A 25-year-old man presents with a 3-day history of bilateral leg weakness and a band-like sensation around his mid-torso. Examination reveals paraplegia, a T8 sensory level to all modalities, and urinary retention requiring catheterization. An MRI of the spine confirms a longitudinally extensive, T2-hyperintense lesion spanning from T7 to T10 with contrast enhancement. A brain MRI is normal. Lumbar puncture is performed. Analysis of the cerebrospinal fluid shows pleocytosis and elevated protein, but oligoclonal bands are absent.
The absence of oligoclonal bands and the presence of a longitudinally extensive spinal cord lesion makes which diagnosis more likely than classic multiple sclerosis?
- Neuromyelitis optica spectrum disorder (correct answer)
- Acute disseminated encephalomyelitis
- Spinal dural arteriovenous fistula
- Progressive multifocal leukoencephalopathy
Explanation: This patient presents with severe, longitudinally extensive transverse myelitis (a lesion spanning three or more vertebral segments). While transverse myelitis can occur in multiple sclerosis (MS), the lesions are typically shorter. The combination of a longitudinally extensive lesion, a normal brain MRI, and negative CSF oligoclonal bands is highly suggestive of Neuromyelitis Optica Spectrum Disorder (NMOSD), an autoimmune condition often associated with antibodies against aquaporin-4. Distinguishing NMOSD from MS is critical as treatments differ significantly.
Question 10
An 82-year-old woman with severe Alzheimer's disease is admitted from a nursing home with pneumonia. She is nonverbal and dependent on staff for all activities of daily living. Her daughter, who is her healthcare proxy, is asked about goals of care. The daughter is tearful and says, 'I just want everything done to keep her alive.' The patient has no living will or advance directive.
What is the most appropriate response by the physician?
- Agree to provide full cardiopulmonary resuscitation and mechanical ventilation if needed.
- Explain the patient's poor prognosis and recommend comfort-focused care.
- Consult the hospital ethics committee to override the daughter's request.
- Ask the daughter about her mother's values and what she would have wanted in this situation. (correct answer)
Explanation: When a patient lacks decision-making capacity and has no advance directive, the healthcare proxy should make decisions based on the principle of substituted judgment—what the patient would have wanted for herself. The physician's role is to guide the proxy in this process. Instead of immediately imposing a recommendation or blindly following the initial emotional request, the most appropriate step is to explore the patient's values, beliefs, and previously expressed wishes with the daughter. This patient-centered approach helps the proxy make a decision that truly honors the patient, rather than one based on guilt or misunderstanding.
Question 11
A 69-year-old retired pilot is evaluated for cognitive concerns. His wife states that his memory is 'as sharp as ever' and he can still manage their complex finances. However, over the past 3 years, he has developed progressive difficulty with his speech. He speaks slowly, with great effort, and often uses the wrong words or mispronounces them. He becomes frustrated because he knows what he wants to say but cannot get the words out correctly. His comprehension is intact. Neurologic examination is otherwise normal.
This patient's presentation is most consistent with which of the following neurodegenerative syndromes?
- Logopenic variant primary progressive aphasia
- Semantic variant primary progressive aphasia
- Nonfluent/agrammatic variant primary progressive aphasia (correct answer)
- Behavioral variant frontotemporal dementia
Explanation: This patient has an isolated, progressive language disorder (primary progressive aphasia, PPA). His symptoms of effortful, halting speech with sound errors (phonemic paraphasias) and incorrect grammar (agrammatism), in the setting of preserved comprehension and memory, are the hallmark features of the nonfluent/agrammatic variant of PPA. This is a form of frontotemporal lobar degeneration. Logopenic PPA is characterized by word-finding difficulty and impaired repetition (often associated with Alzheimer's pathology). Semantic variant PPA involves loss of word meaning.
Question 12
A 48-year-old man with a 15-year history of multiple sclerosis presents with gradually worsening bilateral leg weakness and gait instability over the past 2 years, without any discrete relapses. He now requires a cane to walk 100 meters. His MRI shows a high burden of chronic T2 lesions and evidence of spinal cord atrophy, but no new or enhancing lesions.
This patient's clinical course is best described as which of the following?
- Relapsing-remitting MS
- Clinically isolated syndrome
- Secondary progressive MS (correct answer)
- Primary progressive MS
Explanation: Secondary progressive multiple sclerosis (SPMS) is characterized by a gradual worsening of neurologic disability that is independent of relapses, following an initial relapsing-remitting course. This patient had a history of MS (implying a prior relapsing course) and now has steady progression of disability for 2 years without acute attacks. This transition from relapsing-remitting to progressive disease defines SPMS. Primary progressive MS (PPMS) involves progression from disease onset, without an initial relapsing phase. Clinically isolated syndrome is the first clinical episode suggestive of MS.
Question 13
A 70-year-old man is brought for evaluation of cognitive impairment. His wife reports that for the past 2 years, he has had progressive memory loss. She also notes that he has started acting out his dreams, sometimes shouting or flailing his arms and legs while asleep, occasionally injuring himself. Examination reveals mild cogwheel rigidity and a slow gait. His memory is impaired on formal testing.
The sleep-related symptom described in this patient is most strongly associated with which of the following underlying pathologies?
- Beta-amyloid plaques
- Alpha-synuclein deposition (correct answer)
- Tau protein neurofibrillary tangles
- TDP-43 protein inclusions
Explanation: The patient's clinical picture, including dementia, parkinsonism (rigidity, slow gait), and REM sleep behavior disorder (acting out dreams), is highly suggestive of a synucleinopathy, most likely Dementia with Lewy Bodies (DLB) or Parkinson's disease dementia. REM sleep behavior disorder is caused by the loss of normal muscle atonia during REM sleep and is very strongly associated with the deposition of alpha-synuclein in the brainstem. Beta-amyloid plaques and tau tangles are the hallmarks of Alzheimer's disease. TDP-43 is associated with frontotemporal dementia and ALS.
Question 14
A 28-year-old woman presents with subacute onset of ascending numbness and weakness in her legs over the past 4 days, making it difficult to walk. On examination, she has 4/5 weakness in the lower extremities bilaterally and loss of vibration and proprioception up to the level of her umbilicus. Deep tendon reflexes are 3+ in the lower extremities with bilateral Babinski signs. She also reports urinary urgency. An MRI of the spine with contrast is performed.
The MRI is most likely to show a lesion consistent with which of the following conditions?
- Transverse myelitis (correct answer)
- Spinal epidural abscess
- Anterior spinal artery infarct
- Vitamin B12 deficiency
Explanation: This patient's presentation of subacute, bilateral motor and sensory deficits below a specific spinal cord level (T10 dermatome, at the umbilicus), along with upper motor neuron signs (hyperreflexia, Babinski) and bladder dysfunction, is classic for transverse myelitis. This is a common manifestation of multiple sclerosis. MRI of the spine would show a T2-hyperintense, often contrast-enhancing lesion within the spinal cord. An epidural abscess would typically present with fever and severe back pain. An anterior spinal artery infarct spares the dorsal columns (vibration/proprioception). Vitamin B12 deficiency has a more indolent onset.
Question 15
A 35-year-old woman is diagnosed with relapsing-remitting multiple sclerosis after presenting with optic neuritis and an MRI showing multiple T2 hyperintense lesions in the periventricular white matter and corpus callosum, some of which enhance with gadolinium. She has fully recovered from the optic neuritis after a course of steroids.
Which of the following is the most appropriate next step to reduce the risk of future relapses and disability progression?
- Begin a disease-modifying therapy (correct answer)
- Schedule annual courses of intravenous steroids
- Administer monthly intravenous immunoglobulin
- Observe with serial MRI scans every 3 months
Explanation: Following a diagnosis of relapsing-remitting multiple sclerosis (RRMS), the standard of care is to initiate a long-term disease-modifying therapy (DMT). These medications (e.g., interferons, glatiramer acetate, natalizumab, ocrelizumab) are proven to reduce the frequency of relapses, delay the accumulation of disability, and decrease the development of new MRI lesions. Steroids are used for acute relapses only, not for long-term prevention. IVIG is not a standard DMT for MS. Observation alone is inappropriate as it allows for irreversible neurologic damage to occur.
Question 16
A 72-year-old woman presents with a 6-month history of memory difficulties and gait instability. Her family reports a stepwise decline in her cognitive function, with periods of stability followed by sudden worsening. Her past medical history is significant for hypertension, type 2 diabetes mellitus, and hyperlipidemia. On examination, she has a broad-based, shuffling gait. Focal weakness is noted in her left lower extremity. An MRI of the brain reveals multiple cortical and subcortical infarcts of varying ages.
In addition to lifestyle modifications, which of the following is the most important component of this patient's long-term management?
- Aggressive control of vascular risk factors (correct answer)
- Initiation of a cholinesterase inhibitor
- Levodopa therapy for gait disturbance
- Referral for deep brain stimulation
Explanation: This patient's presentation of a stepwise cognitive decline in the setting of multiple vascular risk factors and focal neurologic findings, confirmed by MRI evidence of multiple infarcts, is characteristic of vascular dementia. The primary goal of management is to prevent further strokes and cognitive decline. This is best achieved through aggressive control of vascular risk factors, including hypertension, diabetes, and hyperlipidemia, along with antiplatelet therapy. While cholinesterase inhibitors are sometimes used off-label, their efficacy is limited, and risk factor control is paramount. Levodopa is for Parkinson's disease, and deep brain stimulation is not indicated.
Question 17
A 68-year-old man is brought to the clinic by his wife due to a 2-year history of progressive memory loss. She notes he frequently misplaces items, forgets conversations, and has difficulty managing their finances. He has become more withdrawn and apathetic. He has no history of stroke. A Mini-Mental State Examination score is 22/30, with prominent deficits in recall and orientation. Neurologic examination is otherwise unremarkable. Laboratory studies, including vitamin B12, TSH, and RPR, are within normal limits. An MRI of the brain shows moderate, diffuse cortical atrophy, most prominent in the medial temporal lobes.
Which of the following is the most appropriate initial pharmacologic treatment for this patient's cognitive symptoms?
- Donepezil (correct answer)
- Levodopa/carbidopa
- Memantine
- Sertraline
Explanation: This patient presents with a classic insidious onset and progressive course of cognitive decline, particularly affecting memory, consistent with Alzheimer's disease (AD). The MRI findings of medial temporal lobe atrophy further support this diagnosis. Donepezil, an acetylcholinesterase inhibitor, is a first-line medication for mild to moderate AD and is the most appropriate initial treatment to manage cognitive symptoms.
Question 18
A 66-year-old man with a 5-year history of amyotrophic lateral sclerosis presents with difficulty swallowing solids and liquids, frequent coughing during meals, and a 10-lb weight loss over the past 3 months. His speech has become markedly slurred and difficult to understand.
Which of the following is the most appropriate intervention to address this patient's nutritional and hydration needs and reduce the risk of aspiration?
- Initiation of a pureed diet and thickened liquids
- Placement of a percutaneous endoscopic gastrostomy (PEG) tube (correct answer)
- Total parenteral nutrition (TPN)
- Prescription of an anticholinergic agent to reduce secretions
Explanation: This patient is exhibiting signs of severe bulbar dysfunction (dysphagia and dysarthria), a common feature of ALS, leading to weight loss and risk of aspiration pneumonia. While dietary modifications like pureed diet are a temporary measure, they often become insufficient and unsafe as dysphagia progresses. Placement of a percutaneous endoscopic gastrostomy (PEG) tube is the standard of care to provide long-term nutritional support, maintain hydration, and reduce the risk of aspiration in patients with ALS and significant dysphagia. It is best placed before respiratory function declines significantly. TPN is invasive and has more complications. Anticholinergics can help with sialorrhea but do not solve the swallowing problem.
Question 19
A 78-year-old man with Alzheimer's disease is brought in by his daughter, who is his primary caregiver. She is overwhelmed by his recent behavioral changes, including agitation, nighttime wandering, and occasional aggression. She asks for medication to 'calm him down'. The patient is not a danger to himself or others at this time.
Which of the following is the most appropriate next step in management?
- Initiate a low-dose atypical antipsychotic
- Prescribe a benzodiazepine for anxiety
- Increase the dose of his donepezil
- Attempt non-pharmacologic behavioral interventions (correct answer)
Explanation: For behavioral and psychological symptoms of dementia (BPSD), non-pharmacologic interventions are first-line, especially when the patient is not an immediate danger. These strategies involve identifying and modifying triggers (e.g., pain, hunger, boredom), establishing routines, providing a calm environment, and using techniques like redirection and reassurance. Antipsychotics carry a black box warning for increased mortality in elderly patients with dementia and should be reserved for severe symptoms that have not responded to non-pharmacologic approaches and pose a risk of harm. Benzodiazepines can worsen confusion and increase fall risk. Increasing donepezil is for cognitive symptoms, not primarily for agitation.