54 yo has several days of low-grade fever, malaise and severe pain on R side of ribcage. On exam, erythematous rash with clusters of dense vesicles, with clear content, on a belt distribution from the front of the chest to the back under the nipple, limited to the R side. Likely causal viral agent? (4x)
Varicella Zoster Virus
79 yo w/ decreasing mental state over 3 weeks has an exaggerated startle response with violent myoclonus that is elicicited by turning on the room lights, speaking loudly, or touching the pt. Myoclonic jerks occur spontaneously, ataxia. EEG: sharp waves. Dx? (5x)
Subcutaneous Spongiform Encephalopathy
98M in ER, unconscious atfer choking, had progressive neuro condition that presented in his early 30’s w/ involuntary irregular movements of all extremities & face but afer 15 yr course evolved into rigid, akinetic condition w/ diff swallowing, speaking. Also progressive dementia & full-time care. After obstruction was relieved pt remained unconscious, had cardiac arrest & died. Post-mortem exam showed generalized brain atrophy. (Pathology picture showing brain atrophy). Dx? (4x)
Huntington's disease
37 yo truck driver w/ numbness of L hand, increased severity in past 2 yrs. Reduced pinprick sensation on L little/ring fingers, atrophy of hypothenar muscle. Dx? (6x)
Ulnar nerve lesion
Persistent numbness in L hand, decreased sensation in 4th/5th digits (palmar/dorsal), weak finger abduction/adduction especially 5th digit. Likely dx? (4x)
Ulnar nerve entrapment at the elbow
17 yo has an insidious onset of unusual behavior and argumentativeness. Exam, the mouth is held slightly open. Pt has mild dysarthria and hoarseness, generalized slowness, rigidity, and a mild resting tremor of the L arm and head. Ruled out drug and/oralcohol abuse. LFTs show elevated transaminases. An increase in which laboratory test is most likely to confirm dx? (4x)
Urinary copper excretion
Parkinson’s treated w/ levodopa. Gets VH. Recommendations? (5x)
Reduce Dose of Levodopa
Pt w/ depression, 3 yr hx of change in personality, irritability, impulsive outbursts, cognitive deterioration, & eccentric or inappropriate social interactions. He subsequently lost his job & is now withdrawn & fidgety. Pt has increased eye blinking, marked tongue impersistence, mild bradykinesia, akinesia & mild hyperreflexia w/o clonus. The pt’s dad died ot severe dementia at 55. Dx? (4x)
Huntington's disease
22 yo w/ pain in R hand that radiates into the forearm and bicep. Paresthesia in the palm of the hand, thumb, index, middle ring finger. Sensory systems in the ring finger split the ring finger longitudinally. Dx? (6x)
Median nerve entrapment at the wrist
Most effective treatment for a patient with involuntary eye blinking? Neuro exam shows symmetric, involuntary contraction of the orbicularis oculi muscles but otherwise unremarkable. (4x)
Botulinum toxin
First-line tx for restless leg syndrome: (7x)
Typical of idiopathic Parkinson’s disease, rather than another Parkinsonian syndrome: (5x)
Asymmetrical onset and progression of motor symptoms
36 yo w/ pain behind L ear progressing to numbness on L side of face, tearing of L eye, discomfort w/ low frequency sounds, L facial weakness on exam. Dx? (12x)
Idiopathic Bell's Palsy
Stiffness of legs while walking and spasms of LE while sleeping. Stiff-legged gait, adducts legs while walking. Increased LE tone/spastic catch, hyperactive knee jerks, ankle jerk clonus. Increased Romberg sway. Dx? (6x)
Cervical Spondylosis
54 yo had a viral upper respiratory infection 2 weeks ago and now presents w/ 3 day episode of progressive, symmetric weakness in the legs, and tingling in the toes and fingers. On exam, Achilles and patellar DTRs diminished. Nerve conduction studies demonstrate decreased conduction velocity and decreased amplitude of action potentials. Likely dx? (4x)
ACUTE INFLAMMATORY DEMYELINATING POLYNEUROPATHY
42M w/ a gradually progressive cognitive deficit also develops jerking movements of extremities, head, and trunk. Imaging shows b/l caudate nuclei atrophy. Pt’s teacher had a similar illness that started at 50 yo. Test to confirm dx? (6x)
DNA analysis for CAG repeats
First-line tx for 65 yo w/ hx of asthma and essential tremor: (4x)
Primidone
Myasthenia gravis associated w/ what EMG finding: (10x)
DECREASED AMPLITUDE WITH REPETITIVE MOTOR NERVE STIMULATION
Atrophy of the intrinsic muscles of the R arm and forearm. Reflexes are generally brisk, plantar reflexes are extensor. Electrophysiology shows widespread fasciculations, fibrillation and sharp waves, normal sensation, muscle spasticity. Positive sharp waves on EMG. Dx? (6x)
Amyotrophic Lateral Sclerosis
Young pt w/ a few days of progressive weakness and numbness of both legs and feet after recovering from a flu-like illness. Exam: weakness and loss of sensation to all sensory modalities below middle of the thorax. DTR: brisker on the lower extremities, plantar reflexes are extensor. Pt has had several episodes of urinary incontinence. Other neuro exam and vitals: normal. LP: 23 mononuclear cells, protein 37 mg/dl, and normal glucose. Dx? (4x)
Acute transverse myelitis
What MRI finding would most specifically indicate a dx of Huntington’s disease? (6x)
Caudate head atrophy
Medication useful in management of orthostatic hypotension in Parkinson’s: (4x)
Fludrocortisone
Tx for trigeminal neuralgia: (7x)
Gabapentin (but most effective is Carbamazepine)
R neck pain, tends to rotate neck to L – touching the chin prevents deviation – prominent R SCM spasm. Tx? (5x)
Botulinum toxin
10 yo w/ frequent episodes of brief lapses of consciousness w/o premonitory sxs. Lasts 2-10 seconds, followed by immediate and full resumption of consciousness without awareness of what happened. These ictal episodes are most likely caused by what kind of seizures? (4x)
Absence