NMS Q&A Family Medicine

Neurology 778. The answer is A. The only therapy that may change thecourse of the disease is ventriculoperitoneal shunt todecompress the cerebrospinal fluid (CSF; however, part ofthe definition of NPH is a “normal” pressure of CSF).Success is best achieved when the symptoms have been ofrelatively short duration such as in the presented case.Even then, estimates of success vary widely, from 33% to90%.9. The answer is E. This clinical picture in a youngwoman of Northern European descent, that of scatteredneurologic findings that exacerbated and remitted andrecurred 3 months later, fits the classic picture of multiplesclerosis. Females are affected twice as frequently as males.The MRI of the brain is nearly completely sensitive,though not perfectly specific. The MRI performed with ahigh-field magnet ( 1.5 T) should show areas of highsignal in the white matter of the brain and spinal cord onT2-weighted images, characteristic of demyelinatinglesions in MS. Therefore, the demyelinating process affectsboth sensory and motor fibers. Lumbar puncture is alsovery helpful in disclosing CSF immunoglobulin-G concentrationincreased relative to other CSF proteins, suchas albumin with oligoclonal bands (90% sensitive and lessspecific than MRI). Lyme disease and subacute combineddegeneration (as in pernicious anemia) should be entertainedand ruled out, the latter by the B 12 level, but theyhave no value in the diagnosis of MS per se. Other teststhat address diseases in the differential diagnosis includethyroid-stimulating hormone, sedimentation rate, antinuclearantibodies, and a serologic test for syphilis. Amore refined list would address more rare conditions, notlikely to be relevant in the early stages of this syndromeencountered in primary care.10. The answer is D. Infliximab is a tumor necrosis factorinhibitor used in rheumatoid arthritis and other autoimmunediseases. Of interest is the fact that, as understandingof MS has developed, it has emerged as an autoimmunedisease. The other drugs mentioned are all capable of aroughly one-third reduction in the rate of exacerbation ofthe disease and appear to address the autoimmune aspectsof MS. A new drug, now approved for Phase II testing, isnatalixumab (Antegren, Tsar), whose early studies indicatea 90% reduction in acute activity and 50% reductionin number of relapses.11. The answer is A. Reversible causes of dementiainclude pernicious anemia, hypothyroidism (especially inthe elderly population), and depression. Serum electrolyteswould diagnose hyponatremia, a cause of stupor, notdementia; liver function blood tests or biopsy would diagnosehepatic coma; carotid angiogram would elucidatecerebral arterial blood flow, which is important in focalneurological symptoms as in transient ischemic attack orstroke. The other modalities and tests offer no significantinformation for diagnosing dementia or disturbance ofconsciousness.12. The answer is B. The tremor in PD is initially unilateral,only later involving both sides. Choreiform movementsare rapid, purposeless movements that occur inHuntington chorea (wormlike movements) and Sydenhamchorea (jerky movements of the extremities, particularlyhands and feet), but not in PD. About 20% of PDpatients eventually develop a dementia, and autonomicfeatures including orthostatic hypotension are common.Constipation and impotence can also be seen. The gait isshuffling with little arm swing. The speech is very soft andmonotonal but delirium is not a part of the picture.13. The answer is C. Orphenadrine (Disapal, Norflex) isan anticholinergic medication. This class of drugs is mosthelpful when the main goal of therapy is to control rigidityand tremor. Other examples of this class used in PDinclude benztropine mesylate (Cogentin), biperidin (Akineton),procyclidine (Kemedrin), and trihexylphenidyl(Artane). Although other medications are superior inimproving rigidity and bradykinesia such as bromocriptine,a dopaminergic drug, the anticholinergics are moreeffective in diminishing the tremor. Dopaminergic drugsare effective in relieving rigidity and bradykinesia.Levodopa alleviates all aspects of PD but does not alterthe progression of the disease. Sinemet is a fixed combinationof carbidopa and levodopa. Selegiline is a monoamineoxidase inhibitor that is sometimes used as anadjunct to levodopa to control the fluctuations in itseffect. Amitriptyline is an antidepressant with stronganticholinergic side effects but is not used in the treatmentof PD.14. The answer is D. This patient has Guillain–Barrésyndrome, which is thought to be based on an autoimmuneprocess. The condition is also called demyelinatingpolyradiculoneuropathy. Twenty percent of cases appearto have links with Campylobacter jejuni infection. Mostother cases have followed apparent viral gastroenteritis,viral upper respiratory tract infection, or influenza byabout 1 to 3 weeks. The disease, though potentially lifethreatening (3% to 5% case mortality) because of theinvolvement of respiratory and deglutition functions, isusually self-limited and reverses within a few weeks. Thedifferential diagnosis includes botulism, intermittent porphyria,diphtheria, Lyme disease, poliomyelitis, heavymetal poisoning, and tetrodotoxin poisoning from contaminatedshellfish. Chronic inflammatory demyelinatingneuropathy is similar in symptomatology and signs exceptthat it is chronic, remitting, and exacerbating. Combinedupper and lower motor neuron disease is a description ofALS. Both sensorimotor and pure sensory polyneuropathiescan occur as nonmetastatic complications of malignancies.On some occasions, this syndrome may precede