Precipitous Deterioration of Motor Function, Cognition, and Behavior

Abstract

Following her son's death, a 72-year-old woman with a history of hypertension and hypothyroidism developed a year of severe depression, fell and broke her arm, and walked cautiously while dragging her right leg. A year later, she developed an unexplained dry cough and hoarseness. Two years after her initial fall, she complained of weakness and developed gait instability, needing to hold on to something when walking ("onset"). From this point, her deterioration was quick and included a sense of leg weakness, "not seeing well," and pronounced apathy. Seven months after "onset," an examination revealed symmetrical hyperreflexia with a jaw jerk and an overactive gag reflex but was otherwise normal, failing to identify weakness or gait unsteadiness. Between months 7 and 13, she was dizzy when turning without experiencing vertigo, and began losing her balance, falling twice. At 14 months, she needed a cane and could not concentrate or manage her checkbook, but she was oriented to the date, had a knowledge of recent events, and had a 3 of 3 oneminute recall.During months 14 through 16 she became more apathetic and depressed, had intermittent memory and orientation problems, worsening parkinsonism (bradykinesia, difficulty initiating movement, micrographia, emerging tremor, and intermittently needing a wheelchair) without ataxia, and favored her left leg when walking. By month 17 her apathy increased, and she occasionally dragged her right foot. On examination, she was alert and oriented to the month and year, had intact naming, and a normal cranial nerve examination, but she was intermittently tearful, had impaired spontaneous word generation and recall, mild hypomimia, mildly slowed bilateral rapid movements, retropulsion on standing, collapse on sitting, and normal stride only with assistance. She did, however, have normal strength, tone, sensation, reflexes, and plantar flexor responses without dysmetria.At 19 months, she had dysphagia, dysarthria, word-finding problems, difficulty turning in bed, and leg movements and talking during sleep. Carbidopa-levodopa failed to improve symptoms. A neurological examination also revealed a blunted affect, a 10 of 25 Montreal Cognitive Assessment score, mild hypomimia, right gaze diplopia, an impaired upgaze, moderately slowed rapid alternating movements, a bilateral pronator drift, extreme micrographia, and generalized parkinsonism, requiring assistance to rise from a chair and walk.At her first visit to the University of California, San Francisco Memory and Aging Center at 21 months, her family reported her intermittent word comprehension difficulty, paraphasias, pseudobulbar affect, and progressive myoclonus. Her examination showed distractibility, severe hypomimia, moderate hypophonia, slowed eyelid opening, a restricted vertical gaze, slowed horizontal and vertical saccades, a saccadic horizontal pursuit, marked axial and limb rigidity, extreme and predominantly right-sided bradykinesia, postural tremor, myoclonus, a marked grasp reflex, brisk reflexes, and upgoing plantar ...