Gertie, a 66-year-old woman, was brought to the hospital by her family because she could neither walk nor feed herself. An alcoholic in remission, she had stopped drinking 6 years previously. Slurred speech, her first neurologic symptom, began at about the same time. Two years later, her gait became unsteady, and then worsened progressively. Most recently, her speech had become so dysarthric, that she could not be understood over the telephone. She had been taking no medication, and had no history of thyroid disease.
On examination, Gertie was a thin woman with an intact intellect, who spoke in a slurred, sing-song voice, which was accentuated when she repeated "Happy Hippopotamus". She was able to sit unaided, but could not walk. Her optic fundi were normal. Eye movements were full and conjugate, with nystagmus. Hearing was intact. Her limbs were strong but dysmetric, wildly so in her legs, and dysmetric enough on finger-to-nose testing that she closed her eyes as her hand approached her face. Ankle jerks were absent, and other reflexes were hypoactive; knee jerks had a pendular swing. Plantar reflexes were flexor. Pain sensation was blunted distal to her mid-calves in a stocking distribution. Perception of vibration (128 Hz) and proprioception were intact in her fingers and toes.
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