A case of alcoholic pellagra encephalopathy presenting with spinal myoclonus

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A 56-year-old man with a history of heavy alcohol consumption (100 g/day for 2 years) had disorientation and memory disturbance for 4 months. He was unable to walk because of instability and then experienced visual and auditory hallucinations. He was admitted to a local hospital and became fully oriented and able to walk after IV administration of thiamine and multivitamin preparations without niacin. One month later, however, his mental status fluctuated and deteriorated again. The patient developed dysuria, gait disturbance, and lower extremity myoclonus and was referred to our hospital. He was emaciated (height 170 cm; weight 47.6 kg; body mass index 16.4), and he had a sacral decubitus ulcer. His face was darkened and hyperkeratotic. He was disoriented and irritated, with flapping tremors. Lower extremity myoclonus was observed every 20–30 seconds (see video at [Neurology.org/cp][1]), which was triggered by a touch on the legs and worsened in the supine position, even during sleep. Deep tendon reflex was exaggerated (more evident at knee jerks than ankle jerks), and Babinski sign was positive bilaterally. Finger-nose-finger and heel-knee tests revealed cerebellar ataxia. Romberg sign was positive and joint position sense of the great toes was disturbed. He had stocking sensory impairment. The patient also had orthostatic hypotension, constipation, and urinary retention and incontinence. [1]: http://cp.neurology.org/lookup/doi/10.1212/CPJ.0000000000000165