Progressive facial and oral weakness and emotional lability, sometimes resulting in flaccid quadriplegia and Òlocked-inÓ state. Onset occurs in adulthood; condition affects alcoholics and patients with nutritional depletion. Associated disorders are Wernicke encephalopathy, WilsonÕs disease, organ transplantation, diabetes, amyloidosis, and leukemia. Treatment is adequate nutrition, thiamine, and correction of electrolyte abnormalities.