The thalamus is affected in diffuse degenerative processes of the nervous system.1 2 3 4 5 However, it has not been established whether a genetically determined degenerative disease may be limited to selected thalamic nuclei. We report here the case of a 53-year-old man who presented with progressive insomnia and dysautonomia (pyrexia, diaphoresis, miosis, and sphincter disturbances). Dreamlike status, dysarthria, tremor, and myoclonus subsequently developed and led to coma and death after nine months.6 Two sisters of the patient and many relatives over three generations had died of a similar disease. Pathological studies of the brains of the patient and one of his sisters
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