The thalamus is affected in diffuse degenerative processes of the nervous system.12345 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… © 1986, Massachusetts Medical Society. All rights reserved.
Lugaresi E., Medori R., Montagna P., Baruzzi A., Cortelli P., Lugaresi A., et al. (1986). Fatal Familial Insomnia and Dysautonomia with Selective Degeneration of Thalamic Nuclei. THE NEW ENGLAND JOURNAL OF MEDICINE, 315(16), 997-1003 [10.1056/NEJM198610163151605].
Fatal Familial Insomnia and Dysautonomia with Selective Degeneration of Thalamic Nuclei
Lugaresi E.;Montagna P.;Baruzzi A.;Cortelli P.;Lugaresi A.Investigation
;Tinuper P.;
1986
Abstract
The thalamus is affected in diffuse degenerative processes of the nervous system.12345 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… © 1986, Massachusetts Medical Society. All rights reserved.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
