From: Sporadic fatal insomnia in a young woman: A diagnostic challenge: Case Report
Date | Clinical signs and symptoms |
---|---|
August 2005 | Onset: age 31†Increased attention deficit†Progressive memory loss†Sleep disturbance‡ |
February 2006 | Bizarre behavior‡ Sitting in chair making loud incoherent noises‡ |
April 2006 | MRI - negative for intracranial abnormalities†|
June 2006 | Anisocoria†Increased agitation‡ Incoherent speech‡ Balance and gait difficulties‡ Talking to self†Referred to psychiatry†Decreased attention, registration and calculation†|
July 2006 | Electroencephalogram (EEG) - bilateral periodic epileptiform discharges†|
August 2006 | Flat affect†Continued decrease in attention, registration and calculation†|
October 2006 | Confused†Constant movement†|
November 2006 | Sleep enhancing medication prescribed‡ Unfocused hand gestures†Continued difficulty with gait†|
January 2007 | Akathisia -inner restlessness†Places arms and legs in sustained postures†Bizarre behavior†|
February 2007 | Bed-bound†Unable to ambulate†MRI - supratentorial parenchymal atrophy with no other acute intracranial findings†|
March 2007 | Cerebrospinal Fluid (CSF) 14-3-3 testing performed - result is not elevated†Awake most of the time‡ |
June 2007 | Death: Age 33 Duration of illness: 22 months Autopsied tissue sent to National Prion Disease Pathology Surveillance Center |
August 2007 | Western blot revealed presence of abnormal protease resistant prion protein Immunohistochemical analysis revealed granular deposits as seen in prion disease MM2 sCJD, thalamic type consistent with "sFI" |