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" |