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Table 1 Progression of clinical signs and symptoms

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"

  1. † Information obtained from medical record
  2. ‡ Information obtained from family member
  3. Disclaimer: The opinions expressed by authors contributing to this journal do not necessarily reflect the opinions of the Centers for Disease Control and Prevention or the institutions with which the authors are affiliated.