Table 1 |
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Progression of clinical signs and symptoms |
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Date |
Clinical signs and symptoms |
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August 2005 |
Onset: age 31† Increased attention deficit† Progressive memory loss† Sleep disturbance‡ |
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February 2006 |
Bizarre behavior‡ Sitting in chair making loud incoherent noises‡ |
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April 2006 |
MRI - negative for intracranial abnormalities† |
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June 2006 |
Anisocoria† Increased agitation‡ Incoherent speech‡ Balance and gait difficulties‡ Talking to self† Referred to psychiatry† Decreased attention, registration and calculation† |
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July 2006 |
Electroencephalogram (EEG) - bilateral periodic epileptiform discharges† |
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August 2006 |
Flat affect† Continued decrease in attention, registration and calculation† |
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October 2006 |
Confused† Constant movement† |
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November 2006 |
Sleep enhancing medication prescribed‡ Unfocused hand gestures† Continued difficulty with gait† |
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January 2007 |
Akathisia -inner restlessness† Places arms and legs in sustained postures† Bizarre behavior† |
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February 2007 |
Bed-bound† Unable to ambulate† MRI - supratentorial parenchymal atrophy with no other acute intracranial findings† |
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March 2007 |
Cerebrospinal Fluid (CSF) 14-3-3 testing performed - result is not elevated† Awake most of the time‡ |
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June 2007 |
Death: Age 33 Duration of illness: 22 months Autopsied tissue sent to National Prion Disease Pathology Surveillance Center |
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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" |
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† Information obtained from medical record ‡ Information obtained from family member 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. |
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Moody et al. BMC Neurology 2011 11:136 doi:10.1186/1471-2377-11-136 |
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