CLINICAL DESCRIPTION

Creutzfeldt-Jacob (CJD) disease is a progressive neurological disorder caused by an "unconventional" filterable agent. It is characterized by confusion, progressive dementia and variable ataxia. Later myoclonic jerks appear, together with a variable spectrum of other neurological signs. CJD must be differentiated from other forms of dementia: Alzheimer disease, other slow infections, toxic and metabolic encephalopathies, and occasionally from tumors or other space occupying lesions. The new form of CJD (vCJD) related in UK to consumption of beef differs from CJD in three ways. The vCJD occurs in a younger age group (20-30 years of age vs. 35 years or older for CJD). The characteristic EEG changes seen in CJD are absent in vCJD. Finally the clinical course of vCJD is much longer than CJD (12-15 months vs. 3-6 months).

CASE CLASSIFICATION

1. CONFIRMED
   
   A clinically compatible case supported by:
   • neuropathological examination (brain biopsy, post-mortem examination),
     

2. PROBABLE

   A clinically compatible case supported by:

   • Characteristic EEG changes (CJD), AND/OR
   • Detection of 14-3-3 protein in spinal fluid (CJD), OR
   • Characteristic posterior thalamic high signal on MRI scan (vCJD).

   
   

3. POSSIBLE
   
   A clinically compatible case not supported by EEG, or other laboratory findings.

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