Variant Creutzfeldt-Jakob disease

This code represents Variant Creutzfeldt-Jakob disease (vCJD), a rare, fatal neurodegenerative disease characterized by spongiform changes in the brain. It is a transmissible prion disease, distinct from sporadic CJD, and is strongly linked to bovine spongiform encephalopathy (BSE) exposure.

Use this code when documentation explicitly states a diagnosis of Variant Creutzfeldt-Jakob disease. This diagnosis is typically made based on clinical presentation (e.g., psychiatric symptoms, sensory disturbances, ataxia, dementia), MRI findings, and neuropathological examination.

• Differentiate carefully from other forms of CJD (e.g., A81.00 for unspecified CJD, A81.09 for other CJD).
• Ensure documentation clearly specifies "variant" CJD to support this specific code.
• Consider sequencing this code as the primary diagnosis when it is the reason for the encounter.