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Chorea as a presenting feature of variant Creutzfeldt-Jakob disease
1Department of Neurology, Greater Manchester Centre for Clinical Neurosciences, Manchester, United Kingdom. david.mckee@srht.nhs.uk
Insights
Variant Creutzfeldt-Jakob disease (vCJD) can present with chorea, a movement disorder. This case highlights an atypical presentation without the usual psychiatric or sensory symptoms.
Area of Science:
- Neurology
- Neurodegenerative Diseases
- Prion Diseases
Background:
- Variant Creutzfeldt-Jakob disease (vCJD) is a fatal prion disease.
- Typical vCJD presentations involve psychiatric disturbances and sensory symptoms preceding neurological deficits.
Observation:
- A patient with pathologically confirmed vCJD presented with chorea as the initial symptom.
- This presentation lacked the characteristic prodromal psychiatric or sensory manifestations.
Findings:
- Chorea can be a presenting feature of variant Creutzfeldt-Jakob disease.
- The absence of typical prodromal symptoms suggests a broader spectrum of vCJD clinical presentations.
Implications:
- This case expands the understanding of vCJD's clinical variability.
- It underscores the importance of considering vCJD in patients with unexplained chorea, even without typical prodromal symptoms.
Abstract:
We report on a patient with pathologically proven variant Creutzfeld-Jakob disease in whom chorea was a presenting feature of the disease, and was unaccompanied by the typical prodrome of psychiatric disturbance or sensory symptoms.