Chorea in Creutzfeldt-Jakob Disease
Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disease that causes rapidly progressing dementia, myoclonus, ataxia, visual disturbance, extrapyramidal, and pyramidal abnormalities. Approximately 90% of patients display abnormal movements, the most common of which is generalized myoclonus in advanced stages. Several other movement disorders have been described in patients with sporadic, familial, or new variant CJD (vCJD) including dystonia, choreoathetosis, tremor, hemiballismus, and atypical parkinsonian features like those sometimes seen in corticobasal syndromes and progressive supranuclear palsy. The frequency of movement disorders increases with disease duration, but these have also been reported as initial manifestations. Myoclonus develops particularly early in patients with methionine/methionine or methionine/valine at codon 129 of the prion protein gene and with the scrapie variant of prion protein PrPSc type 1.
KeywordsMovement Disorder Prion Protein Progressive Supranuclear Palsy Initial Manifestation Spontaneous Speech
The patient is intubated because of respiratory insufficiency. She exhibits generalized chorea, particularly involving both upper extremities, face, and upper trunk.
Hemichorea-hemiballismus.mp4 (MP4 5,168KB)