Huntington disease-like 1
Synonyms: Early-onset prion disease with prominent psychiatric features | HDL1
A rare genetic human prion disease characterized by adult-onset neurodegenerative manifestations associated with a movement disorder and psychiatric/behavioral disturbances. Patients typically present personality changes aggressiveness manias anxiety and/or depression in conjunction with rapidly progressive cognitive decline (presenting with dysarthria apraxia aphasia and eventually leading to dementia) as well as ataxia (manifesting with gait disturbances unsteadiness coordination problems) Parkinsonism myoclonus and/or chorea. Additional features may include generalized spasticity seizures urine incontinence and pyramidal abnormalities.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Reference: Access aggregated data from Orphanet at Orphadata.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024
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Huntington disease-like 1?
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