Parkinson-dementia complex of Guam
Synonyms: G-PDC | Guam disease | Guam parkinsonism-dementia complex | Lytico-Bodig disease
A rare neurodegenerative disease characterized by extrapyramidal symptoms (rigidity tremor bradykinesia) and dementia typically beginning in the fifth or sixth decade of life and progressing to a vegetative state with pelvicrural flexion contractures within few years. Oculomotor signs olfactory dysfunction and autonomic disturbances may also be observed. Neuropathological hallmarks are frontotemporally accentuated cerebral atrophy as well as neurofibrillary tangles and neuronal loss in a characteristic distribution in cortical and subcortical regions. The disease is endemic to the Pacific island of Guam.
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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Parkinson-dementia complex of Guam?
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