Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare, neurodegenerative disease characterized by progressive cataracts, hearing loss, cerebellar ataxia, paranoid psychosis and dementia. Neuropathological features are diffuse atrophy of all parts of the brain, chronic diffuse encephalopathy and the presence of extremely thin and almost completely demyelinated cranial nerves. Disease data Classification Clinical subtype Synonyms Familial dementia, Danish type Rodzinna demencja, typ duński ORPHA code 97346 OMIM code 117300 ICD10 code I68.0* ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl