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Kleinert, R; Cervós-Navarro, J; Kleinert, G; Walter, GF; Steiner, H.
Predominantly cerebral manifestation in Urbach-Wiethe's syndrome (lipoid proteinosis cutis et mucosae): a clinical and pathomorphological study.
CLIN NEUROPATHOL. 1987; 6(1): 43-45. (- Case Report)
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Leading authors Med Uni Graz: Kleinert Gertrude
Co-authors Med Uni Graz: Walter Gerhard
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Abstract:: The neurological and psychiatric manifestations in a case of Urbach-Wiethe's disease are reported. The present case is characterized by the predominance of cerebral symptoms: ataxia, psychomotoric seizures, paranoic hallucinatory psychosis and attacks of rage. At autopsy, deposits of amyloid-like material in the walls of the brain vessels could be observed. This finding, together with the mucocutaneous alterations, confirmed the diagnosis of the rare disease know as "lipoid proteinosis cutis et mucosae".
Find related publications in this database (using NLM MeSH Indexing): Adult -; Brain - pathology; Delirium, Dementia, Amnestic, Cognitive Disorders - diagnosis; Female - diagnosis; Humans - diagnosis; Lipidoses - pathology; Lipoid Proteinosis of Urbach and Wiethe - diagnosis; Syndrome - diagnosis