fatal familial insomnia

fatal familial insomnia
an autosomal dominant prion disease caused by mutation in the prion protein gene (PRNP, locus: 20pter-p12), affecting primarily the ventral and dorsomedial nuclei of the thalamus and characterized by progressive insomnia, hallucinations, stupor, and coma ending in death within 6 months to 3 years of onset; autonomic and motor disturbances are also present.

Medical dictionary. 2011.

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  • Fatal familial insomnia — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 32177 ICD10 = ICD10|A|81|9|a|80 ICD9 = ICD9|046.8 ICDO = OMIM = 600072 MedlinePlus = eMedicineSubj = eMedicineTopic = MeshID = D034062 Fatal familial insomnia (FFI) is a very rare autosomal… …   Wikipedia

  • fatal familial insomnia — an autosomal dominant disorder due to a mutation in the gene for the prion protein (PrP): it is an example of a spongiform encephalopathy. Patients present with intractable progressive insomnia, disturbances of the autonomic nervous system, and… …   Medical dictionary

  • fatal familial insomnia — an autosomal dominant disorder due to a mutation in the gene for the prion protein (PrP): it is an example of a spongiform encephalopathy. Patients present with intractable progressive insomnia, disturbances of the autonomic nervous system, and… …   The new mediacal dictionary

  • Insomnia — This article is about the sleeping disorder. For other uses, see Insomnia (disambiguation). Insomnia Classification and external resources ICD 10 F51.0, G47.0 …   Wikipedia

  • Tödliche familiäre Schlaflosigkeit — Bei der tödlichen familiären Schlaflosigkeit (engl. Fatal Familial Insomnia, kurz FFI), handelt es sich um eine erbliche, sehr seltene und im Verlauf von Monaten bis Jahren stets tödlich endende übertragbare spongiforme Enzephalopathie (TSE). Sie …   Deutsch Wikipedia

  • Familiäre fatale Insomnie — Bei der tödlichen familiären Schlaflosigkeit (engl. Fatal Familial Insomnia, kurz FFI), handelt es sich um eine erbliche, sehr seltene und im Verlauf von Monaten bis Jahren stets tödlich endende übertragbare spongiforme Enzephalopathie (TSE). Sie …   Deutsch Wikipedia

  • Creutzfeldt–Jakob disease — Classification and external resources Tonsil biopsy in variant CJD. Prion Protein immunostaining. ICD 10 A …   Wikipedia

  • Prion — DiseaseDisorder infobox Name = Prion Diseases (TSEs) ICD10 = A81 ICD9 = ICD9|046 A prion (IPAEng|ˈpriːɒn [OED|Prion] Audio|Pronunciation prion.ogg|listen) is thought to be an infectious agent that, according to current scientific consensus, is… …   Wikipedia

  • PRNP — (PRioN Protein (Creutzfeld Jakob disease, Gerstmann Sträussler Scheinker syndrome, fatal familial insomnia)) is a genecite journal | author = Kretzschmar HA, Stowring LE, Westaway D, Stubblebine WH, Prusiner SB, Dearmond SJ | title = Molecular… …   Wikipedia

  • Фатальная семейная бессонница — МКБ 10 A81.981.9 МКБ 9 046.8046.8 OMIM …   Википедия

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