sporadic fatal insomnia is a relatively recently described prion disease with only a limited number of cases reported in the published literature to date. we describe a unique autopsy-proven case in a woman who clinically had progressive cerebellar deterioration of approximately 18 months' duration. ...
Familial fatal insomnia (FFI) (in member of a family with PRNP178 mutation): thalamic degeneration, variable spongiform change in cerebrum. Kuru (in the... H Budka,A Aguzzi,P Brown,... - 《Brain Pathology》 被引量: 1221发表: 1995年 Creutzfeldt-Jakob Disease: II. Clinical, Pathologic, ...
Presents a case study of a 44-year-old man with typical symptoms of fatal familial insomnia. Loss of cough reflex; Ataxia; Deterioration of short-term memo... James,A.,Mastrianni,... - 《New England Journal of Medicine》 被引量: 388发表: 1999年 Sporadic fatal insomnia in a young woman...
They cause lethal sporadic, familial and infectious neurodegenerative conditions in man, including Creutzfeldt-Jakob disease (CJD), Gerstmann-Str¤ussler-Scheinker syndrome (GSS), kuru, sporadic fatal insomnia (SFI) and likely variable protease-sensitive prionopathy (VPSPr). The most prevalent of ...
Inflammation of lymphatic vessels, usually caused by virulent organisms, oftenSTREPTOCOCCI(seeSTREPTOCOCCUS). Lymphangitis causes conspicuous red streaks under the skin with fever and general upset. The condition indicates a potentially dangerous infection and calls for urgent antibiotic treatment. ...
Amyotrophic lateral sclerosis (ALS) is a fatal disorder caused by the progressive degeneration of motoneurons in brain and spinal cord. Despite identification of disease-linked mutations, the diversity of processes involved and the ambiguity of their rel
Sporadic fatal insomnia: A case study. Ann Neurol 2000;48:665Y68Scaravilli F, Cordery RJ, Kretzschmar H, Gambetti P, Brink B, Fritz V et al (2000) Sporadic fatal insomnia: a case study. Ann Neurol 48:665-668.Scaravilli F;Cordery RJ;Kretzschmar H;.Spo radic fatal insomnia:a case ...
Journal of Neuropathology and Experimental NeurologyParchi P, Capellari S, Chin S, Powers JM, Schwarz HB, Burns DK et al. Fatal sporadic insomnia (thalamic form of sporadic Creutzfeldt-Jakob Disease). J Neurooathol Exu Neuiol 1998; 57 : 518....
Both fatal familial insomnia and sporadic fatal insomnia are associated with a protease-resistant prion protein with the electrophoretic mobility of the protease-resistant prion protein type 2. Currently there is no effective treatment for fatal familial insomnia and sporadic fatal insomnia. Modeling of ...
BackgroundSporadic fatal insomnia (sFI) and fatal familial insomnia (FFI) are rare human prion diseases.MethodsWe describe a case of a 33 year old female who died of a prion disease for whom the diagnosis of sFI or FFI was not considered clinically.ResultsAn interview with a close family ...