<unk>  <unk> , also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is an invariably fatal degenerative brain disorder. Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. Later symptoms include dementia, involuntary movements, blindness, weakness, and coma. About 70% of people die within a year of diagnosis. The name<unk>  was introduced by Walther Spielmeyer in 1922, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob.<unk>  is caused by a type of abnormal protein known as a prion. Infectious prions are misfolded proteins that can cause normally folded proteins to also become misfolded. About 85% of cases of<unk>  occur for unknown reasons, while about 7.5% of cases are inherited from a person's parents in an autosomal dominant manner. Exposure to brain or spinal tissue from an infected person may also result in spread. There is no evidence that sporadic<unk>  can spread among people via normal contact or blood transfusions, although this is possible in variant<unk> . Diagnosis involves ruling out other potential causes. An electroencephalogram, spinal tap, or magnetic resonance imaging may support the diagnosis. There is no specific treatment for<unk> . Opioids may be used to help with pain, while clonazepam or sodium valproate may help with involuntary movements.<unk>  affects about one per million people per year. Onset is typically around 60 years of age. The condition was first described in 1920. It is classified as a type of transmissible spongiform encephalopathy. Inherited<unk>  accounts for about 10% of prion disease cases. Sporadic<unk>  is different from bovine spongiform encephalopathy (mad cow disease) and variant<unk>  (vCJD).
Creutzfeldt–Jakob disease
Anti-Hu associated encephalitis
Powassan encephalitis
Kuru (disease)  Kuru  1
Saint Louis encephalitis
Variant Creutzfeldt–Jakob disease  Variant Creutzfeldt–Jakob disease  1
Autoimmune encephalitis
Viliuisk encephalomyelitis