クロイッフェルトヤコブ病と脳波活性 : BSEと食の安全性
スポンサーリンク
概要
- 論文の詳細を見る
Jakob in 1921 originally described Creutzfeldt-Jokob disease(CJD) as a progressive illness. It is one of the prion diseases, which include Gerstmann-Scheinker disease, Kuru and fatal familial insomnia. Prion diseases are, for the large part, transmissible and are characterized by celebral deposition of an abnormal protease-resistant isoform of a membrane-bound glycoprotein called prion protein(PrP). Gerstmann-Straussler Scheinker disease and fatal familial insomnia are familial diseases linked to a mutation in the PrP gene; Kuru is acquired only by transmission; and CJD includes sporadic, familial and iatrogenically transmitted form. The sporadic form is the most frequent, accounting for approximately 85% of ill human prion diseases. Sporadic CJD is typically characterized by rapidly progressive dementia, myoclonus, periodic sharp-wave electroencephalographic(EEG) activity, and wide-spread spongiform degeneration.