Article
Genetic prion disease: Experience of a rapidly progressive dementia center in the United States and a review of the literature.
American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics - 1 Jan 2017
Takada Leonel T, Kim Mee-Ohk, Cleveland Ross W, Wong Katherine, Forner Sven A, Gala Ignacio Illán, Fong Jamie C, Geschwind Michael D
Abstract excerpt
Although prion diseases are generally thought to present as rapidly progressive dementias with survival of only a few months, the phenotypic spectrum for genetic prion diseases (gPrDs) is much broader. The majority have a rapid decline with short survival, but many patients with gPrDs present as slowly progressive ataxic or parkinsonian disorders with progression over a few to several years. A few very rare...
Topics
- Adult
- Creutzfeldt-Jakob Syndrome
- Dementia
- Female
- Gerstmann-Straussler-Scheinker Disease
- Humans
- Insomnia, Fatal Familial
- Male
- Middle Aged