Kuru disease
| Kuru | |
|---|---|
| Classification and external resources | |
| Specialty | Neurology |
| ICD-10 | A81.8 |
| ICD-9-CM | 046.0 |
| OMIM | 245300 |
| DiseasesDB | 31861 |
| MedlinePlus | 001379 |
| MeSH | D007729 |
Kuru is a very rare, incurable neurodegenerative disorder which was prevalent among the Fore people of Papua New Guinea in the 1950s and 60s. Kuru is caused by the transmission of abnormally folded prion proteins, which leads to symptoms such as tremors, loss of coordination, and neurodegeneration.
The term kuru derives from the Fore word kuria or guria ("to shake"), due to the body tremors that are a classic symptom of the disease and kúru itself means "trembling". It is now widely accepted that kuru was transmitted among members of the Fore tribe of Papua New Guinea via funerary cannibalism. Deceased family members were traditionally cooked and eaten, which was thought to help free the spirit of the dead. Females and children usually consumed the brain, the organ in which infectious prions were most concentrated, thus allowing for transmission of kuru. The disease was therefore more prevalent among women and children.
The disease has lingered due to kuru’s long incubation period of anywhere from 10 to over 50 years. The epidemic has declined, however, from 200 deaths per year in 1957 to 1 or no deaths annually in 2005.
Kuru, a transmissible spongiform encephalopathy, is a disease of the nervous system that causes physiological and neurological effects which ultimately lead to death. It is characterized by progressive cerebellar ataxia, or loss of coordination and control over muscle movements.
The preclinical or asymptomatic phase, also called the incubation period, averages between 10–13 years, but can be as short as 5 and has been estimated to last as long as 50 years or more after initial exposure. The youngest individual recorded to have kuru was 12 years old.
The clinical stage, which begins at the first onset of symptoms, lasts an average of 12 months. The clinical progression of kuru is divided into three specific stages, the ambulant, sedentary and terminal stages. While there is some variation in these stages between individuals, they are highly conserved among the affected population. Before the onset of clinical symptoms, an individual can also present with prodromal symptoms including headache and joint pain in the legs.
...
Wikipedia