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- Title
Prion diseases and sleep disorders.
- Authors
ZHAN Shu-qin; GUO Cai-feng; WANG Yu-ping; JIA Jian-ping
- Abstract
Prion diseases (PrD) are a group of encephalopathies with neurodegenerative changes caused by prion protein (PrP) whose characteristic datum is transmissibility. In most cases they occur in a sporadic form although a group of them are familial associated with mutations in PrP gene. Phenotypic variability of fatal familial insomnia (FFI) versus familial Creutzfeldt - Jakob disease178 (fCJD178) seems to determine the different methionine-valine polymorphism at codon 129 of the PrP gene. Sleep disorders is one of the important clinical features for the diagnosis and definition of PrD. FFI, a hereditary disorder characterized by loss of physiological sleep with oneiric stupor, autonomic and motor hyperactivity. The polysomnography (PSG) shows disappearance of the physiological pattern of non - rapid eye movement (NREM) and rapid eye movement (REM) sleep, as well as sleep spindles and K-complexes were absent. The hypothesis of the origin of these disorders is thalamic neuronal loss, especially in the anterior and dorsomedial nuclei, described in the neuropathology of these patients; besides, PET reveals hypofunction of thalamic nuclei, centres responsible for controlling wake-sleep. In CJD the wake-sleep disorders is not considered characteristic; nonetheless, frequent alterations have been found in the electroencephalographic registers of sleep. Besides thalamic neurodegeneration, there could be common etiopathogenic mechanisms in PrD in relation to the biological function of PrP.
- Subjects
CREUTZFELDT-Jakob disease diagnosis; PRION disease diagnosis; ACADEMIC medical centers; GENETIC disorders; GENETIC mutation; NEUROSURGERY; NEUROLOGY; SLEEP; SLEEP disorders; PHENOTYPES
- Publication
Chinese Journal of Contemporary Neurology & Neurosurgery, 2013, Vol 13, Issue 6, p471
- ISSN
1672-6731
- Publication type
Article
- DOI
10.3969/j.issn.1672-6731.2013.06.002