Sleep disturbances in variant of Creutzfeldt-Jakob disease.

摘要

Sleep disorders have been well studied in prion disease, particularly in fatal familial insomnia (FFI) [1], but not in variant Creutzfeldt-Jakob disease (vCJD). We report the case of a 32-year-old French woman with neuropathologically confirmed vCJD who underwent five polysomnographies. She was homozygous for methionine at codon 129 on the prion protein gene. The first polysomnography was performed 8 months after the onset because she complained of insomnia. It showed a dramatic reduction in total sleep time (106 min). Only residual spindles were found. Six months later another polysomnography showed a normal total sleep time (409 min) with only one slow-wave sleep period and a single and short REM sleep period. In the second part of night, sleep became fragmented with increased interspersed wakefulness. One month later polysomnography was performed during two consecutive nights and one day. Total sleep time was reduced (315 and 225 min in nights, 71 min in day). It showed almost lack of sleep spindles, slow-wave sleep and difficulty maintaining sleep. Only short REM sleep periods persisted.