Delirium associated with Joseph disease.

Abstract

Three Japanese patients with Joseph disease from different families developed sleep disturbance, followed by delirium at the middle to end stage. Brain CT scans of the three patients showed brainstem tegmental atrophy. EEG revealed slowing of background activity. Two necropsy cases showed degeneration of the reticular formation, raphe nuclei and locus ceruleus in the brainstem tegmentum in addition to the common pathological findings of Joseph disease. The clinicopathological correlation between the delirium and the brainstem tegmental atrophy in Joseph disease is discussed.

REM behavior disorder and excessive daytime somnolence in Machado–Joseph disease (SCA‐3)

“…Cognitive decline is not a feature of typical MJD, however 9. In another interesting case, the offspring of a consanguineous marriage, with psychotic behavior and dementia, had possible MJD with possible RBD 10. His CAG repeat numbers were 60 & 60, which is higher than normal but smaller than required for a genetic diagnosis of MJD.…”

Homozygous Machado–Joseph disease presenting as REM sleep behaviour disorder and prominent psychiatric symptoms

“…A few investigators, mainly from Japan, have reported that prominent psychiatric symptoms, as well as sleep disturbance, are associated with this condition. We found 11 such patients (including ours) from the literature (Ishino et al ., 1971; Ikeda, 1987; Fukutani et al ., 1993; Kushida et al ., 1995; Inui et al ., 2000; Mizoguchi et al ., 2000; Takama et al ., 2000). MJD was diagnosed clinicopathologically for six and for five by molecular genetics.…”

Presumed rapid eye movement behavior disorder in Machado‐Joseph disease (spinocerebellar ataxia type 3)

“…This report is the first of SCA 3 being associated with RBD‐like symptoms. There are two publications describing peculiar sleep behavior in SCA 39, 10 that does not fit the RBD syndrome. In one case, the patient had episodes lasting several minutes during both non‐REM as well as REM sleep,9 whereas in the other, 3 patients had delirium starting in but persisting after sleep 10.…”

“…There are two publications describing peculiar sleep behavior in SCA 39, 10 that does not fit the RBD syndrome. In one case, the patient had episodes lasting several minutes during both non‐REM as well as REM sleep,9 whereas in the other, 3 patients had delirium starting in but persisting after sleep 10. These 3 unrelated patients all were demented, a problem not reported in other SCA 3 families 10.…”

“…In one case, the patient had episodes lasting several minutes during both non‐REM as well as REM sleep,9 whereas in the other, 3 patients had delirium starting in but persisting after sleep 10. These 3 unrelated patients all were demented, a problem not reported in other SCA 3 families 10. Interestingly, in my small population, the prevalence was extremely high, suggesting that this finding may be a very common, previously overlooked, aspect of this disorder.…”