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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1993 Nov;56(11):1207–1212. doi: 10.1136/jnnp.56.11.1207

Delirium associated with Joseph disease.

Y Fukutani 1, K Katsukawa 1, R Matsubara 1, K Kobayashi 1, I Nakamura 1, N Yamaguchi 1
PMCID: PMC489822  PMID: 8229032

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.

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Selected References

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