Abstract
Abundant inclusion bodies were found within the cytoplasm of thalamic neurons in 6 patients with myotonic dystrophy, 4 of whom had mental defect. Electron microscopy studies revealed that these bodies contain a proteinacious fibrillar material surrounded by a ribosome-bearing membrane and result from coalescence of dilated cisternae of rough endoplasmic reticulum. Cytoplasmic bodies represent a structural alteration of thalamic neurons very likely related to progressive dementia, behavioral changes, hypersomnia and slow dominant posterior EEG rhythms which are manifestations of CNS dysfunction frequently encountered in patients with myotonic dystrophy.
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