Cytoplasmic inclusion bodies within neurons of the thalamus in myotonic dystrophy: A light and electron microscope study☆
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Future directions in hypersomnias: diagnosis and treatment
2023, Encyclopedia of Sleep and Circadian Rhythms: Volume 1-6, Second EditionThe actigraphic documentation of circadian sleep-wake rhythm dysregulation in myotonic dystrophy type 1
2021, Sleep MedicineCitation Excerpt :All these findings confirmed and extended the previously shown degeneration of crucial brain areas involved in circadian regulation of different neuroendocrine and biological patterns present in DM1 patients. Although neuropathological studies documented degeneration of the thalamus, brainstem and hypothalamic areas crucial for the regulation of the sleep-wake cycle in patients with DM1 [12–14], only case reports documented the impairment of the circadian sleep-wake rhythm in patients with DM1 [22]. Hence, the dysregulation of the sleep-wake cycle in DM1 patients objectively and subjectively shown by this study can be explained by the neuronal loss of the dorsal raphe nucleus and the superior central nucleus, serotoninergic brainstem areas, already related to slow-wave sleep deterioration [14].
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2016, Sleep Medicine ClinicsCitation Excerpt :Nocturnal polysomnography in 11 patients with myotonic dystrophy uncovered alveolar hypoventilation in all patients, along with mild sleep disordered breathing of a central type in stage 1 and REM sleep.41 Patients with myotonic dystrophy may also have intrinsic hypersomnia related to degeneration of nerve cells in dorsomedial nuclei of the thalamus and perhaps hypothalamus manifested by cytoplasmic eosinophilic inclusion bodies.42 Laberge and colleagues,43 in their series of patients with DM1, found characteristics similar to those encountered in patients with idiopathic hypersomnia.
Neuromuscular Disorders, Sleep and
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This work was supported in part by NIH Grant NS-5126 and the Muscular Dystrophy Association of America, Massachusetts Chapter Fund to the Department of Neurology, Boston University.