Hypersomnia in Dystrophia Myotonica

Phemister, Jennifer; Small, J

doi:10.1136/jnnp.24.2.173

Cited by 46 publications

(23 citation statements)

References 1 publication

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“…The presence of hypersomnia in myotonic dystrophy may also be explained by the theory that the condition is primary and of central origin (Phemister and Small, 1961;Kilburn et al 1959b) or that it is caused by a reduction in the proprioceptive impulses originating in the respiratory muscles. These impulses have a stimulatory effect on alertness as well as on the respiratory centre (Kilburn et al, 1959a;Gillam et al, 1964).…”

Section: Discussionmentioning

confidence: 99%

Alveolar hypoventilation and hyperosmnia in myotonic dystrophy.

Coccagna¹,

Mantovani²,

Parchi³

et al. 1975

Journal of Neurology, Neurosurgery & Psychiatry

101

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SYNOPSIS A case of myotonic dystrophy accompanied by alveolar hypoventilation and hypersomnia is presented. Radiological studies and EMG examination of the intercostal muscles demonstrated that the respiratory muscles were affected by the disease, while polygraphic recordings showed that the alveolar hypoventilation and pulmonary hypertension worsened during sleep. The hypersomnia preceded the appearance of clinical signs of the muscular disease by many years and persisted even after treatment when the blood gas analysis values were greatly improved. During both diurnal and nocturnal sleep, the patient frequently fell asleep directly into a REM stage. The possibility is discussed that, concomitant with the respiratory musculature involvement, there is an alteration in the central nervous system in myotonic dystrophy which is at least partially responsible for both the alveolar hypoventilation and the hypersomnia.

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Section: Discussionmentioning

confidence: 99%

Alveolar hypoventilation and hyperosmnia in myotonic dystrophy.

Coccagna¹,

Mantovani²,

Parchi³

et al. 1975

Journal of Neurology, Neurosurgery & Psychiatry

101

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“…The DM1 EDS seems unrelated to the duration and the quality of nighttime sleep. Sleepiness is believed to occur when attention is not being held, or in monotonous situations, which contrasts with narcolepsy in which daytime sleep occurs in an episodic fashion and tends to arise during activity [47][48][49].…”

Section: Sleepiness In Dm1mentioning

confidence: 99%

Report of the second Outcome Measures in Myotonic Dystrophy type 1 (OMMYD-2) international workshop San Sebastian, Spain, October 16, 2013

Gagnon

Meola

Hébert

et al. 2015

Neuromuscular Disorders

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“…Somnolence has long been associated with DM, the first published reference to this being in 1916 by Rohrer [6], who noted apathy, excessive somnolence and lack of motivation. In 1961 Phemister and Small [7] reported four cases of hypersomnia in DM although one case was complicated by bronchiectasis and another by coarctation of the aorta. However, the ptosis, myopathic facies and dysarthric speech noted in DM can give a false impression of somnolence, and therefore the true extent of the problem could be overestimated.…”

Section: Introductionmentioning

confidence: 98%

Daytime somnolence in myotonic dystrophy

Phillips

Steer

et al. 1999

Journal of Neurology

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Somnolence in myotonic dystrophy (DM) has not been measured using a reliable daytime somnolence scale. The aim of this study was to compare somnolence in DM patients with healthy controls and Charcot-Marie-Tooth disease (CMT) patients using such a scale and to compare this with potential contributory factors. We investigated 35 subjects with adult-onset DM, 16 healthy controls and 13 CMT controls. The Epworth Sleepiness Scale (ESS) was the principal measurement of daytime somnolence. Nocturnal sleep was assessed using a sleep diary. Other assessments measured daytime respiratory function, cognitive function, motor impairment, disability, swallowing capacity and depression. DM and CMT patients had greater daytime sleepiness than unaffected controls. In the DM group significant correlations were found between somnolence and measures of disability, sleep quality and some measures of depression. It was concluded that there is an abnormal level of daytime somnolence in DM, which is partially associated with disability.

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Hypersomnia in Dystrophia Myotonica

Abstract: We have been interested to note the occurrence of marked hypersomnia and somnolence in four patients suffering from dystrophia myotonica. Somnolence has been described before in this Protected by copyright.

Cited by 46 publications

References 1 publication

Alveolar hypoventilation and hyperosmnia in myotonic dystrophy.

Alveolar hypoventilation and hyperosmnia in myotonic dystrophy.

Report of the second Outcome Measures in Myotonic Dystrophy type 1 (OMMYD-2) international workshop San Sebastian, Spain, October 16, 2013

Daytime somnolence in myotonic dystrophy

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