Tag Archives: sleepiness

Interventions helps 30% of myotonic dystrophy patients with sleep issues

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Sleepiness and Sleep-related Breathing Disorders in Myotonic Dystrophy and Responses to Treatment: A Prospective Cohort Study

Article type: Research Article

Authors: West, Sophie D.a; * | Lochmüller, Hannsb | Hughes, Joana | Atalaia, Antoniob | Marini-Bettolo, Chiarab | Baudouin, Simon V.a | Anderson, Kirstie N.a

Affiliations: [a] Newcastle Regional Sleep Service, Newcastle upon Tyne Hospitals NHS Trust, NE7 7DN, UK | [b] Institute of Genetic Medicine, The John Walton Muscular Dystrophy Research Centre, MRC Centre for Neuromuscular Diseases, Newcastle University, Newcastle upon Tyne, NE1 3BZ, UK

Correspondence: [*] Correspondence to: Sophie D. West, Newcastle Regional Sleep Service, Newcastle upon Tyne Hospitals NHS Trust, NE7 7DN, UK. Tel.: +44 191 233 6161; Fax: +44 191 2137397; E-mail: sophie.west@nuth.nhs.uk.

Abstract: Objective: We conducted prospective assessments in people with myotonic dystrophy type 1 (DM1) with daytime sleepiness, provided targeted therapies and assessed response. Methods: Patients had overnight sleep assessments. Treatment with continuous positive airway pressure (CPAP) for OSA, non-invasive ventilation (NIV) for respiratory failure, modafinil for excessive daytime sleepiness were commenced. Results: 120 people were studied: mean age 46.9 years (SD 13.2, range 18–74), body mass index 27.9 kg/m2 (7.2, 16–53), Epworth Sleepiness Score (ESS) 13.1 (4.7, 2–24). Twenty one people (18% of group) had OSA: mean age 49.6, BMI 31.1, ESS 14.3, ODI 22, pO2 11.3, pCO2 5.4. All were offered CPAP; seven continued with benefit but 14 had intolerance or no benefit. Thirty-three people (27%) had respiratory failure and abnormal sleep study: mean age 51.5, BMI 31.3, ESS 13.9, ODI 22.9, pO2 8.7, pCO2 6.8. All were offered NIV; 12 continued with benefit but 18 had intolerance or no benefit, 1 died and 2 declined commencement. Thirty-six people (30%) had predominantly sleepiness: mean age 44.8, BMI 24.6, ESS 14.1, ODI 9.2, pO2 11.7, pCO2 5.4. All were offered modafinil; 12 continued this with benefit but 10 had intolerance or no benefit, one was unkeen to start, 11 did not attend further clinic and two had other sleep disorders. Comparing means of treatment responders to non-responders showed no significant difference in any variable, except ESS: 15.9 vs.11.9 respectively, p < 0.0001. Conclusions: Causes of sleepiness are variable in DM1, but include obstructive sleep apnoea, respiratory failure and sleepiness with a normal sleep study; 29% of this studied cohort benefited from targeted sleep therapies.

Keywords: Assisted ventilation, respiratory muscles, respiratory monitoring, sleep apnoea

DOI: 10.3233/JND-160191

Journal: Journal of Neuromuscular Diseases, vol. 3, no. 4, pp. 529-537, 2016

Published: 29 November 2016

New Fatigue Scale available for Clinical Use

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There has always been some questions in my mind about the common tests for fatigue and sleepiness with myotonic dystrophy. A new study from the Netherlands confirms this. They have developed a new modified scale that works much better for myotonic dystrophy patients.

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Provigil Survey Finds Strong Support From Myotonic Dystrophy Patients

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For quite a few years, I have been strongly advocating the use of Provigil the brand name for modafinil for helping with sleep related issues n Myotonic Dystrophy. Many patients with myotonic dystrophy have tremendous issues with excessive sleep and sleep disruptions.  A study from France in 2009 concluded that a controlled study showed “no significant effects on daytime sleepiness” This study puzzled me as many patients reported very good results from this drug.

A survey conducted by the Myotonic Dystrophy Support Group,  MDSG in England has concluded that over 80% of survey respondents found that the drug had a marked benefit or a dramatic benefit. This concurs with the informal feedback that I have had here in the USA. This also focuses attention on patient centered approaches versus medical centered approaches. Patient centered approaches are very important to give direct patient feedback rather than just visibility to medical professionals opinions based on limited patient facts.

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Daytime Sleepiness in Myotonic Dystrophy Causes

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This is a study (1994)  that looked at the causes of sleepiness in patients with myotonic dystrophy. They concluded that these sleep issues are not caused by sleep apnea. A number of the patients were able to get some relief with Ritalin. Ritalin has a low amount of cardiac side effects. However, this drug may have some cardiac side effects so clear it with your doctor cardiologist before use

Daytime Sleepiness is not caused by Sleep Apnea

J Neurol Neurosurg Psychiatry. 1994 May;57(5):626-8.

Daytime sleep in myotonic dystrophy is not caused by sleep apnoea.

Source

Department of Neurology, University Hospital Rotterdam-Dijkzigt, The Netherlands.

Abstract

Daytime sleepiness is common in myotonic dystrophy and might be attributed to disturbed nocturnal breathing. Seventeen out of 22 patients complained of excessive daytime sleepiness, resembling “idiopathic hypersomnolence”. Sleep apnoea might have contributed to daytime sleepiness in only three of 17 patients. Treatment with the central stimulant methylphenidate produced sustained benefit in seven out of 11 patients tested. Daytime sleepiness in myotonic dystrophy is usually caused by dysfunction of central sleep regulation and not by disturbed nocturnal breathing.

Ritalin and Daytime Sleepiness in Myotonic Dystrophy

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Sleepiness is a  issue with some adults and children with Myotonic Dystrophy. Sleepiness or Hypersomnia can have great impact on the social life of patients with myotonic dystrophy. Some patients have reported relief with Provigil although studies have indicated that there is no overall benefit. Provigil may work to help mobilize the sleep patterns so that a person can attend an event but then catch up on sleep later. A new study is looking at a different drug.

This study is by one of the worlds experts in the Disease (DM1) Dr. Puymirat in Quebec, Canada. This article  studies Dr. J PuyrimatRitalin (20mg) and Myotonic dystrophy related to sleepiness with some good results. However, it is noted that palpitations are a side effect of the study. The risks with Ritalin are low but make sure to see a cardiologist before using or having Ritalin prescribed.

Clin Ther. 2012 May;34(5):1103-11.