Provigil Survey Finds Strong Support From Myotonic Dystrophy Patients

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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Skin problems

Patient with DM have complained about Skin Problems. Here we will try to answer information about Skin problems and provide more information.

A Case of Myotonic Dystrophy Complicated with
Raynaud’s Phenomenon

S. Fujimaki, A. Kurihara, K. Tamura and Z. Shiozawa

We reported a case of myotonic dystrophy complicated with Raynaud’s phenomenon. The patient was a 66-year-old male, who was admitted to our hospital with complaints of muscle atrophy, general fatigue and coldness of the hands. He noted muscle power loss about 20-years ago and later developed felt urinary incontinence, constipation, dysphagia, hoarseness and muscle atrophy. The diagnosis of myotonic dystrophy was confirmed by EMG and the biopsy of M.biceps brachii. By the cold stress test and blood analysis, he was diagnosed as Raynaud’s disease. The findings indicate that Raynaud’s phenomenon is included in the clinical feature of myotonic dystrophy. This phenomenon was the rare manifestation of smooth muscle involvement by myotonic dystrophy.
Tissue or System Affected

Symptoms

Eye
-cataracts
-changes in retinal pigmentation

Endocrine system
-testicular atrophy leading to reduced fertility
-insulin resistance
-pituitary dysfunction
-hyperparathyroidism

Skin
-frontal balding
-pilomatrixoma/ calcifying epithelioma

Cardiovascular system
-cardiac conduction defects
-systemic hypotension

Respiratory
-respiratory insufficiency largely due to respiratory muscle weakness

Alimentary canal
-feeding and swallowing difficulties
-gastrointestinal pseudobstruction
-general gut motility problems

Nervous system
-mental retardation and cognitive impairment
-sensorineural deafness

Immune system
-reduced levels immunoglobulins and some complement components

 

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