Curschmann-Batten-Steinert syndrome

[miriam]

I have an adult patient with Steinert syndrome - Curschmann-Batten-Steinert syndrome (myotonic dystrophy).
Which kind of exercise is indicated? Her muscular disorders are mostly in his hands and feet (steppage gait)
I have no experience in this kind of disease!!!

[physiobob]

Originally Posted by miriam

I have an adult patient with Steinert syndrome - Curschmann-Batten-Steinert syndrome (myotonic dystrophy).
Which kind of exercise is indicated? Her muscular disorders are mostly in his hands and feet (steppage gait)
I have no experience in this kind of disease!!!

To get things started he is a bit of info about the diesease and common names for it:

Curschmann-Batten-Steinert syndrome

Also known as:
Batten’s disease
Batten's syndrome
Batten-Gibb syndrome
Batten-Steinert syndrome
Curschmann-Batten syndrome
Curschmann-Steinert syndrome
Curschmann-Steinert-Batten disease
Rossolimo-Curschmann-Batten-Steinert myotinic dystrophy
Rossolimo-Curschmann-Batten-Steinert syndrome
Steinert’s disease
Steinert’s myotonic dystrophy

Associated persons:
Frederick Eustace Batten
Grigorii Ivanovich Rossolimo

Description:
Rare hereditary familial disease characterized by progressive muscular wasting, myotonia, cataracts, hypogonadism, and mental deterioration. The myotonic dystrophy involves both smooth and striated muscle and may present with limb weakness.

There may be gastrointestinal problems such as dysphagia, anosphinctal abnormalities and poor gall bladder contractibility and cholelithiasis as well as anicteric cholestasis. Cardiac involvement usually is due to abnormalities of the Bundle of His and Purkinje but cardiac muscular dystrophy can cause supra ventricular and ventricular arrythmias.

Aspiration pneumonia is a complication. Very long list of symptoms. Appears slightly more frequently in male between 20 and 30 years of age. The syndrome is transmitted as an autosomal dominant trait with complete penetrance and variation in expression.