Myopathies are a group of diseases of the skeletal muscles characterised by gradual progressive degeneration of these muscles.

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1- Clumsy gait.
2- Inability to climb the stairs.
3- Protuberant abdomen.
4- Weakness and wasting of certain muscles
(shoulder and pelvic girdles and trunk).

5-There is selectivity of the involved muscles.
6- E.C.G. changes, histological changes in the heart and cardiomyopathy may be found specially in Duchennc type.
7-No sensory changes, fasciculations or sphincteric disturbances.

Clinical types of progressive muscular dystrophies
1. Shoulder girdle types:
- Scapulo-humeral type.
- Facio-scapulo-humeral type.
2. Pelvic girdle types: a)-Pseudo-hypertrophic type: ( Duchenne
&Becker).
b)-Atrophic type.

3- Other rare types: a) Distal type of Gower.
b) Ocular type.
c) Oculo-pharyngeal type.

1- Age of onset
2- Course
Duchenne
1 st decade progressive
Becker
2 nd decade
Slowly progressive
3- Skeletal deformities present absent
4- E.C.G changes Commonly present absent

Wasting shoulder girdle
Wasting pelvic girdle
Pseudohypertrophy of calf muscles

GENETICALLY MUSCULAR DYSTROPHIES
1-X-linked pseudohypertrophic types:
2- Autosomal dominant: Facio-scapulo-humeral type.
3- Autosomal recessive: Limb girdle type.

1- Estimation of creatine and creatinine in urine.
2- Estimation of serum enzyme.
3- Creatine tolerance test.
4- E.M.G.
5- Muscle biopsy.

There is no specific treatment;management is supportive:
1- A.T.P. 25 mg daily I.M.
2- Vitamin E as it helps the retention of creatine in the body.
3- Physiotherapy

I- Paralysis of respiratory muscles.
II- Infections specially hypostatic pneumonia.
III- Cardiomyopathy in Duchenne type.

It is a disorder of transmission at the neuromuscular junction, manifesting itself clinically by easy fatiguability of the skeletal muscles,

AETIOLOGY:
- It is an auto-immune disease.

• CLINICAL PICTURE :

• DEFINITION :
Myotonic phenomenon is delayed relaxation of the skeletal muscles after voluntary, mechanical or electrical stimulation.