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MYOPATHY
Dr.Shamekh M. El-Shamy
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• Definition: Myopathies are a group of
diseases of the skeletal muscles characterised by gradual progressive degeneration of these muscles.
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.Clinical Picture:
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• SYMPTOMS: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).
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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.
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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.
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3- Other rare types:
a)Distal type of Gower.
b)Ocular type.
c) Oculo-pharyngeal type.
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Duchenne Becker
1 -Age of onset 1st decade 2nd decade
2 -Course progressive Slowly progressive
3 -Skeletal deformities
present absent
4 -E.C.G changes Commonly present
absent
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Gower’s Sign
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Wasting shoulder girdle
Wasting pelvic girdle
Pseudohypertrophy of calf muscles
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GENETICALLY MUSCULAR DYSTROPHIES
1-X-linked pseudohypertrophic types:
2- Autosomal dominant: Facio-scapulo-humeral type.
3- Autosomal recessive: Limb girdle type.
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OTHER CAUSES OF MYOPATHY
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INVESTIGATIONS
1- Estimation of creatine and creatinine in urine.
2- Estimation of serum enzyme.
3- Creatine tolerance test.
4- E.M.G.
5- Muscle biopsy.
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TREATMENT
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
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Causes of death in muscle dystrophies
I- Paralysis of respiratory muscles.
II- Infections specially hypostatic pneumonia.
III- Cardiomyopathy in Duchenne type.
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Myasthenia
Myasthenia gravis: It is a disorder of transmission at
the neuromuscular junction, manifesting itself clinically by easy fatiguability of the skeletal muscles,
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AETIOLOGY:
- It is an auto-immune disease.
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• CLINICAL PICTURE:
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Myotonia
• DEFINITION:
Myotonic phenomenon is delayed relaxation of the skeletal muscles after voluntary, mechanical or electrical stimulation.
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Thank you