MYOPATHY

Dr.Shamekh M. El-Shamy

• Definition: Myopathies are a group of

diseases of the skeletal muscles characterised by gradual progressive degeneration of these muscles.

.Clinical Picture:

• 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).

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.

 

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

Gower’s Sign

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.

OTHER CAUSES OF MYOPATHY

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.

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

Causes of death in muscle dystrophies

I- Paralysis of respiratory muscles.

II- Infections specially hypostatic pneumonia.

III- Cardiomyopathy in Duchenne type.

Myasthenia

Myasthenia gravis: 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:

Myotonia

• DEFINITION:

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

Thank you