1. MYOPATHY Dr.Shamekh M. El-Shamy

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

3. . Clinical Picture:

4. 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. 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.

6. 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.

7. 3- Other rare types: a)Distal type of Gower. b)Ocular type. c) Oculo-pharyngeal type.

8. DuchenneBecker 1- Age of onset1 st decade2 nd decade 2- CourseprogressiveSlowly progressive 3- Skeletal deformities presentabsent 4- E.C.G changesCommonly present absent

9. Gower’s Sign

10. Wasting shoulder girdle Wasting pelvic girdle Pseudohypertrophy of calf muscles

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

12. OTHER CAUSES OF MYOPATHY

13. 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.

14. 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

15. Causes of death in muscle dystrophies I- Paralysis of respiratory muscles. II- Infections specially hypostatic pneumonia. III- Cardiomyopathy in Duchenne type.

16. Myasthenia Myasthenia gravis: It is a disorder of transmission at the neuromuscular junction, manifesting itself clinically by easy fatiguability of the skeletal muscles,

17. AETIOLOGY: - It is an auto-immune disease.

18. CLINICAL PICTURE:

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

21. Thank you