Objectives  To recognize existence of muscle diseases  To differentiate them from other disorders.  To know about various causes of myopathy.  To know of basic investigations of myopathy.

CLINCIAL PICTURE  Proximal, large muscles.  Steadily progressive, s.t. episodic  Symmetric  Preserved tone and tendon reflexes  No sensory\autonomic abnormality  Cardiac failure  Cardiac conduction abnormalities  Respiratory problems (sleep apnea)

Differential Diagnosis  Lower motor neuron disorders  Neuropathy (reflexes, distal, Sensory, autonomic)  Neuromuscular (bizarre, asymmetric, variable)  Anterior horn cell (reflexes)

Outline of investigations  Creatinine phosphokinase (CK)  EMG-NCS  Muscke Biopsy (histo-biochemistry)  ECG  CXR  Underlying cause:  Genetic\metabolic screen and counseling  Inflammatory/Endocrine/Electrolytes etc..

Causes  Hereditary  Dystrophies  Congenital  Inherited metabolic  Channelopathies

Duchenne Muscular dystrophy

Muscular Dystrophies

Fascio-Scapulo-Humoral Dystrophy

Periodic Paralysis:  Hypokalemic :  Na, Ca channel  After heavy carbohydrate meals, salt  Treament:  Low sugar\salt diet, rich K diet  Acetazolamide  Hyperkalemic :  Na channel  After K rich meals  Treatment: low K diet

Acquired causes: