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MUSCULAR DYSTROPHIES: It is a group of disorders of primary muscular atrophy of unknown cause. They are best characterized by the lack of any evidence of denervation and yet there is degeneration of the muscle fibres and increases in the content of fat and fibrous tissue in the muscle. They are commonly seen and have features similar to myopathies. The common ones are discussed below:

Duchenne Muscular Dystrophy: It is an X-linked disorder also called pseudohypertrophic muscular dystrophy as the calf muscles appear large and bulky though they are weak. It presents at birth but becomes apparent at 3-5 years of age. Patients present with 'Gowers manoeuvre' i.e. uses his hands to climb up himself. Contractures are common and fixed. Scoliosis develops over a period of time. Muscle weakness is progressive. Cardiac cause of death is uncommon but presents with cardiomyopathy and CCF may occur. Intellectual impairment is common. Investigations show CPK levels increased by 20-100 times of normal. EMG features are similar to those of myopathy. Muscle Biopsy shows groups of necrotic and regenerating fires.

Becker's Muscular Dystrophy: It is also an X-linked muscular disease. Patients present with proximal muscle weakness. They present between 5-15yrs of age. Mental retardation is more common. Average life span is up to 4th-5th decade.

Congenital Muscular Dystrophy: Children present at birth or in the first few months of life with hypotonia and proximal limb weakness. There is varying degrees of contracture seen at birth called arthrogryposis. Death may ensue because of respiratory insufficiency early in life


TYPES OF SURGERIES REQUIRED IN THESE PATIENTS:

These patients usually come for: