PROGRESSIVE SPINAL MUSCULAR ATROPHY CHILDREN DISEASES

(Werdnig Hoffmann Paralysis)

This is a hereditary disease occurring in infants. Wasting of muscles start usually acutely in pelvic girdles and thigh muscles and ultimately spreads to the extremities. Juvenile spinal muscular atrophy due to Hexosaminidase deficiency with abnormal rectal changes and low Hexosaminidase level in blood and leucocyte may be seen.

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