A group of anterior horn cell disorders. An estimated 1 in 40 persons carry the SMA gene. Classification relies on age of onset, rate of progression, distribution and mode of inheritance.
Type I - Werdnig Hoffman disease (Acute Infantile SMA) This is an autosomal recessive disorder. Incidence 1:25 000 births
Reduced large fibres in peripheral nerve
„ Grouped or 'neurogenic' ✓ atrophy of either type 1 or type 2 muscle fibres different from other causes of 'floppy' infant.
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