Werdnig-Hoffmann disease

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A hereditary disorder – a severe form of spinal muscular atrophy – in which the cells of the spinal cord begin to die between birth and the age of six months, causing a symmetrical muscle weakness. Affected infants become floppy and progressively weaker; respiratory and facial muscles become affected. Children usually die by the age of 20 months from respiratory failure and there is no treatment. Genetic counselling is required for parents of an affected child as each of their subsequent children has a one in four chance of being affected. G. Werdnig (1844–1919),Austrian neurologist; J. Hoffmann (1857–1919), German neurologist

Subjects: Medicine and Health.

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