The natural history of the disease has been investigated. Spinal muscular atrophy sma is characterized by muscle weakness and atrophy resulting from progressive degeneration and irreversible loss of the anterior horn cells in the spinal cord i. Request pdf spinal atrophy type i werdnighoffmann syndrome. Rapid motor neuron death causes inefficiency of the major bodily organs especially of the respiratory system and pneumoniainduced respiratory failure is the most frequent. The onset of weakness ranges from before birth to adulthood. The patient was in the pediatric intensive care unit of a medical center. Sma type i, the most severe form werdnig hoffmann disease can be detected in utero or during the. Baixe no formato ppt, pdf, txt ou leia online no scribd. It is an autosomal recessive hereditary condition, with no treatment, progressive in nature and usually.