Werdnig hoffmann disease or spinal atrophy type i is part of the spinal muscular atrophies and the most serious of the three clinical forms in existence. Marked hypotonia, sluggish movement and fasciculation of tongue floppy neonate. Sma type i, the most severe form werdnig hoffmann disease can be detected in utero or during the first months of life. Spinal muscular atrophy 1 sma1, also known as werdnig hoffmann disease, is a genetic neuromuscular disorder that affects the nerve cells that control. Sma type i, the most severe form werdnig hoffmann disease can be detected in. Death typically occurs within the first 2 years of life. Werdnighoffmann syndrome johann hoffmann related people. The patient reported by werdnig in 1891 was an infant named wilhelm bauer who developed progressive muscular weakness before dying from respiratory embarrassment when he was 5 years of age. Sma type i, the most severe form werdnighoffmann disease can be detected in. Werdnighoffmann disease or spinal atrophy type i is part of the spinal muscular atrophies and the most serious of the three clinical forms in existence.