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November 1979

Congenital Hypotonia due to Myosin Degeneration

Author Affiliations

Child Neurology Unit Department of Pediatrics
Department of Pathology Hadassah University Hospital Jerusalem, Israel

Arch Neurol. 1979;36(11):727. doi:10.1001/archneur.1979.00500470097026

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To the Editor.—  In the February 1977 Archives (34:114-115, 1977), we described an infant who had severe generalized congenital hypotonia, in whom selective myosin degeneration was found in a muscle biopsy specimen. During the following year, we reexamined the girl and performed another biopsy on her contralateral quadriceps femoris muscle. We would like to report here the benign course of this disorder.During the second year of life, the infant showed great improvement in motor performance. At 1 year of age, she was able to stand against the wall and walk with assistance. At 15 months of age, she learned to roll over and to sit up. At 18 months of age, she was able to get up from the floor and walk unassisted. At 27 months of age, she still had a positive Gowers' maneuver and tired easily. The results of a Bayley development test showed a two-month delay

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