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Hereditary Stiff-Baby Syndrome

Hereditary Stiff-Baby Syndrome Abstract • Clinical and electrophysiologic features of an infant with stiff-baby syndrome were studied; his father, uncle, and paternal grandmother suffered from a similar disorder. They all had a similar facial appearance during infancy (an alert, frightened, tense look) and a tendency to vomit because of hiatal hernias. The condition is benign but causes anxiety and, later, embarrassment as affected adults suddenly fall when stumbling or startled. We suggest that the condition be called hereditary stiff-baby syndrome. The family history is suggestive of autosomal dominant inheritance. (Am J Dis Child 1981;135:909-911) References 1. Klein R, Haddow JE, De Luca C: Familial congenital disorder resembling stiff-man syndrome . Am J Dis Child 1972;124:730-731. 2. Stevens H: ' Jumping Frenchmen of Maine .' Arch Neurol 1965;12:311-314.Crossref 3. Moersch FB, Woltman HW: Progressive fluctuating muscular rigidity and spasms (stiffman syndrome) . Proc Staff Meet Mayo Clin 1956;31:421. 4. Gordon EE, Januszko DM, Kaufman L: A critical survey of stiff-man syndrome . Am J Med 1967;42;582-599.Crossref 5. Andermann F, Keene D, Andermann E, et al: Startle disease or hyperekplexia . Brain 1980;103:985-997.Crossref 6. Beard GM: Remarks on 'Jumpers or Jumping Frenchmen.' J Nerv Ment Dis 1878;5:526. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1981 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1981.02130340021008
Publisher site
See Article on Publisher Site

Abstract

Abstract • Clinical and electrophysiologic features of an infant with stiff-baby syndrome were studied; his father, uncle, and paternal grandmother suffered from a similar disorder. They all had a similar facial appearance during infancy (an alert, frightened, tense look) and a tendency to vomit because of hiatal hernias. The condition is benign but causes anxiety and, later, embarrassment as affected adults suddenly fall when stumbling or startled. We suggest that the condition be called hereditary stiff-baby syndrome. The family history is suggestive of autosomal dominant inheritance. (Am J Dis Child 1981;135:909-911) References 1. Klein R, Haddow JE, De Luca C: Familial congenital disorder resembling stiff-man syndrome . Am J Dis Child 1972;124:730-731. 2. Stevens H: ' Jumping Frenchmen of Maine .' Arch Neurol 1965;12:311-314.Crossref 3. Moersch FB, Woltman HW: Progressive fluctuating muscular rigidity and spasms (stiffman syndrome) . Proc Staff Meet Mayo Clin 1956;31:421. 4. Gordon EE, Januszko DM, Kaufman L: A critical survey of stiff-man syndrome . Am J Med 1967;42;582-599.Crossref 5. Andermann F, Keene D, Andermann E, et al: Startle disease or hyperekplexia . Brain 1980;103:985-997.Crossref 6. Beard GM: Remarks on 'Jumpers or Jumping Frenchmen.' J Nerv Ment Dis 1878;5:526.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Oct 1, 1981

References