Familial syringomyelia: case report and review of the literature.

Autor: Zakeri A; Department of Neurosurgery, Millard Fillmore Hospital, Erie County Medical Center, Buffalo, New York, USA., Glasauer FE, Egnatchik JG
Jazyk: angličtina
Zdroj: Surgical neurology [Surg Neurol] 1995 Jul; Vol. 44 (1), pp. 48-53.
DOI: 10.1016/0090-3019(96)85515-7
Abstrakt: Background: Syringomyelia is an uncommon disease of the spinal cord, occurring sporadically. However, rare familial cases with autosomal dominant or recessive inheritance patterns are reported and their incidence quoted as approximately 2%. Only one previous report originated from the United States.
Methods: We present a brother and sister with syringomyelia and associated Chiari type I malformation; both patients responded to surgical treatment. We review the world literature and briefly discuss pathogenetic theories of syringomyelia as well as the relevance of the histocompatibility leukocyte antigen profile.
Results: Both genetic and environmental factors appear to be involved in familial syringomyelia.
Conclusion: We recommend that close relatives of patients affected with familial syringomyelia undergo routine neurologic and radiologic surveys.
Databáze: MEDLINE
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