Document Detail

Hypoplasia of posterior spinal roots and dorsal spinal tracts with arthrogryposis multiplex congenita.
MedLine Citation:
PMID:  2360413     Owner:  NLM     Status:  MEDLINE    
We report a male infant with arthrogryposis multiplex congenita (AMC) who survived for 19 weeks following birth at 36 weeks gestational age. No heritable or acquired cause of neuromuscular disease was found. He manifested joint contractures of upper and lower extremities, diffuse hypotonia requiring ventilatory support, and areflexia; the general examination also showed facial dysmorphisms, and an ichthyotic rash. Pathological examination of the brain and spinal cord revealed severe hypoplasia of dorsal roots and posterior columns, nondecussation of pyramidal tracts, and anterior horns of an unusual configuration; the brain was normal, and the cerebellum contained Purkinje cell heterotopias. Muscle spindles could not be identified. To our knowledge, these spinal cord abnormalities in association with AMC have not been previously reported, thus raising interesting speculations about the possible role of such abnormalities in the pathogenesis of AMC.
H Vogel; D Halpert; D S Horoupian
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta neuropathologica     Volume:  79     ISSN:  0001-6322     ISO Abbreviation:  Acta Neuropathol.     Publication Date:  1990  
Date Detail:
Created Date:  1990-07-31     Completed Date:  1990-07-31     Revised Date:  2007-11-09    
Medline Journal Info:
Nlm Unique ID:  0412041     Medline TA:  Acta Neuropathol     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  692-6     Citation Subset:  IM    
Department of Pathology, Stanford University Medical Center, CA 94305.
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MeSH Terms
Arthrogryposis / pathology*
Infant, Newborn
Spinal Cord / pathology*
Spinal Nerve Roots / pathology*
Erratum In:
Acta Neuropathol (Berl) 1991;81(4):474

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