Document Detail


Hypoplasia of posterior spinal roots and dorsal spinal tracts with arthrogryposis multiplex congenita.
MedLine Citation:
PMID:  2360413     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
H Vogel; D Halpert; D S Horoupian
Related Documents :
12408183 - Phenylketonuria in adulthood: a collaborative study.
14670783 - Brain haemorrhage in five infants with coagulopathy.
8571933 - Neonatal jaundice and molecular mutations in glucose-6-phosphate dehydrogenase deficien...
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    
Affiliation:
Department of Pathology, Stanford University Medical Center, CA 94305.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Arthrogryposis / pathology*
Female
Humans
Infant, Newborn
Spinal Cord / pathology*
Spinal Nerve Roots / pathology*
Comments/Corrections
Erratum In:
Acta Neuropathol (Berl) 1991;81(4):474

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Transferrin receptors in injured brain.
Next Document:  Cerebral medulloepithelioma--electron microscopy and immunohistochemistry.