Axonal dystrophy in a case of connatal thalamic and brain stem degeneration

A neonate with a rapidly fatal disease characterized by connatal hypertonia and arthrogryposis multiplex is described. Neuropathological investigations revealed bilateral thalamus and brain stem degeneration, axonal degeneration of pyramidal and other tracts in the spinal cord, and axonal spheroids...

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Bibliographic Details
Published in:Acta neuropathologica 1984-01, Vol.64 (1), p.68-71
Main Authors: JENNEKENS, F. G. I, BARTH, P. G, FLEURY, P, VELDMAN, H, KEUNING, J. F, WESTDORP, J
Format: Article
Language:English
Subjects:
Anterior Horn Cells - ultrastructure
Axons - ultrastructure
Biological and medical sciences
Brain Stem - pathology
Humans
Inclusion Bodies - ultrastructure
Infant, Newborn
Male
Medical sciences
Microscopy, Electron
Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis
Myelin Sheath - ultrastructure
Nerve Degeneration
Neurology
Peripheral Nerves - pathology
Spinal Cord - pathology
Spinal Nerve Roots - pathology
Thalamic Diseases - congenital
Thalamic Diseases - pathology
Thalamus - pathology
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Summary:A neonate with a rapidly fatal disease characterized by connatal hypertonia and arthrogryposis multiplex is described. Neuropathological investigations revealed bilateral thalamus and brain stem degeneration, axonal degeneration of pyramidal and other tracts in the spinal cord, and axonal spheroids in areas of origin of lower motor neurons and in the brain stem reticular substance. Congenital thalamic and brain stem degeneration is generally assumed to be the result of intrauterine asphyxia. The widespread occurrence of axonal spheroids in the present neonate points to the possibility of a genetic or toxic origin for at least some of these cases.
ISSN:0001-6322
1432-0533
DOI:10.1007/BF00695608