A study of infantile motor neuron disease with neurofilament and ubiquitin immunocytochemistry

S. Lee, Y. D. Park, S. H C Yen, H. Ksiezak-Reding, J. E. Goldman, D. W. Dickson

Research output: Contribution to journalArticle

63 Citations (Scopus)

Abstract

We report a patient with infantile motor neuron disease who had pathologic findings consistent with multisystem degeneration. Although the muscle showed denervation atrophy or ballooning degeneration consistent with lower motor neuron disease, the infant was hypertonic and spastic. Degenerative changes were also detected in the dorsal root ganglia, cerebellum, and thalamus. Immunohistochemical studies showed a paucity of neurofilament (NF) staining in the corticospinal tract and accumulation of phosphorylated NF ballooned neurons. Antibodies to ubiquitin immunostained ballooned neurons in the dorsal root ganglia, anterior horns, and thalamus. Accumulation of ubiquitinated and phosphorylated NF epitopes in degenerating neurons suggest that basic abnormalities in the neuronal cytoskeleton may be instrumental in the pathogenesis of this disorder.

Original languageEnglish (US)
Pages (from-to)107-111
Number of pages5
JournalNeuropediatrics
Volume20
Issue number2
StatePublished - 1989

Fingerprint

Motor Neuron Disease
Intermediate Filaments
Ubiquitin
Immunohistochemistry
Spinal Ganglia
Thalamus
Neurons
Muscle Denervation
Pyramidal Tracts
Muscle Spasticity
Horns
Cytoskeleton
Cerebellum
Atrophy
Epitopes
Staining and Labeling
Antibodies

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

Cite this

Lee, S., Park, Y. D., Yen, S. H. C., Ksiezak-Reding, H., Goldman, J. E., & Dickson, D. W. (1989). A study of infantile motor neuron disease with neurofilament and ubiquitin immunocytochemistry. Neuropediatrics, 20(2), 107-111.

A study of infantile motor neuron disease with neurofilament and ubiquitin immunocytochemistry. / Lee, S.; Park, Y. D.; Yen, S. H C; Ksiezak-Reding, H.; Goldman, J. E.; Dickson, D. W.

In: Neuropediatrics, Vol. 20, No. 2, 1989, p. 107-111.

Research output: Contribution to journalArticle

Lee, S, Park, YD, Yen, SHC, Ksiezak-Reding, H, Goldman, JE & Dickson, DW 1989, 'A study of infantile motor neuron disease with neurofilament and ubiquitin immunocytochemistry', Neuropediatrics, vol. 20, no. 2, pp. 107-111.
Lee S, Park YD, Yen SHC, Ksiezak-Reding H, Goldman JE, Dickson DW. A study of infantile motor neuron disease with neurofilament and ubiquitin immunocytochemistry. Neuropediatrics. 1989;20(2):107-111.
Lee, S. ; Park, Y. D. ; Yen, S. H C ; Ksiezak-Reding, H. ; Goldman, J. E. ; Dickson, D. W. / A study of infantile motor neuron disease with neurofilament and ubiquitin immunocytochemistry. In: Neuropediatrics. 1989 ; Vol. 20, No. 2. pp. 107-111.
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