[A clinicopathological study of the somatic motor efferents in the pseudopolyneuritic form of amyotrophic lateral sclerosis]

Rinsho Shinkeigaku. 1991 Feb;31(2):163-9.
[Article in Japanese]


Histopathology of the somatic motor efferents of three cases with pseudopolyneuritic from of amyotrophic lateral sclerosis (ALS) was analyzed, and the results were correlated with the clinical symptoms. The population of myelinated fibers in the lateral corticospinal tract at the thoracic segment, and in the fourth ventral roots, and motor neurons in the fourth ventral horns were morphometrically quantified. The patients were two males and one female ranging in age from 43 to 67 years, and showed a clinical course of 1.8 to 9.5 years. All three cases did not show any pyramidal sign nor spasticity but showed an extensive leg involvement, particularly the distally accentuated muscular weakness with depressed deep tendon reflexes. The clinical manifestations were consistent with the pseudopolyneuritic form of ALS. Fiber size profile of the myelinated fibers in the lateral corticospinal tracts at T7 segments and L4 lumbar ventral roots were estimated on the epon-embedded transverse sections as previously described (Sobue et al, 1981). Number and size distribution of anterior horn cells in the L4 segments were measured on 300-500 consecutive 10 microns-thick paraffin sections stained with Klüver-Barrera technique, using a TGZ-3 particle size analyzer (Zeiss). The location of the remained anterior horn cells was also quantitatively estimated. Three cases with common form of ALS and three control cases were analyzed in the same manner. The large myelinated fibers in the lateral corticospinal tract were extensively and predominantly depleted in all three cases. The small myelinated fibers were also depopulated but in lesser degree. The mode of these myelinated fiber loss was almost compatible with those in common form of ALS.(ABSTRACT TRUNCATED AT 250 WORDS)

Publication types

  • English Abstract

MeSH terms

  • Adult
  • Aged
  • Amyotrophic Lateral Sclerosis / pathology*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Motor Neurons / pathology*
  • Neurons, Efferent / pathology*
  • Polyneuropathies / pathology*