Acta Neuropathologica

, Volume 75, Issue 5, pp 523–528 | Cite as

Ultrastructural study of chromatolytic neurons in an adult-onset sporadic case of amyotrophic lateral sclerosis

  • H. Kusaka
  • T. Imai
  • S. Hashimoto
  • T. Yamamoto
  • K. Maya
  • M. Yamasaki
Case Reports


Ultrastructural features of chromatolytic neurons observed in a sporadic case with amyotrophic lateral sclerosis (ALS) are reported. A 70-year-old woman died of weakness and atrophy of the four limbs, bulbar and facial muscles, and hyperreflexia, of 3 1/2 years' duration. Neuronal loss was marked in the anterior horn of the spinal cord, with degeneration of the pyramidal tracts. Most of the remaining neurons showed chromatolysis. Some of the chromatolytic neurons contained faintly eosinophilic inclusions with a halo. Few spheroids were observed. Hypoglossal nuclei, nucleus ambiguus, motor nuclei of N. VII and N.V were well populated, but contained several chromatolytic neurons. Ultrastructurally, the chromatolytic neurons contained aggregates of fibrils thicker than the 10-nm neurofilaments. These fibrils were arranged randomly, and were closely associated with granular materials as well as rough endoplasmic reticulum. Neurofilamentous accumulations reported to be common in sporadic ALS were rare in this case. No Bunina body was observed.

Key words

Amyotrophic lateral sclerosis Chromatolysis Ultrastructure Motor neuron disease Hyaline intraneuronal inclusions 


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Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • H. Kusaka
    • 1
  • T. Imai
    • 1
  • S. Hashimoto
    • 2
  • T. Yamamoto
    • 1
  • K. Maya
    • 1
  • M. Yamasaki
    • 1
  1. 1.Department of Neurology, Neurological CenterKitano HospitalKita-ku, OsakaJapan
  2. 2.Department of NeurologyTenri HospitalTenri city, NaraJapan

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