Acta Neuropathologica

, Volume 64, Issue 1, pp 22–29 | Cite as

Familial amyotrophic lateral sclerosis: Features of multisystem degeneration

  • J. Tanaka
  • H. Nakamura
  • Y. Tabuchi
  • K. Takahashi
Original Works


Two sibling cases of familial amyotrophic lateral sclerosis (ALS) revealed degerneration usually associated with other systemic degenerative disorders. The changes in the 41-year-old sister were compatible with those reported in other familial ALS cases affecting the upper and lower motor neurons, posterior columns, and spinocerebellar tracts. The 45-year-old sister revealed more wide-spread degenerative changes involving not only motor neuron systems, but also proprioceptive, general somatic afferent and spinocerebellar afferent systems. Intracytoplasmic hyaline inclusions were observed in the oculomotor nuclei. Clinical manifestations of urinary disturbance and oculomotor imparirment seldom seen in sporadic ALS were interpreted to be due to the unusual distribution of the morbid process. These pathologic findings suggest that familial ALS may be a multisystemic degenerative disorder, frequently involving the spinocerebellar tracts, but occasionally involving other systems as well.

Key words

Famihal ALS Oculomotor impairment Urinary disturbance Multisystemic degeneration 


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Copyright information

© Springer-Verlag 1984

Authors and Affiliations

  • J. Tanaka
    • 1
  • H. Nakamura
    • 2
  • Y. Tabuchi
    • 2
  • K. Takahashi
    • 2
  1. 1.Division of Neuropathology Institute of Neurological SciencesTottori University School of MedicineYonagoJapan
  2. 2.Division of Neurology, Institute of Neurological SciencesTotton University School of MedicineYonagoJapan

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