Acta Neuropathologica

, Volume 17, Issue 4, pp 331–340

Neuroaxonal dystrophy

A case of delayed onset and protracted course
  • B. Rozdilsky
  • C. F. Bolton
  • M. Takeda
Original Investigations

DOI: 10.1007/BF00685019

Cite this article as:
Rozdilsky, B., Bolton, C.F. & Takeda, M. Acta Neuropathol (1971) 17: 331. doi:10.1007/BF00685019

Summary

an unusual case of generalized neuroaxonal dystrophy is presented. This variant was characterized by late onset with visual deterioration at the age of 9 years and subsequent slow but steady progression resulting in impairment of visual and motor function, spasticity and ataxia. A more rapid deterioration occurred after the age of 18 years resulting in complete blindness, cranial nerve palsies and spasticity. He died of intercurrent infection at the age of 21 years. Examination of the brain revealed widespread neuroaxonal dystrophy which severely affected all the grey matter of the cerebral hemisphere but caused minimal involvement of the cerebellum. The pigmentation of the pallidum was only slightly increased. It is felt that this case constitutes a juvenile variant of neuroaxonal dystrophy, possibly a link between infantile neuroaxonal dystrophy and the adult form of Hallervorden-Spatz disease.

Key-Words

Infantile Neuroaxonal Dystrophy Juvenile Type Hallervorden-Spatz Disease Cortical Involvement Sparing of Cerebellum 

Copyright information

© Springer-Verlag 1971

Authors and Affiliations

  • B. Rozdilsky
    • 1
  • C. F. Bolton
    • 1
  • M. Takeda
    • 1
  1. 1.Divisions of Neuropathology and NeurologyUniversity of Saskatchewan College of MedicineSaskatoonCanada

Personalised recommendations