Article Text

PDF

Strümpell's familial spastic paraplegia: genetics and neuropathology
  1. Wilhelmina M. H. Behan1,
  2. Maria Maia2
  1. Department of Neuropathology, Radcliffe Infirmary, Oxford
  2. The M.R.C. Population Genetics Unit, Oxford

    Abstract

    Uncomplicated Strümpell's disease (Strümpell's familial spastic paraplegia) with a dominant mode of inheritance is recorded in six families. The neuropathological findings in two cases from these families are given, bringing the total of similar histologically documented reports in the literature to 11. It is concluded that, although exact classification and identification of the many different hereditary neurological degenerative diseases is not yet practicable, cases conforming to the picture described by Strümpell can be separated from larger general group of familial spastic paraplegias, show a consistent clinical picture, and have a standard pathology. It is suggested that, since the lesions are confined to the longest fibre tracts in the central nervous system, the pathological process may be different from that found in the `system' degenerations.

    Statistics from Altmetric.com

    Footnotes

    • 1 Present address: 6 Ledlameroch Crescent, Bearsden, Glasgow. G614AA.

    • 2 Present address: Department of Neurology, Sto. Antonio Hospital, Oporto, Portugal.

    Request permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.