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J Neurol Neurosurg Psychiatry 37:8-20 doi:10.1136/jnnp.37.1.8
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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.

      Footnotes

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

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

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