Skip to main content
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1977 Oct;40(10):1003–1008. doi: 10.1136/jnnp.40.10.1003

Strumpell's pure familial spastic paraplegia: case study and review of the literature.

G L Holmes, B A Shaywitz
PMCID: PMC492884  PMID: 591968

Abstract

A family with pure Strumpell's familial paraplegia is presented. There were 11 afflicted members involving three generations. The mode of inheritance was dominant, the onset in the first decade, and in this family the disease was mild. Literature data from 104 families with 536 members dating from 1880 are tabulated. This report confirms others regarding mode of inheritance, age of onset, distribution between sexes, and disease manifestations. However, contrary to other reports, we found the dominant and recessive form of pure Strumpell's familial spastic paraplegia to be similar in severity. There are now clinical and pathological data supporting the separation of pure Strumpell's familial spastic paraplegia from the other heredodegenerative diseases of the nervous system.

Full text

PDF
1003

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BRUYN G. W., MECHELSE K. The association of familial spastic paraplegia and epilepsy in one family. Psychiatr Neurol Neurochir. 1962 Jul-Aug;65:280–292. [PubMed] [Google Scholar]
  2. Baar H. S., Gabriel A. M. Sex-linked spastic paraplegia. Am J Ment Defic. 1966 Jul;71(1):13–18. [PubMed] [Google Scholar]
  3. Behan W. M., Maia M. Strümpell's familial spastic paraplegia: genetics and neuropathology. J Neurol Neurosurg Psychiatry. 1974 Jan;37(1):8–20. doi: 10.1136/jnnp.37.1.8. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. DICK A. P., STEVENSON C. J. Hereditary spastic paraplegia; report of a family with associated extrapyramidal signs. Lancet. 1953 May 9;1(6767):921–923. doi: 10.1016/s0140-6736(53)92061-3. [DOI] [PubMed] [Google Scholar]
  5. Frank G., Vuia O. Chorea Huntington--Amyotrophische Lateralsklerose--Spastische Spinalparalyse. Zur Kombination von Systemerkrankungen. Z Neurol. 1973 Nov 5;205(3):207–220. [PubMed] [Google Scholar]
  6. GARLAND H. G., ASTLEY C. E. Hereditary spastic paraplegia with amyotrophy and pes cavus. J Neurol Neurosurg Psychiatry. 1950 May;13(2):130–133. doi: 10.1136/jnnp.13.2.130. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. GILMAN S., HORENSTEIN S. FAMILIAL AMYOTROPHIC DYSTONIC PARAPLEGIA. Brain. 1964 Mar;87:51–66. doi: 10.1093/brain/87.1.51. [DOI] [PubMed] [Google Scholar]
  8. Gamboa I., Hernández Peniche J. Diplejia espástica familiar Estudio de una familia mexicana. Rev Invest Clin. 1973 Jan-Mar;25(1):47–50. [PubMed] [Google Scholar]
  9. JOHNSTON A. W., McKUSICK V. A. A sex-linked recessive form of spastic paraplegia. Am J Hum Genet. 1962 Mar;14:83–94. [PMC free article] [PubMed] [Google Scholar]
  10. KJELLIN K. Familial spastic paraplegia with amyotrophy, oligophrenia, and central retinal degeneration. Arch Neurol. 1959 Aug;1:133–140. doi: 10.1001/archneur.1959.03840020007002. [DOI] [PubMed] [Google Scholar]
  11. Koenig R. H., Spiro A. J. Hereditary spastic paraparesis with sensory neuropathy. Dev Med Child Neurol. 1970 Oct;12(5):576–581. doi: 10.1111/j.1469-8749.1970.tb01965.x. [DOI] [PubMed] [Google Scholar]
  12. LANDAU W. M., GITT J. J. Hereditary spastic paraplegia and hereditary ataxia: a family demonstrating a variety of phenotypic manifestations. AMA Arch Neurol Psychiatry. 1951 Sep;66(3):346–354. doi: 10.1001/archneurpsyc.1951.02320090095008. [DOI] [PubMed] [Google Scholar]
  13. POSER C. M., DEWULF A., VAN BOGAERT L. Atypical cerebellar degeneration associated with leucodystrophy; a study of the relationship between dissimilar degenerative processes. J Neuropathol Exp Neurol. 1957 Apr;16(2):209–237. doi: 10.1097/00005072-195704000-00002. [DOI] [PubMed] [Google Scholar]
  14. REFSUM S., SKILLICORN S. A. Amyotrophic familial spastic paraplegia. Neurology. 1954 Jan;4(1):40–47. doi: 10.1212/wnl.4.1.40. [DOI] [PubMed] [Google Scholar]
  15. SCHWARZ G. A. Hereditary (familial) spastic paraplegia. AMA Arch Neurol Psychiatry. 1952 Nov;68(5):655–662. doi: 10.1001/archneurpsyc.1952.02320230081010. [DOI] [PubMed] [Google Scholar]
  16. SJOGREN T., LARSSON T. Oligophrenia in combination with congenital ichthyosis and spastic disorders; a clinical and genetic study. Acta Psychiatr Neurol Scand Suppl. 1957;113:1–112. [PubMed] [Google Scholar]
  17. SUTHERLAND J. M. Familial spastic paraplegia; its relation to mental and cardiac abnormalities. Lancet. 1957 Jul 27;273(6987):169–170. doi: 10.1016/s0140-6736(57)90618-9. [DOI] [PubMed] [Google Scholar]
  18. VAN BOGAERT L. Etude génétique sur les paraplégies spasmodiques familiales. J Genet Hum. 1952 May;1(1):6–23. [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES