Diseases & Conditions


Paraplegia, Hereditary Spastic

Synonyms of Paraplegia, Hereditary Spastic
  • Familial Spastic Paraplegia
  • FSP
  • HSP
  • Spastic Spinal Familial Paralysis
  • Strumpell Disease
  • Strumpell-Lorraine Syndrome
  • Strumpell-Lorrain Familial Spasmodic Paraplegia
  • Strumpell's Familial Paraplegia

Disorder Subdivisions

  • Hereditary spastic paraplegia, complicated
  • Hereditary spastic paraplegia, uncomplicated (pure)

General Discussion
Hereditary spastic paraplegia (HSP) is a group of inherited neurological disorders characterized by progressive weakness (paraplegia) and increased muscle tone and stiffness (spasticity) of leg muscles. HSP is also sometimes referred to as familial spastic paraplegia (FSP) or Strumpell-Lorraine syndrome. The age at symptom onset and the degree of muscle weakness and spasticity may be extremely variable from case to case, including among individuals within the same family (kindred). According to reports in the medical literature, symptom onset may occur as early as infancy or as late as the eighth or ninth decade of life; however, symptoms may most often develop during early to mid-adulthood. Initial findings typically include stiffness and relatively mild weakness of leg muscles, balance difficulties, unexplained tripping and falls, and an unusually clumsy manner of walking (gait). As the disorder progresses, walking may become increasingly difficult. However, complete loss of the ability to walk is relatively rare.

HSP may be classified into two major subtypes: uncomplicated or complicated HSP. In individuals with uncomplicated (or pure) HSP, progressive spastic paraplegia occurs as an isolated, primary finding. In those with complicated HSP, additional neurologic abnormalities are present. Some individuals with uncomplicated HSP may develop muscle spasms and difficulties with bladder control. In those with complicated HSP, associated symptoms and findings may include visual and/or hearing impairment, mental retardation, impaired control of voluntary movements (ataxia), and/or other abnormalities.

According to researchers, changes (mutations) of many different genes may cause HSP. In most cases, such mutations appear to be transmitted as an autosomal dominant trait. More rarely, mutations for HSP may be inherited as an autosomal recessive or X-linked recessive trait. The basic underlying defect or defects in HSP are unknown. However, associated symptoms appear to result from progressive degenerative changes of regions of the spinal cord (corticospinal tracts) that convey motor impulses from the brain to muscles involved in controlling certain voluntary movements

Organizations related to Paraplegia, Hereditary Spastic
  • MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
    150 Custer Court
    Green Bay WI 54301-1243
    Phone #: 920-336-5333
    800 #: 877-336-5333
    e-mail: [email protected]
    Home page: http://www.netnet.net/mums/
  • National Institute of Neurological Disorders and Stroke (NINDS)
    31 Center Drive
    Bethesda MD 20892-2540
    Phone #: 301-496-5751
    800 #: 800-352-9424
    e-mail: [email protected]
    Home page: http://www.ninds.nih.gov/
  • Spastic Paraplegia Foundation
    11 Douglass Green
    Woburn MA 01801
    Phone #: N/A
    800 #: 877-773-4483
    e-mail: [email protected]
    Home page: http://sp-foundation.org
  • Tom Wahlig Foundation - JENA
    Veghestrasse 22
    Muenster Intl 48149
    Phone #: 49 -251- 2007 9120
    800 #: --
    e-mail: [email protected]
    Home page: http://www.fsp-info.de
  • WE MOVE (Worldwide Education and Awareness for Movement Disorders)
    204 West 84th Street
    New York NY 10024
    Phone #: 212-875-8312
    800 #: N/A
    e-mail: [email protected]
    Home page: http://www.wemove.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html