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Hereditary spastic paraplegia

Disease definition

A genetically and clinically heterogeneous group of slowly progressive neurological disorders which in the pure form is characterized by pyramidal signs (weakness, spasticity, brisk tendon reflexes, and extensor plantar responses) predominantly affecting the lower limbs and with possible association of sphincter disturbances and deep sensory loss; and in the complex form by the addition of variable neurological or non-neurological features.


Classification level: Group of disorders
  • Synonym(s):
    • Familial spastic paraplegia
    • HSP
    • Hereditary spastic paraparesis
    • SPG
    • Strümpell-Lorrain disease
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant or Autosomal recessive or X-linked recessive 
  • Age of onset: All ages
  • ICD-10: G11.4
  • ICD-11: 8B44.0
  • OMIM: -
  • UMLS: C0037773
  • MeSH: D015419
  • GARD: 6637
  • MedDRA: 10019903

Detailed information

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