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Condition

Hereditary spastic paraplegia

Diseases of the Nervous System

Hereditary spastic paraplegia, often shortened to HSP, is a group of inherited diseases whose main feature is a progressive gait disorder, with growing stiffness and contraction in the lower limbs. It is also called hereditary spastic paraparesis, familial spastic paraplegia, French settlement disease, Strumpell disease or Strumpell-Lorrain disease. The symptoms arise from dysfunction of the long axons in the spinal cord, and the affected cells are the primary motor neurons, so it counts as an upper motor neuron disease. It is not a form of cerebral palsy, although it can look similar and some of the same anti-spasticity medicines are used for both. The German neurologist Adolph Strumpell first described it in 1880, Maurice Lorrain described it more fully in 1888, and Anita Harding coined the term in 1983.

Facts
Classification
Body System
Nervous System 1
ICD-10 Chapter
VI: Diseases of the Nervous System 2
Causative Agent
Causative Agent (category)
Genetic / Hereditary 1
First Described
1880 1
Sources
1. Hereditary spastic paraplegia (Wikipedia)
  • Lead, first paragraph
    the disease is an upper motor neuron disease
  • Lead, first sentence
    Hereditary spastic paraplegia (HSP) is a group of inherited diseases
  • Lead, third paragraph
    The disease was first described in 1880 by the German neurologist Adolph
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2. World Health Organization: ICD-10 Version:2019 - G10-G14 Systemic atrophies primarily affecting the central nervous system
World Health OrganizationICD-10 2019 category G11.4, Chapter VI (condition-kind)
Quote, ICD-10 2019 category G11.4, Chapter VI (condition-kind)
G11.4 Hereditary spastic paraplegia
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