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Hereditary spastic paraplegia

Codes

ICD-10: G11.4

ORPHA: 685

General information

Estimated occurrence
Very rare.
Cause
Genetic. Hereditary spastic paraplegia (HSP) consists of a number of subgroups with similar symptoms. A variety of inheritance patterns are represented. In persons with HSP, a gradual degeneration of motor neural structures takes place.
General symptoms
Spasticity and insidiously progressive muscle weakness that primarily affects gait pattern. Spasticity may lead to foot deformities. Arm and hand function are often unaffected. Unstable balance and ataxia (impaired control of voluntary movements) occur. Other symptoms that may be associated with HSP include speech difficulties, visual impairment, learning disability, epilepsy and urinary symptoms. Symptom onset occurs between 10 and 40 years of age.
Synonyms
Hereditary Spastic Paraparesis, Strumpell-Lorraine Syndrome.

Orofacial/odontological symptoms

Oral motor function may also be affected in HSP and produce symptoms such as eating difficulties, speech difficulties (dysarthria) and drooling. There are no known specific odontological symptoms associated with HSP.

Advice on follow-up and treatment

  • Speech and language impairments are to be diagnosed and treated by a speech-language pathologist.
  • Oral motor training and stimulation may be relevant in cases of eating difficulties, speech impairment and drooling.

Sources

Updated: 2019-11-12 14:47