Hereditary Spastic Paraplegia

Reports from the MHC data base

Report from the Questionnaire (PDF)
Report from the Observation Chart (PDF)

Synonyms

  • Hereditary Spastic Paraplegia
  • Strumpell-Lorraine Syndrome

Codes

ICD-10: G11.4
ORPHA: 685

Estimated occurrence

Uncommon

Etiology

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.

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.

Orofacial/odontological treatment

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

Sources

  • The rare disease database of the Swedish National Board of Health and Welfare.
  • The MHC database - The Mun-H-Center database on oral health and orofacial function in rare diseases.
  • The Documentation from the Ågrenska national competence centre for rare diseases.
Updated: 2017-09-12 14:23