The following report from the MHC-database is based on the latest version of the Mun-H-Center Observation Chart that has been in use since 2008. Please note, that this report exclude children younger than 3 years.
Reports based on the original forms (1996-2008) are presented at the bottom of this page - below the description of the diagnosis
Synonyms
Hereditary Spastic Paraplegia, Strumpell-Lorraine Syndrome
ICD-10
G11.4
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.
MHC-basen – The Mun-H-Center database on oral health and orofacial function in rare diseases.
The Newsletter of the Ågrenska Center
