HEREDITARY MYELOPATHY

Spinal Cord Disorders p. 65-86 June 2005, Vol.11, No.3 doi: 10.1212/01.CON.0000293721.86238.6a
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Hereditary spastic paraplegia (HSP) is a clinically and genetically heterogeneous group of motor disorders characterized by bilateral leg spasticity and weakness. HSP is classified according to the clinical phenotype as either pure or complex, the latter presenting with neurological and systemic impairments in addition to spastic paraparesis. Inheritance may be autosomal dominant, X linked, or autosomal recessive. Many cases present without a clear family history and represent a challenge for the neurologist. A number of genes have been identified, and some are available for clinical testing, allowing genetic diagnosis in a portion of patients with HSP. Identifying the genetic cause is important for diagnosis of the patient's condition and for genetic counseling of at-risk individuals. Effective management requires a multidisciplinary approach involving neurology, genetics, physical medicine, and orthopedics.

© 2005 American Academy of Neurology