Strumpell-lorrain Disease
Strumpell-lorrain Disease Definition
The common feature of these syndromes is progressive, often severe, spasticity in the lower extremities of the patient's body. Hereditary spastic paraplegia (HSP) is also known as familial spastic paraparesis and Strumpell-Lorrain syndrome. A number of clinical reports have documented that HSP syndromes are heterogeneous. Syndromes are categorized as uncomplicated or pure when only spinal involvement occurs, and they are classified as complicated when they are associated with neurologic abnormalities such as ataxia, mental retardation, dementia, extrapyramidal dysfunctions, visual or hearing dysfunctions, adrenal insufficiency, and ichthyosis. Inheritance of the disease may be X-linked, autosomal recessive, or autosomal dominant. The most useful classifications now are based on inheritance and genetic linkage. Clinical distinctions between pure and complicated forms of HSP have some utility; however, age of onset usually has no clear relation to the HSP genotype.