Book chapter
Hereditary spastic paraplegia
Handbook of Clinical Neurophysiology, pp.633-653
Elsevier Health Sciences
2004
DOI: 10.1016/S1567-4231(04)04037-7
Abstract
This chapter describes hereditary spastic paraplegia (HSP) or the Strümpell–Lorrain syndrome, which is the heterogeneous group of clinically and pathologically related inherited disorders where the main clinical feature is progressive bilateral lower limb spasticity and weakness. HSP is a genetically and clinically diverse group of disorders whose primary feature is progressive spasticity and pyramidal weakness of the lower extremities. HSP might be more prevalent than estimated because of incomplete ascertainment of cases. The condition arises because of degeneration of the longest motor and sensory axons in the spinal cord. The clinical picture is dependent on whether the disorder is pure or complicated. The diagnosis is still clinical and based on the exclusion of other similarly presenting disorders. Investigations help in excluding other disorders rather than confirming the diagnosis. Genetic molecular testing plays a role in the diagnosis but is still far from being comprehensive. Therapy is mainly supportive, aiming at the maintenance of ambulation as much as possible.
Details
- Title: Subtitle
- Hereditary spastic paraplegia
- Creators
- Hatem El-Shanti - University of Iowa Hospitals and Clinics
- Resource Type
- Book chapter
- Publication Details
- Handbook of Clinical Neurophysiology, pp.633-653
- Publisher
- Elsevier Health Sciences
- DOI
- 10.1016/S1567-4231(04)04037-7
- ISSN
- 1567-4231
- Language
- English
- Date published
- 2004
- Academic Unit
- Stead Family Department of Pediatrics; Medical Genetics and Genomics
- Record Identifier
- 9984354038002771
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