What is hereditary spastic paraparesis (HSP)?

Updated: Apr 28, 2014
  • Author: Hidehiro Takei, MD; Chief Editor: Adekunle M Adesina, MD, PhD  more...
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Answer

Answer

HSP, also known as familial spastic paraplegias or Strumpell-Lorrain disease, comprises a clinically and genetically heterogeneous group of hereditary disorders characterized by slowly progressive spastic paraparesis. This condition is clinically classified as taking either a pure (uncomplicated) form or a complicated form, depending on whether the paraparesis exists in isolation or in conjunction with other major clinical features. There is significant overlap in clinical characteristics between pure HSP and PLS. [2]

Genetically, HSPs are classified by the mode of inheritance (autosomal dominant, autosomal recessive, and X-linked) and are subdivided by chromosomal locus or causative gene. Pure autosomal dominant HSP is the most common form.


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