Which histologic findings are characteristic of 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

The pathways that are most severely affected in pure hereditary spastic paraparesis (HSP) are the crossed and uncrossed corticospinal tracts to the lower limbs and the fasciculus gracilis fibers from the lower limbs. The primary neuropathologic feature of pure HSP is axonal degeneration that is maximal in the terminal portions of the corticospinal tracts (descending tracts) and, to a lesser extent, of the dorsal column fibers (ascending tracts), suggesting a process of “dying back.”

Demyelination and gliosis can accompany the axonal loss. About 50% of HSP cases show spinocerebellar tract involvement. [29] Dorsal root ganglia, posterior roots, and peripheral nerves are unremarkable, as are the anterior horn cells.


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