Hereditary Spastic Paraplegia

Hereditary Spastic Paraplegia (HSP) is a heterogeneous group of inherited neurodegenerative disorders with weakness and spasticity of the lower limbs as its main clinical feature. Hereditary spastic paraplegia (HSP), also called hereditary spastic paraparesis, familial spastic paralysis and Strümpell-Lorrain syndrome was first reported in 1876 by Seeligmüller, followed by reports by Strumpell and Lorraine. HSP is a heterogeneous group of neurodegenerative disorders with progressive lower limb spasticity and weakness leading to abnormal gait as the principal clinical feature. HSP is generally classified as pure when lower limb spasticity and weakness, hyperreflexia, extensor-plantar responses, decreased vibration sense at the ankles, bladder dysfunction, pes cavus and scoliosis are the only signs; if there are additional neurologic or extraneurologic signs, it is classified as complicated HSP.The primary neuropathology of pure HSPs is axonal degeneration of the long sensory and motor axons, affecting primarily the distal ends of the corticospinal tracts at the thoracic level, and the dorsal column fibers (especially fasciculus gracilis) at the cervico-medullary level.

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Last date updated on September, 2024