Development and validation of a rating scale for hereditary spastic paraplegia

Hereditary spastic paraplegia (HSP) is a clinically and genetically heterogeneous group of disorders characterized by slowly progressive lower extremity spasticity and weakness due to degeneration of the corticospinal tracts. Complicating symptoms of HSP include cognitive impairment, seizures, amyotrophy and peripheral neuropathy. Little is known about the natural history of HSP and tools to measure severity of the disease are lacking. We aimed to develop and validate a HSP specific rating scale for clinical assessment of functional impairment caused by HSP. The rating scale was designed by a task force of the B…