Hereditary spastic paraplegia: a practical approach.

Abstract

Hereditary spastic paraplegia (HSP) comprises a clinically and genetically diverse group of inherited neurodegenerative disorders unified by slowly progressive, usually symmetrical lower-limb spasticity secondary to corticospinal tract degeneration. For practising neurologists, the first step is recognising when a presentation is consistent with HSP and when it is not, and then carefully excluding treatable causes. We outline a pragmatic approach to assessing HSP, highlighting the broad clinical spectrum, 'red flags' that should trigger re-evaluation and the common acquired and genetic mimics. We summarise the relevant clinical and supportive investigations that can narrow the differential diagnosis. We also discuss the strengths and limitations of contemporary genetic testing. Finally, we focus on practical management: multidisciplinary care, spasticity treatments, and proactive recognition and management of additional symptoms outside of spasticity.