Hereditary Spastic Paraplegia

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Introduction

Hereditary spastic paraplegia is a group of inherited neurodegenerative disorders characterized by spasticity and weakness in the lower extremities . HSP may develop at any time throughout the lifespan. Generally speaking, if the disorder is developed in early childhood, the symptoms will be non-progressive, whereas the opposite holds true if developed later in life. HSP is classified as either uncomplicated (pure) or complicated. Uncomplicated HSP, as the name suggests, means the individual presents with symptoms most typically associated with HSP, including lower extremity spasticity and weakness, hypertonic urinary disturbance, and mild deficits in lower extremity vibration sense . Furthermore, uncomplicated HSP does not involved other deficits related to the upper extremities, speech, or swallowing. Complicated HSP is distinguished by the presence of uncomplicated symptoms, in addition to many other symptoms, including ataxia, seizures, intellectual disability, dementia, amyoatrophy, extrapyramidal disturbance, and peripheral neuropathy . Causes of additional symptoms are yet to be accurately identified .

It was in 1880 where the first reported cases of HSP occured. HSP was seen in two middle aged siblings from the Estonia region by a neurologist named Ernst Adolf Gustav Gottfried von Strümpell Later that decade Maurice Lorrain, would publish a more detailed account of HSP. As a result of Strümpell’s and Lorrain’s early research HSP is also referred to today as Strümpell- Lorrain disease . HSP has also been referred to as familial spastic paraparesis .

Outcome Measures

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