REVIEW |
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Year : 2018 | Volume
: 3
| Issue : 3 | Page : 95-100 |
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What the future holds for the challenging hereditary spastic paraplegia?
Jumana Bisharat-Kernizan1, Carlton Watson2, Konstantinos Margetis MD, PhD 3
1 Department of Surgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA 2 American University of the Caribbean School of Medicine, Cupecoy, St. Maarten 3 Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA
Correspondence Address:
Konstantinos Margetis Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, NY USA
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2542-3975.242956
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Since it was initially described by and named after Strumpell and Lorrain in the late 1800s, hereditary spastic paraplegia (HSP) or familial spastic paraplegia, has remained a source of interest and study for the medical community. This rare disease, or rather spectrum of neurological diseases, is undergoing a fresh wave of unveiling as molecular and genetic techniques have bolstered our understanding of HSP. HSP is a neurodegenerative disease with a wide range of effects on patients. The mainstays of lower extremity spasticity, urinary urgency and impairment of lower extremity vibratory sensation can present alone or accompanied by a list of additional symptoms such as: epilepsy, dementia and peripheral neuropathy. In this review, some of the more recent studies are discussed, in which pathophysiology, imaging, and genetics are investigated. The review of these studies may not only help to advance our knowledge and management of HSP, but may serve as a future paradigm for similar groups of diseases that experience a wide spectrum of clinical symptoms. |
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