Burden of Surgical Treatment for the Management of Cervical Myelopathy in Mucopolysaccharidoses: A Systematic Review

Overview

Journal Brain Sci

Publisher MDPI

Date 2023 Jan 21

PMID 36672030

Authors

Roberta Costanzo

Lapo Bonosi

Massimiliano Porzio

Federica Paolini

Lara Brunasso

Andrea Evier Giovannini

Manikon Poullay Silven

Giuseppe Roberto Giammalva

Giuseppe Emmanuele Umana

Gianluca Scalia

Domenico Gerardo Iacopino

Rosario Maugeri

Affiliations

Soon will be listed here.

Abstract

Mucopolysaccharidoses (MPSs) are a rare group of heterogeneous genetic and metabolic disorders, caused by loss of functions of several enzymes that are involved in glycosaminoglycan catabolism. Their progressive accumulations in cells, tissues, and consequently, organs lead to several clinical manifestations, such as musculoskeletal involvement. Indeed, the most common manifestation in the central nervous system is represented by cervical spinal stenosis due to bony alterations or dural thickening. Cervical involvement can commonly cause myelopathy and instability exerting severe symptoms. A prompt diagnosis and treatment of the aforementioned conditions is mandatory to ensure a better quality of life in patients with such debilitating disorders. Nevertheless, a clear consensus about their management (i.e., surgical or not) is still lacking, leading to an inevitable delay. This review aims to investigate and discuss the main causes of myelopathy in patients with mucopolysaccharidoses, available therapeutic strategies, and the impact and role of surgery on the neurological outcome.

Citing Articles

Congenital Cervical Stenosis: a Review of the Current Literature.

Goodwin A, Hsu W Curr Rev Musculoskelet Med. 2023; 16(9):438-445.

PMID: 37452915 PMC: 10427602. DOI: 10.1007/s12178-023-09857-9.

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