Burden of Surgical Treatment for the Management of Cervical Myelopathy in Mucopolysaccharidoses: A Systematic Review
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 manif...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-12-01
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| Series: | Brain Sciences |
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| Online Access: | https://www.mdpi.com/2076-3425/13/1/48 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Roberta Costanzo |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-09T13:23:03Z |
| format | Article |
| id | doaj.art-20c0fbfd8a824624a7d7ec4bf20d0cdf |
| institution | Directory Open Access Journal |
| issn | 2076-3425 |
| language | English |
| last_indexed | 2024-03-09T13:23:03Z |
| publishDate | 2022-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Brain Sciences |
| spelling | doaj.art-20c0fbfd8a824624a7d7ec4bf20d0cdf2023-11-30T21:26:51ZengMDPI AGBrain Sciences2076-34252022-12-011314810.3390/brainsci13010048Burden of Surgical Treatment for the Management of Cervical Myelopathy in Mucopolysaccharidoses: A Systematic ReviewRoberta Costanzo0Lapo Bonosi1Massimiliano Porzio2Federica Paolini3Lara Brunasso4Andrea Evier Giovannini5Manikon Poullay Silven6Giuseppe Roberto Giammalva7Giuseppe Emmanuele Umana8Gianluca Scalia9Domenico Gerardo Iacopino10Rosario Maugeri11Neurosurgical Clinic, AOUP “Paolo Giaccone”, Post Graduate Residency Program in Neurologic Surgery, Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, 90127 Palermo, ItalyNeurosurgical Clinic, AOUP “Paolo Giaccone”, Post Graduate Residency Program in Neurologic Surgery, Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, 90127 Palermo, ItalyNeurosurgical Clinic, AOUP “Paolo Giaccone”, Post Graduate Residency Program in Neurologic Surgery, Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, 90127 Palermo, ItalyNeurosurgical Clinic, AOUP “Paolo Giaccone”, Post Graduate Residency Program in Neurologic Surgery, Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, 90127 Palermo, ItalyNeurosurgical Clinic, AOUP “Paolo Giaccone”, Post Graduate Residency Program in Neurologic Surgery, Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, 90127 Palermo, ItalyNeurosurgical Clinic, AOUP “Paolo Giaccone”, Post Graduate Residency Program in Neurologic Surgery, Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, 90127 Palermo, ItalyNeurosurgical Clinic, AOUP “Paolo Giaccone”, Post Graduate Residency Program in Neurologic Surgery, Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, 90127 Palermo, ItalyNeurosurgical Clinic, AOUP “Paolo Giaccone”, Post Graduate Residency Program in Neurologic Surgery, Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, 90127 Palermo, ItalyDepartment of Neurosurgery, Cannizzaro Hospital, Trauma Center, Gamma Knife Center, 95125 Catania, ItalyHead and Neck Surgery Department, Neurosurgery Unit, Garibaldi Hospital, 95123 Catania, ItalyNeurosurgical Clinic, AOUP “Paolo Giaccone”, Post Graduate Residency Program in Neurologic Surgery, Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, 90127 Palermo, ItalyNeurosurgical Clinic, AOUP “Paolo Giaccone”, Post Graduate Residency Program in Neurologic Surgery, Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, 90127 Palermo, ItalyMucopolysaccharidoses (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.https://www.mdpi.com/2076-3425/13/1/48mucopolysaccharidosescervicalmyelopathyinstability |
| spellingShingle | 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 Burden of Surgical Treatment for the Management of Cervical Myelopathy in Mucopolysaccharidoses: A Systematic Review Brain Sciences mucopolysaccharidoses cervical myelopathy instability |
| title | Burden of Surgical Treatment for the Management of Cervical Myelopathy in Mucopolysaccharidoses: A Systematic Review |
| title_full | Burden of Surgical Treatment for the Management of Cervical Myelopathy in Mucopolysaccharidoses: A Systematic Review |
| title_fullStr | Burden of Surgical Treatment for the Management of Cervical Myelopathy in Mucopolysaccharidoses: A Systematic Review |
| title_full_unstemmed | Burden of Surgical Treatment for the Management of Cervical Myelopathy in Mucopolysaccharidoses: A Systematic Review |
| title_short | Burden of Surgical Treatment for the Management of Cervical Myelopathy in Mucopolysaccharidoses: A Systematic Review |
| title_sort | burden of surgical treatment for the management of cervical myelopathy in mucopolysaccharidoses a systematic review |
| topic | mucopolysaccharidoses cervical myelopathy instability |
| url | https://www.mdpi.com/2076-3425/13/1/48 |
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