Remote spinal cord injury in mucopolysaccharidosis type IVA after cervical decompression.
نویسندگان
چکیده
Morquio A syndrome (Online Mendelian Inheritance in Man #253000) is a lysosomal storage disease caused by deficiency of N-acetylgalactosamine-6-sulfatase encoded by the GALNS gene. Key clinical features are skeletal dysplasia and short stature.
منابع مشابه
Neurologic outcome of early versus late surgery for cervical spinal cord injury.
STUDY DESIGN A prospective analysis evaluating neurologic outcome after early versus late surgery for cervical spinal cord trauma. OBJECTIVES The study was conducted to determine whether neurologic and functional outcome is improved in traumatic cervical spinal cord-injured patients (C3-T1, American Spinal Injury Association grades A-D) who had early surgery (<72 hours after spinal cord injur...
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One third of all spinal injuries involve cervical vertebrae, and the impact of injury to the cervical spinal cord is profound and requires systemic treatment. The role and timing of surgical decompression after an acute spinal cord injury (SCI) remains one of the most controversial topics pertaining to spinal surgery. Lack of controlled, prospective, multicenter clinical studies has contributed...
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Objective(s): Glial cell line-derived neurotrophic factor (GDNF) can effectively promote axonal regeneration，limit axonal retraction，and produce a statistically significant improvement in motor recovery after spinal cord injury (SCI). However, the role in primate animals with SCI is not fully cognized. Materials and Methods:18 healthy juvenile rhesuses were divided randomly into six groups, obs...
متن کاملThe influence of the energy of trauma, the timing of decompression, and the impact of grade of SCI on outcome
STUDY DESIGN Retrospective cohort study. OBJECTIVES To find out: (1) if the energy of trauma (high and low) influence the outcome after cervical spinal cord injury; (2) if time to decompression and degree of injury (complete and incomplete) influence the outcome after high- and low-energy cervical spinal cord injury. METHODS Twenty-one consecutive patients with low-energy cervical spinal...
متن کاملNatural history and clinical assessment of Taiwanese patients with mucopolysaccharidosis IVA
BACKGROUND Mucopolysaccharidosis IVA (MPS IVA) is a rare lysosomal storage disorder caused by N-acetylgalactosamine-6-sulfatase deficiency, which catalyzes a step in the catabolism of glycosaminoglycans, keratan sulfate and chondroitin-6-sulfate. This disease has a variable age of onset and rate of progression. METHODS A retrospective analysis of medical records of 24 patients with MPS IVA (1...
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عنوان ژورنال:
- Neurology
دوره 82 15 شماره
صفحات -
تاریخ انتشار 2014