{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T16:52:28Z","timestamp":1792515148573,"version":"4.5.0"},"reference-count":8,"publisher":"Scientific Scholar","content-domain":{"domain":["surgicalneurologyint.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:sec id=\"st1\">\n<jats:title>Background: <\/jats:title>\n<jats:p>Mucopolysaccharidosis type VI (MPS VI) is an autosomal recessive lysosomal genetic storage disorder caused by the accumulation of glycosaminoglycans in tissues and organs. A 10-month-old male with MPS VI had originally undergone foramen magnum decompression (FMD)\/C1 followed by lifelong enzyme replacement therapy (ERT). At age 15, the patient underwent successful surgical treatment for retro-odontoid disease and recurrent cranio-cervical junction (CCJ) stenosis through a C1\u2013C3 laminectomy and expansive duroplasty.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"st2\">\n<jats:title>Case Description: <\/jats:title>\n<jats:p>A 10-month-old male with MPS VI and brain stem\/spinal cord compression originally underwent a cervical FMD\/C1 laminectomy. Despite ERT administration, signs of gait disturbances and myelopathy recurred before 1 year of age. At age 15, both computed tomography and magnetic resonance imaging revealed a retro-odontoid mass causing foramen magnum stenosis\/upper cervical cord compression. Following an extended FMD that included a C1\u2013C3 laminectomy and expansive duroplasty, his gait disturbance gradually improved.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"st3\">\n<jats:title>Conclusion: <\/jats:title>\n<jats:p>Patients with MPS VI may experience recurrent CCJ stenosis and spinal cord compression despite the early initiation of ERT.<\/jats:p>\n<\/jats:sec>","DOI":"10.25259\/sni_333_2025","type":"journal-article","created":{"date-parts":[[2025,6,20]],"date-time":"2025-06-20T07:11:52Z","timestamp":1750403512000},"page":"255","update-policy":"https:\/\/doi.org\/10.25259\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["A case of recurrent spinal cord compression at craniocervical junction due to type IV mucopolysaccharidosis"],"prefix":"10.25259","volume":"16","author":[{"given":"Jun","family":"Hashimoto","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshinari","family":"Kawasaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tamaki","family":"Kobayashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshihiko","family":"Ioroi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Motohiro","family":"Takayama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"10894","published-online":{"date-parts":[[2025,6,20]]},"reference":[{"key":"10.25259\/SNI_333_2025\/ref-1","first-page":"48","article-title":"Burden of surgical treatment for the management of cervical myelopathy in mucopolysaccharidoses: A systematic 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