Cervical cord compression and severe hydrocephalus in a child with Saudi variant of multiple sulfatase deficiency. Report of case
| Autor: | M. O. Hassanen, A. R. Choudhury, K. R. Al-Moutaery |
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| Rok vydání: | 1994 |
| Předmět: |
musculoskeletal diseases
Male medicine.medical_specialty Cord Decompression Ventriculoperitoneal Shunt Postoperative Complications Multiple sulfatase deficiency Spinal cord compression Medicine Humans Foramen magnum business.industry Brain Diseases Metabolic Brain Anatomy Cervical cord compression Spinal cord medicine.disease Magnetic Resonance Imaging Surgery Hydrocephalus medicine.anatomical_structure Spinal Cord Child Preschool Neurology (clinical) Sulfatases business Spinal Cord Compression |
| Zdroj: | Acta neurochirurgica. 131(1-2) |
| ISSN: | 0001-6268 |
| Popis: | We report a 2.5-year-old boy with Saudi variant of multiple sulfatase deficiency (MSD or Austin's disease). He presented with the features of cervical cord compression and a severe form of hydrocephalus. The former was due to a thickened posterior arch of the atlas and the latter from a narrow foramen magnum and meningeal thickening. Decompression of the cord was achieved by removal of the posterior margin of the foramen magnum and posterior arch of the atlas, and followed by a duroplasty. At a later date, ventricular decompression was achieved by insertion of a ventricular-peritoneal shunt. NMR did not demonstrate white matter changes in the brain. In this regard the reported case differs from the earlier description of the Saudi Variant of MSD. |
| Databáze: | OpenAIRE |
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