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[Cervical myelopathy in mucopolysaccharidosis type II (Hunter's syndrome). Neuroradiologic, clinical and histopathologic findings].
- Source :
-
Deutsche medizinische Wochenschrift (1946) [Dtsch Med Wochenschr] 1990 Sep 07; Vol. 115 (36), pp. 1348-52. - Publication Year :
- 1990
-
Abstract
- A moderately severe spasticity affecting all four limbs, but especially the legs and the right side, developed in a 36-year-old man with mucopolysaccharidosis II (Hunter syndrome) who for 6 months had suffered from pain in the neck radiating into the shoulder. Myelography, computed tomography and magnetic resonance imaging revealed thickening of the cervical meninges as the cause of the symptoms. A laminectomy was performed and the foramen magnum enlarged by an occipital craniotomy. At operation dorsal thickening of the dura was evident; thickened tissue was also palpable in front of the dural sac. Histological examination of a tissue sample revealed deposition of acid mucopolysaccharides. The gait of the patient was improved after the operation and the neck pain disappeared. Similar findings were present in other family members.
- Subjects :
- Adult
Dura Mater pathology
Humans
Iduronate Sulfatase urine
Laminectomy
Magnetic Resonance Imaging
Male
Myelography
Neck
Spinal Cord diagnostic imaging
Spinal Cord Diseases diagnostic imaging
Spinal Cord Diseases genetics
Spinal Cord Diseases pathology
Spinal Cord Diseases surgery
Tomography, X-Ray Computed
Mucopolysaccharidosis II complications
Mucopolysaccharidosis II diagnostic imaging
Mucopolysaccharidosis II genetics
Mucopolysaccharidosis II pathology
Mucopolysaccharidosis II surgery
Spinal Cord Diseases etiology
Subjects
Details
- Language :
- German
- ISSN :
- 0012-0472
- Volume :
- 115
- Issue :
- 36
- Database :
- MEDLINE
- Journal :
- Deutsche medizinische Wochenschrift (1946)
- Publication Type :
- Academic Journal
- Accession number :
- 2118851
- Full Text :
- https://doi.org/10.1055/s-2008-1065163