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Myelopathy in mucopolysaccharidosis type II (Hunter syndrome)
Author(s) -
Ballenger Clarence E.,
Swift Thomas R.,
Leshner Robert T.,
El Gammal Taher A.,
McDonald Thomas F.
Publication year - 1980
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410070418
Subject(s) - medicine , myelopathy , hunter syndrome , mucopolysaccharidosis , spinal cord , tropical spastic paraparesis , surgery , surgical decompression , decompression , respiratory failure , anesthesia , pathology , psychiatry
A 24‐Year‐old man with Hunter syndrome had spastic quadriparesis due to impingement of thickened meninges upon the cervical spinal cord. Tracheal narrowing due to submucosal deposits (presumably mucopolysaccharide) produced serious ventilatory complications during induction of anesthesia and necessitated tracheostomy before surgical decompression of the spinal cord could be attempted. Recognition of compressive myelopathy and tracheal compromise as late complications of Hunter syndrome may promote early therapy and prevent respiratory catastrophe.