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Diagnosis of Atlantoaxial Subluxation in Morquio's Syndrome and Spondyloepiphyseal Dysplasia Congenita
Author(s) -
Takeda Eiji,
Hashimoto Toshiaki,
Tayama Masanobu,
Miyazaki Masahito,
Shirakawa Etsuhisa,
Shiino Yoshiro,
Saijo Takahiko,
Ito Michinori,
Naito Etsuo,
Huq A.H.M. Mahbubul,
Kuroda Yasuhiro
Publication year - 1991
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1991.tb01877.x
Subject(s) - medicine , magnetic resonance imaging , subluxation , spinal cord compression , spinal cord , radiology , pathology , alternative medicine , psychiatry
Compression of the spinal cord due to atlantoaxial subluxation was diagnosed in a patient with Morquio's syndrome and in another with spondyloepiphyseal dysplasia (SED) congenita by cervical radiography and magnetic resonance imaging (MRI). The patient with Morquio's syndrome, a 15 year old boy, had no neurologic symptoms and his somatosensory evoked potential (SSEP) was normal. However, MRI demonstrated spinal cord compression at C1‐C2. In contrast, the patient with SED congenita, an 11 year old girl, had neck pain, hyperreflexia and loss of vibration sense in both legs. These findings were explained by the absence of P3 and later waves in SSEP and by compression of the spinal cord observed on MRI. Both SSEP and MRI should be used for evaluating disorders in which atlantoaxial subluxation might be present.