Diagnosis of Atlantoaxial Subluxation in Morquio's Syndrome and Spondyloepiphyseal Dysplasia Congenita

Eiji Takeda, Toshiaki Hashimoto, Masanobu Tayama, Masahito Miyazaki, Etsuhisa Shirakawa, Yoshiro Shiino, Takahiko Saijo, Michinori Ito, Etsuo Naito, A. H.M.Mahbubul Huq, Yasuhiro Kuroda

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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.

Original languageEnglish
Pages (from-to)633-638
Number of pages6
JournalPediatrics International
Issue number5
StatePublished - Oct 1991


  • Atlantoaxial subluxation
  • Key Words
  • Magnetic resonance imaging
  • Morquio's syndrome
  • Somatosensory evoked potential
  • Spondyloepiphyseal dysplasia congenital


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