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Updated: Jun 15, 2026

Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe
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Two patients with proatlas segmentation malformation.

Shangchen Xu1, Qi Pang, Kaining Zhang

  • 1Department of Neurosurgery, Shangdong Provincial Hospital, Shandong University, 314 Jingwuweiqi Road, Jinan 250021, China.

Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
|March 2, 2010
PubMed
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Proatlas segmentation malformation (PSM) causes progressive spastic quadriparesis. Aggressive surgical intervention, confirmed by MRI and 3D-CT scans, can significantly improve neurological status and decompress the cervico-medulla junction.

Area of Science:

  • Neurosurgery
  • Radiology
  • Orthopedic Surgery

Background:

  • Progressive spastic quadriparesis can result from complex craniovertebral junction abnormalities.
  • Proatlas segmentation malformation (PSM) is a rare congenital anomaly affecting the upper cervical spine.
  • Early diagnosis and intervention are crucial for managing neurological deficits associated with PSM.

Observation:

  • Two patients with PSM presented with progressive spastic quadriparesis of significant duration.
  • Three-dimensional reconstructive CT scans and MRI confirmed the diagnosis of PSM in both cases.
  • One patient underwent a multi-stage surgical procedure including anterior decompression, cranial traction, and posterior occipital-cervical fixation.

Findings:

  • Postoperative imaging demonstrated successful realignment of the occipito-atlanto-axial complex.

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  • Surgical decompression of the cervico-medulla junction was achieved.
  • The patient experienced remarkable improvement in neurological status and relief of syringomyelia.

    • Implications:

    • MRI and 3D-CT are definitive diagnostic modalities for PSM.
    • Aggressive surgical management is indicated for PSM to improve outcomes.
    • Timely intervention can prevent irreversible neurological damage and improve quality of life.