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A case of recurrent spinal cord compression at craniocervical junction due to type IV mucopolysaccharidosis

Jun Hashimoto1, Toshinari Kawasaki1, Tamaki Kobayashi1

  • 1Department of Spinal Neurosurgery, Kyoto Katsura Hospital, Kyoto, Japan.

Surgical Neurology International
|July 14, 2025
PubMed
Abstract

Insights

Mucopolysaccharidosis type VI (MPS VI) patients may require surgical intervention for cranio-cervical junction (CCJ) stenosis, even with enzyme replacement therapy (ERT). This case highlights successful surgical decompression for recurrent CCJ stenosis in an MPS VI patient.

Area of Science:

  • Biochemistry
  • Genetics
  • Neurology

Background:

  • Mucopolysaccharidosis type VI (MPS VI) is a genetic disorder causing glycosaminoglycan accumulation.
  • Early enzyme replacement therapy (ERT) is standard for MPS VI patients.
  • Cranio-cervical junction (CCJ) stenosis is a known complication of MPS VI.

Observation:

  • A 10-month-old male with MPS VI underwent foramen magnum decompression (FMD)/C1 laminectomy.
  • Recurrent myelopathy and gait disturbances occurred despite ERT.
  • At age 15, imaging revealed a retro-odontoid mass causing CCJ stenosis.

Findings:

  • The patient underwent an extended FMD (C1-C3 laminectomy and expansive duroplasty) for retro-odontoid disease.
  • Surgical decompression led to gradual improvement in gait disturbances.
  • This demonstrates the potential need for surgical intervention in recurrent CCJ stenosis.

Implications:

  • Recurrent CCJ stenosis can occur in MPS VI patients despite ERT.
  • Surgical management, including extended FMD, can be effective for severe CCJ stenosis.
  • This case underscores the importance of vigilant monitoring and tailored treatment strategies for MPS VI.
Keywords:
Cranio-cervical junctionEnzyme replacement therapyForamen magnum decompressionMucopolysaccharidosis VISpinal cord compression

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