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Published on: February 14, 2011

Idiopathic hypertrophic pachymeningitis responsive to mycophenolate

Angelos Assiotis1, Marios Hadjivassiliou

  • 1Trauma and Orthopaedics CT2, Charing Cross Hospital, London, UK. aassiotis9@hotmail.com

Acta Neurologica Belgica
|December 7, 2011
PubMed

Insights

Idiopathic hypertrophic pachymeningitis (IHP) is a rare central nervous system condition. Mycophenolate mofetil showed promise in stabilizing a patient who did not respond to other immunosuppressants.

Area of Science:

  • Neurology
  • Immunology
  • Rare Diseases

Background:

  • Idiopathic hypertrophic pachymeningitis (IHP) is a rare, challenging inflammatory condition affecting the central nervous system.
  • Standard treatments including high-dose steroids and pulsed cyclophosphamide can be insufficient for managing IHP.

Observation:

  • A patient presented with neurological symptoms including dysphagia, ataxia, and dysarthria, alongside headache, vomiting, and weight loss.
  • The patient was diagnosed with IHP and showed clinical deterioration despite initial immunosuppressive therapy.

Findings:

  • Administration of mycophenolate mofetil resulted in significant clinical improvement and stabilization of the patient's condition.
  • This case represents the first documented use of mycophenolate mofetil in treating IHP in English literature.

Implications:

  • Mycophenolate mofetil may be a viable therapeutic option for refractory cases of idiopathic hypertrophic pachymeningitis.
  • Further research is warranted to establish the efficacy and safety of mycophenolate mofetil in managing IHP.

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