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Idiopathic hypertrophic pachymeningitis.

M J Kupersmith1, V Martin, G Heller

  • 1Institute of Neurology and Neurosurgery at Beth Israel Medical Center, New York Eye and Ear Infirmary, New York University School of Medicine, New York, NY, USA. mkuper@bethisraelny.org

Neurology
|March 10, 2004
PubMed
Summary

Idiopathic hypertrophic pachymeningitis (IHP) is a rare dural thickening disorder. MRI aids diagnosis, and while steroids offer initial relief for IHP symptoms like headache and vision loss, relapses are common, often necessitating immunosuppressants.

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Area of Science:

  • Neurology
  • Radiology
  • Pathology

Background:

  • Hypertrophic pachymeningitis (HP) is a rare condition causing dura mater thickening, linked to autoimmune diseases, infections, and cancer.
  • Idiopathic hypertrophic pachymeningitis (IHP) is less understood, with limited data on MRI findings, clinical outcomes, and treatment responses.

Purpose of the Study:

  • To evaluate the clinical and laboratory features of idiopathic hypertrophic pachymeningitis (IHP).
  • To correlate Magnetic Resonance Imaging (MRI) findings with clinical course and treatment outcomes.
  • To review existing literature on IHP.

Main Methods:

  • A retrospective case series of 12 patients diagnosed with IHP via imaging and/or biopsy.
  • Documentation of clinical presentation, laboratory results, contrast-enhanced MRI findings, treatment strategies, and patient outcomes.

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  • Mean follow-up duration of 3.5 years.

    • Main Results:

    • Common symptoms included headache (11/12) and vision loss (7/12).
    • MRI showed abnormal dural enhancement, particularly in the sphenoid wing area, correlating with clinical symptoms.
    • Corticosteroids improved vision (7/8) and headache (10/11), but recurrences were frequent (6/12) upon steroid tapering, necessitating immunosuppressive agents (methotrexate or azathioprine) in some cases. MRI correlated with clinical status (80% concordance).

    Conclusions:

    • IHP can be suspected based on MRI findings and confirmed by pathological biopsy.
    • Untreated IHP typically leads to severe headache, progressive neurological decline, and vision loss.
    • While initial treatment with corticosteroids is effective, long-term management often requires additional immunosuppressive therapy due to frequent relapses.