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Hypertrophic pachymeningitis.

Lewis D Hahn1, Robert Fulbright2, Joachim M Baehring3

  • 1Department of Medicine, Yale University School of Medicine, New Haven, CT 06510, United States.

Journal of the Neurological Sciences
|July 18, 2016
PubMed
Summary
This summary is machine-generated.

Hypertrophic pachymeningitis (HP), inflammation of the dura mater, presents variably. Idiopathic HP and neurosarcoidosis were most common, with imaging showing subtle differences, often requiring biopsy for diagnosis.

Keywords:
DuraEnhancementHypertrophic pachymeningitis

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Area of Science:

  • Neurology
  • Radiology
  • Pathology

Background:

  • Hypertrophic pachymeningitis (HP) involves inflammation and thickening of the dura mater.
  • Causes can be idiopathic or secondary to various conditions.
  • Clinical presentation ranges from asymptomatic to severe neurological deficits.

Purpose of the Study:

  • To determine the relative incidence of different etiologies of HP.
  • To compare associated imaging findings across etiologies.
  • To compare clinical features of underlying HP syndromes.

Main Methods:

  • Retrospective review of 22 consecutive HP cases over 10 years.
  • Analysis of clinical presentations and neuroimaging findings.
  • Comparison of etiologies, imaging characteristics, and clinical features.

Main Results:

  • Idiopathic HP and neurosarcoidosis were the most frequent causes.
  • No single imaging feature was specific for an etiology.
  • Idiopathic HP showed diffuse enhancement; neurosarcoidosis often displayed nodular enhancement.
  • Headache and cranial neuropathies were the most common symptoms.
  • Steroid treatment showed limited efficacy, with complete responses being rare.

Conclusions:

  • HP presents a diagnostic challenge due to non-specific findings on non-invasive studies.
  • Distinguishing etiologies based solely on imaging is difficult.
  • Biopsy is frequently necessary for definitive diagnosis and guiding therapy.