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Hypertrophic pachymeningoencephalitis associated with temporal giant cell arteritis

Gesine Boisch1, Sven Duda2, Christian Hartmann3

  • 1Department of Neurology, Ammerland Klinik GmbH, Westerstede, Germany.

BMJ Case Reports
|September 29, 2018
PubMed

Insights

This case study details hypertrophic pachymeningoencephalitis associated with temporal arteritis in an 80-year-old male. Treatment with corticosteroids improved neurological deficits caused by inflammation.

Area of Science:

  • Neurology
  • Immunology
  • Pathology

Background:

  • Hypertrophic pachymeningoencephalitis is a rare condition affecting the brain's meninges.
  • Temporal arteritis, a form of giant cell arteritis, is a systemic inflammatory disease.
  • Association between hypertrophic pachymeningoencephalitis and giant cell arteritis is infrequently reported.

Observation:

  • An 80-year-old male with a history of temporal arteritis presented with paresis and ataxia.
  • Brain MRI revealed enhancement of the left frontoparietal meninges with adjacent cortical edema.
  • Leptomeningeal biopsy confirmed autoimmune-mediated immunoglobulin G4-associated hypertrophic pachymeningoencephalitis.

Findings:

  • The patient was diagnosed with hypertrophic pachymeningoencephalitis associated with giant cell arteritis.
  • Cerebral perifocal edema was identified as a potential cause of central neurological deficits.
  • High-dose corticosteroid therapy led to gradual improvement of the patient's symptoms.

Implications:

  • Hypertrophic pachymeningoencephalitis can be a rare complication of giant cell arteritis.
  • This condition may manifest with central neurological deficits due to cerebral edema.
  • Early diagnosis and treatment with corticosteroids are crucial for managing this rare neurological complication.
Keywords:
cerebral palsyneurosurgerypathologyvasculitis

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