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Isolating Central Nervous System Tissues and Associated Meninges for the Downstream Analysis of Immune cells
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Published on: May 19, 2020

Cranial pachymeningitis: a rare neurological syndrome with heterogeneous aetiology

Norbert Brüggemann1, Stefan Gottschalk, Konstanze Holl-Ulrich

  • 1Department of Neurology, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany.

Journal of Neurology, Neurosurgery, and Psychiatry
|February 27, 2010
PubMed

Insights

Cranial pachymeningitis, a condition causing leptomeningeal thickening, presents with diverse symptoms and MRI findings. This study highlights central nervous system vasculitis and rheumatoid arthritis as key causes in five patients.

Area of Science:

  • Neurology
  • Radiology
  • Pathology

Background:

  • Cranial pachymeningitis is an under-recognized syndrome characterized by leptomeningeal thickening and characteristic MRI enhancement.
  • Understanding its diverse clinical and etiological features is crucial for diagnosis and management.

Observation:

  • Five patients with focal and diffuse cranial pachymeningitis exhibited varied initial symptoms including headache, seizures, hemiparesis, ataxia, and confusion.
  • MRI revealed leptomeningeal enhancement and cortical swelling, with some cases showing hemorrhage.
  • Cerebrospinal fluid analysis often indicated lymphomonocytic pleocytosis, while infectious causes were ruled out.

Findings:

  • Leptomeningeal biopsies in two patients demonstrated perivascular inflammation, suggesting central nervous system vasculitis.
  • The identified causes of pachymeningitis included primary central nervous system vasculitis (2 cases) and rheumatoid arthritis (2 cases).
  • One case remained idiopathic.

Implications:

  • This study underscores the importance of considering central nervous system vasculitis and rheumatoid arthritis in the differential diagnosis of cranial pachymeningitis.
  • Further research is needed to elucidate the pathophysiology and identify novel therapeutic targets for this rare condition.
  • Improved diagnostic strategies may enhance early detection and intervention, potentially improving patient outcomes.

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