Article Text

PDF
Cranial pachymeningitis: a rare neurological syndrome with heterogeneous aetiology
  1. Norbert Brüggemann1,
  2. Stefan Gottschalk2,
  3. Konstanze Holl-Ulrich3,
  4. Jan Stewen4,
  5. Wolfgang Heide4,
  6. Gunter Seidel1
  1. 1Department of Neurology, University of Lübeck, Lübeck, Germany
  2. 2Institute of Neuroradiology, University of Lübeck, Lübeck, Germany
  3. 3Institute of Pathology, University of Lübeck, Lübeck, Germany
  4. 4Department of Neurology, General Hospital Celle, Celle, Germany
  1. Correspondence to Professor Seidel, Department of Neurology, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany; guenter.seidel{at}neuro.uni-luebeck.de

Abstract

Cranial pachymeningitis is a poorly understood syndrome, defined by leptomeningeal thickening and typical gadolinium enhanced MRI. The heterogeneous clinical and aetiological features of five patients with both focal and diffuse pachymeningitis are presented. The initial symptoms included headache (n=3), sensory Jackson seizures (n=1), hemiparesis (n=1), episodes of short lasting hemiataxia (n=1), hemihypaesthesia (n=1), aphasia (n=1) and confusion (n=2). MRI scans revealed focal (n=3) or diffuse (n=2) leptomeningeal gadolinium enhancement and cortical swelling (n=4). In addition, one case presented with a subarachnoid and a second with an intracerebral haemorraghe. CSF findings were variable and showed clear lymphomonocytic pleocytosis in 3/5 cases. Infectious diseases were extensively excluded in all cases. Leptomeningeal biopsies of two cases revealed perivascular inflammation, indicating central nervous system vasculitis. In the cases presented, pachymeningitis was caused by primary central nervous system vasculitis (n=2) and rheumatoid arthritis (n=2). In one case, the cause remained unclear.

  • Infectious diseases
  • MRI
  • tuberculosis
  • vasculitis
  • central nervous system vasculitis
  • leptomeningeal enhancement
  • leptomeningeal inflammation
  • pachymeningitis
  • rheumatoid arthritis

Statistics from Altmetric.com

Footnotes

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.