Cranial pachymeningitis: a rare neurological syndrome with heterogeneous etiology

Norbert Brüggemann (1) , Stefan Gottschalk (2) , Konstanze Holl-Ulrich (3) , Jan Stewen (4) , Wolfgang Heide (4) , Günter Seidel (1)

1 Department of Neurology, University of Luebeck
2 Institute of Neuroradiology, University of Luebeck
3 Institute of Pathology, University of Luebeck
4 Department of Neurology, General Hospital Celle

Cranial pachymeningitis is a poorly understood syndrome defined by leptomeningeal thickening and typical Gadolinium-enhanced magnetic resonance imaging (MRI). We present the heterogeneous clinical and aetiological features of five patients with both focal and diffuse pachymeningitis. 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.

Mots clés: INFECTIOUS DISEASES; MRI; TUBERCULOSIS; VASCULITIS; central nervous system vasculitis; leptomeningeal enhancement; leptomeningeal inflammation; pachymeningitis; rheumatoid arthritis

Norbert Brüggemann, Stefan Gottschalk, Konstanze Holl-Ulrich, Jan Stewen, Wolfgang Heide, et al.. Cranial pachymeningitis: a rare neurological syndrome with heterogeneous etiology. Journal of Neurology, Neurosurgery and Psychiatry, 2010, 81 (3), pp.294. ⟨10.1136/jnnp.2008.160457⟩. ⟨hal-00552722⟩