Description
A 58-year-old diabetic woman presented with a 5-week history of sudden-onset diplopia and progressive headache. One week before admission, she also complained of nausea. She reported no history of allergic rhinitis or arthritis. Physical examination revealed isolated left-cranial-nerve VI palsy. Routine biological tests were unremarkable, apart from hyperglycaemia 300 mg/dl and glycosylated haemoglobin 12.4%. Eosinophilia was normal. Lumbar puncture revealed pleocytosis with 8/mm3 cells and protein at 170 mg/dl.
MRI of the brain disclosed abnormal linear dural enhancement and T2 hyperintensity of dura mater, including falx and tentorium (figure 1).
Figure 1.
T1 MRI: abnormal dural enhancement of dura mater.
On clinical and MRI grounds, hypertrophic cranial pachymeningitis (HCP) was diagnosed, and idiopathic HCP was ascertained after second-line investigations failed to ascertain a known aetiology: serological tests to syphilis and Lyme disease were negative; all cerebrospinal fluid stains and cultures to fungi and Mycobacterium tuberculosis were negative; chest CT was normal; c-ANCA were negative; minor salivary glands biopsy was normal; biopsy of the right superficial temporal artery revealed no arteritis. Leptomeningeal biopsy showed infiltrates of mature lymphocytes, without evidence of granuloma, vasculitis or neoplastic cells.
HCP is a rare disorder due to local or diffuse inflammation of the dura mater with abnormal dural enhancement revealed on MRI.1 Aetiological diagnosis of HCP is still a challenge. Once intracranial hypotension is ruled out on the basis of clinical features – orthostatic headache – four conditions should be evoked: (1) infectious diseases, mainly tuberculosis,2 syphilis, HTLV1, Lyme disease and fungal infections; (2) neoplasia, mainly lymphoma3 and dural carcinomatosis; (3) granulomatous diseases such as neurosarcoidosis or Wegener's disease4; (4) vasculitis, either primary5 or secondary to rheumatoid arthritis or temporal arteritis.
Idiopathic HCP is ascertained after these diagnoses are ruled out.
Footnotes
Competing interests None.
Patient consent Obtained.
References
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