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Images in... Idiopathic cranial pachymeningitis – diabetes was not the brains Emmanuel Sagui, Arnaud Jouvion, Mathieu Planchard, Michel Bregigeon, Christian Brosset, Service de Neurologie, Hôpital d’Instruction des Armées Laveran, Marseille Cedex 13, France Correspondence to Emmanuel Sagui, [email protected] DESCRIPTION A 58-year-old diabetic woman presented with a 5-week his- tory of sudden-onset diplopia and progressive headache. One week before admission, she also complained of nau- sea. 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 haemo- globin 12.4%. Eosinophilia was normal. Lumbar puncture revealed pleocytosis with 8/mm 3 cells and protein at 170 mg/dl. MRI of the brain disclosed abnormal linear dural enhancement and T2 hyperintensity of dura mater, includ- ing falx and tentorium (figure 1). On clinical and MRI grounds, hypertrophic cranial pachy- meningitis (HCP) was diagnosed, and idiopathic HCP was ascertained after second-line investigations failed to ascer- tain 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. Lep- tomeningeal biopsy showed infiltrates of mature lympho- cytes, without evidence of granuloma, vasculitis or neoplastic cells. HCP is a rare disorder due to local or diffuse inflamma- tion 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 con- ditions should be evoked: (1) infectious diseases, mainly tuberculosis, 2 syphilis, HTLV1, Lyme disease and fungal infections; (2) neoplasia, mainly lymphoma 3 and dural car- cinomatosis; (3) granulomatous diseases such as neurosar- coidosis or Wegener's disease 4 ; (4) vasculitis, either primary 5 or secondary to rheumatoid arthritis or temporal arteritis. Idiopathic HCP is ascertained after these diagnoses are ruled out. 1 of 3 BMJ Case Reports 2010; doi:10.1136/bcr.11.2009.2508 on 10 February 2021 by guest. Protected by copyright. http://casereports.bmj.com/ BMJ Case Reports: first published as 10.1136/bcr.11.2009.2508 on 10 August 2010. Downloaded from
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Idiopathic cranial pachymeningitis – diabetes was not thebrains
Emmanuel Sagui, Arnaud Jouvion, Mathieu Planchard, Michel Bregigeon, Christian Brosset,
Service de Neurologie, Hôpital d’Instruction des Armées Laveran, Marseille Cedex 13, France
Correspondence to Emmanuel Sagui, [email protected]
DESCRIPTIONA 58-year-old diabetic woman presented with a 5-week his-tory of sudden-onset diplopia and progressive headache.One week before admission, she also complained of nau-sea. She reported no history of allergic rhinitis or arthritis.Physical examination revealed isolated left-cranial-nerve VIpalsy. Routine biological tests were unremarkable, apartfrom hyperglycaemia 300 mg/dl and glycosylated haemo-globin 12.4%. Eosinophilia was normal. Lumbar puncturerevealed pleocytosis with 8/mm3 cells and protein at170 mg/dl.
MRI of the brain disclosed abnormal linear duralenhancement and T2 hyperintensity of dura mater, includ-ing falx and tentorium (figure 1).
On clinical and MRI grounds, hypertrophic cranial pachy-meningitis (HCP) was diagnosed, and idiopathic HCP wasascertained after second-line investigations failed to ascer-tain a known aetiology: serological tests to syphilis andLyme disease were negative; all cerebrospinal fluid stainsand cultures to fungi and Mycobacterium tuberculosis were
negative; chest CT was normal; c-ANCA were negative;minor salivary glands biopsy was normal; biopsy of theright superficial temporal artery revealed no arteritis. Lep-tomeningeal biopsy showed infiltrates of mature lympho-cytes, without evidence of granuloma, vasculitis orneoplastic cells.
HCP is a rare disorder due to local or diffuse inflamma-tion of the dura mater with abnormal dural enhancementrevealed on MRI.1 Aetiological diagnosis of HCP is still achallenge. Once intracranial hypotension is ruled out on thebasis of clinical features – orthostatic headache – four con-ditions should be evoked: (1) infectious diseases, mainlytuberculosis,2 syphilis, HTLV1, Lyme disease and fungalinfections; (2) neoplasia, mainly lymphoma3 and dural car-cinomatosis; (3) granulomatous diseases such as neurosar-coidosis or Wegener's disease4; (4) vasculitis, eitherprimary5 or secondary to rheumatoid arthritis or temporalarteritis.
Idiopathic HCP is ascertained after these diagnoses areruled out.
1 of 3BMJ Case Reports 2010; doi:10.1136/bcr.11.2009.2508
on 10 February 2021 by guest. P
rotected by copyright.http://casereports.bm
j.com/
BM
J Case R
eports: first published as 10.1136/bcr.11.2009.2508 on 10 August 2010. D
ownloaded from
Competing interests None.
Patient consent Obtained.
REFERENCES1. Kupersmith MJ, Martin V, Heller G, et al. Idiopathic hypertrophic
pachymeningitis. Neurology 2004;62:686–94.2. Thurtell MJ, Keed AB, Yan M, et al. Tuberculous cranial pachymeningitis.
Neurology 2007;68:298–300.
3. Hochberg FH, Miller DC. Primary central nervous system lymphoma.J Neurosurg 1988;68:835–53.
4. D rr J, Elitok S, Dieste FJ, et al. Treatment-resistant chronic headaches andfocal pachymeningitis in a 46-year-old man: a rare presentation of Wegener’sgranulomatosis. Lancet Neurol 2008;7:368–72.
5. Salvarani C, Brown RD, Jr Calamia KT, et al. Primary central nervous systemvasculitis with prominent leptomeningeal enhancement: a subset with abenign outcome. Arthritis Rheum 2008;58:595–603.
Figure 1 T1 MRI: abnormal dural enhancement of dura mater.
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Sagui E, Jouvion A, Planchard M, Bregigeon M, Brosset C. Idiopathic cranial pachymeningitis – diabetes was not the brains. BMJ Case Reports 2010;10.1136/bcr.11.2009.2508, date of publication
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eports: first published as 10.1136/bcr.11.2009.2508 on 10 August 2010. D
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