Granulomatosis with polyangiitis presenting with pachymeningitis
ABSTRACT Observations: This case report describes a 75 year-old Caucasian male who initially presented with persistent daily headaches. Physical exam elicited multiple cranial nerve palsies. Gadolinium-enhanced brain MRI demonstrated pachymeningitis. Labs revealed elevated proteinase-3 antineutrophil cytoplasmic antibodies (PR3-ANCA) and cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), confirming a diagnosis of CPA. Conclusion: GPA should be considered in older patients presenting with persistent daily headaches and pachymeningitis. Contrast-enhanced brain MRI is an important diagnostic tool.
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ABSTRACT: A 27-year-old woman was admitted for intractable right-sided neck, ear, and jaw pain with gradual development of tinnitus and hearing loss. A cerebral MRI showed meningo-dural enhancement, and additional diagnostic workup revealed a right pulmonary infiltrate and positive PR-3 ANCA. Biopsies from nasal mucosa and lung showed chronic inflammation with granuloma formation. Based on these findings the patient was diagnosed with Wegener's granulomatosis with pachymeningitis. There was no clinical response to oral Prednisolone and Cyclophosphamide, but complete clinical and imaging remission was achieved by adding Rituximab.06/2011; 2011:608942. DOI:10.5402/2011/608942
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ABSTRACT: The structural organization of the dura and leptomeninges is reflected in its magnetic resonance (MR) imaging appearance in normal and disease states. Two distinct enhancement patterns are characterized: dura-arachnoid enhancement and pia-subarachnoid space enhancement. The dura-arachnoid pattern consists of curvilinear enhancement overlying the brain and immediately deep to the inner table of the calvaria, as well as along the falx and tentorium. Pial enhancement closely follows the brain surface into sulci and outlines the basal cisterns. Recognition of these enhancement patterns and other MR imaging characteristics may enhance the role of MR imaging in the detection, diagnosis, and follow-up of neoplastic and nonneoplastic disorders affecting the meninges. The focus of this article (Part I) is the MR appearance of the normal meninges and nonneoplastic causes of meningeal disease. Part II of this series will discuss neoplastic conditions affecting the coverings of the brain and spinal cord with an emphasis on pathways of disease spread.Radiology 12/1996; 201(2):297-308. DOI:10.1148/radiology.201.2.8888215 · 6.21 Impact Factor
Practical Neurology 03/2013; DOI:10.1136/practneurol-2012-000340