Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss syndrome, is an antineutrophil cytoplasmic antibody associated vasculitis, accompanied by asthma, hypereosinophilia, nonfixed pulmonary infiltrates, and sinusitis. Peripheral neuropathy is common in patients with EGPA; however, a few cases of EGPA with central nervous system (CNS) involvement have been reported. A 45-year-old female referred for right side weakness and posterior neck pain was diagnosed as EGPA with subarachnoid hemorrhage and mononeuritis multiplex. She was effectively treated with a high dose glucocorticoid, cyclophosphamide, and intravenous immunoglobulin. EGPA with CNS involvement is uncommon and causes significant morbidity and mortality. Therefore more rapid and accurate diagnostic evaluation may be required. EGPA should be considered in patients with neurological symptoms and hypereosinophilia.
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Figure 1.
(A) Non-contrast-enhanced brain computed tomography image demonstrated subarachnoid hemorrhage in basal cistern, suprasellar cistern, perimesencephalic cistern and pre-pontine cistern. (B) Volume-ren-dered cerebral angiography image shows mild segmental stenosis at the P2 segment of left posterior cerebral artery (arrow).
![jrd-22-190f1.tif](/upload/SynapseXML/1010jrd/thumb/jrd-22-190f1.gif)
Figure 2.
This chest computed tomography shows diffuse ground glass opacity, interlobular septal thickening. Small nodular lesion was seen in peripheral left upper lobe and right middle lobe (arrow).
![jrd-22-190f2.tif](/upload/SynapseXML/1010jrd/thumb/jrd-22-190f2.gif)
Figure 3.
The progress report of this patient. Eosinophil count, antineutrophil cytoplasmic antibody (ANCA) titer and peripheral neuropathy are improved gradually after administration of high dose methylprednisolone, cylclophosphamide and intravenous immunoglobulin (IVIG).
![jrd-22-190f3.tif](/upload/SynapseXML/1010jrd/thumb/jrd-22-190f3.gif)
Table 1.
Summary of reports of subarachnoid hemorrhage in eosinophilic granulomatosis with polyangiitis patients