J Rheum Dis.  2015 Jun;22(3):190-194. 10.4078/jrd.2015.22.3.190.

A Case of Eosinophilic Granulomatosis with Polyangiitis Presenting with Subarachnoid Hemorrhage and Mononeuritis Multiplex

Affiliations
  • 1Division of Rheumatology, Department of Internal Medicine, Inha University School of Medicine, Incheon, Korea. khjung@inha.ac.kr
  • 2Department of Radiology, Inha University School of Medicine, Incheon, Korea.
  • 3Department of Neurosurgery, Inha University School of Medicine, Incheon, Korea.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss syndrome, is an anti-neutrophil 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.

Keyword

Churg-Strauss syndrome; Subarachnoid hemorrhage; Mononeuropathies

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Asthma
Central Nervous System
Churg-Strauss Syndrome
Cyclophosphamide
Eosinophils*
Female
Humans
Immunoglobulins
Middle Aged
Mononeuropathies*
Mortality
Neck Pain
Peripheral Nervous System Diseases
Sinusitis
Subarachnoid Hemorrhage*
Vasculitis
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide
Immunoglobulins

Figure

  • 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).

  • 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).

  • 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).


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