A Patient with Wegener&amp;rsquo;s Granulomatosis Presenting with a Subarachnoid Hemorrhage: Case Report and Review of CIMS Disease Associated with Wegener&amp;rsquo;s Granulomatosis

Cruz, Dinna N.; Segal, Aaron

doi:10.1159/000169095

Cited by 37 publications

(14 citation statements)

References 15 publications

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“…6,7,18) In the present case, systemic survey detected no extracranial nodules or other indicators for Wegener's granulomatosis, but we cannot exclude the possibility considering the occasional negative ANCA for a long time, unusual imaging features, occasional relapse after remission, and lack of specificity in the histological findings. 1,2) Therefore, long-term follow up is needed after the initial remission to detect possible relapse.…”

Section: Discussionmentioning

confidence: 50%

Isolated Angiitis in the Hypothalamus Mimicking Brain Tumor -Case Report-

Tsutsumi

Ito

Yasumoto

et al. 2008

Neurol. Med. Chir.(Tokyo)

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A 64-year-old female presented with exaggerating somnolence without contributory medical and lifestyle histories. She was not aware of any preceding infection or headache. Cerebral magnetic resonance imaging demonstrated an isolated enhanced mass in the hypothalamus without meningeal enhancement. Blood and cerebrospinal fluid examinations showed no significant findings except for hypernatremia and hyperprolactinemia. She underwent an open biopsy via the interhemispheric route. Histological examination revealed marked perivascular lymphocytic aggregation with polyclonal immunostaining both for B and T lymphocytes. No findings suggestive of underlying malignancy were recognized. Extensive work-up aiming at systemic vasculitis and lymphoma revealed no signs of extracranial lesion, so the most probable diagnosis was isolated angiitis in the hypothalamus. Angiitis may originate from the hypothalamus and should be considered in the differential diagnosis of hypothalamic lesion mimicking brain tumor on neuroimaging.

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Section: Discussionmentioning

confidence: 50%

Isolated Angiitis in the Hypothalamus Mimicking Brain Tumor -Case Report-

Tsutsumi

Ito

Yasumoto

et al. 2008

Neurol. Med. Chir.(Tokyo)

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“…The CT and MRI findings in our patients, all who had CNS and cranial nerve involvement, could be explained by three major mechanisms (vasculitis, granulomatous lesions resulting from contiguous invasion of nasal, paranasal or orbital cavities and granulomatous lesions in intracerebral tissue) that were proposed by Drachman to account for CNS disease in patients with GPA [22]. Although hemorrhagic stroke directly attributable to GPA is uncommon [22,23], fatal intracranial hemorrhage in several of our patients may have been related to advanced and widespread necrotizing vasculitis.…”

Section: Discussionmentioning

confidence: 88%

Wegener's Granulomatosis with Nervous System Involvement: A Hospital-Based Study

Huang

Lyu

et al. 2015

Eur Neurol

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Background: The aim of this study was to ascertain the clinical manifestations of granulomatosis with polyangiitis (Wegener's) (GPA) with the involvement of the peripheral nervous system (PNS) and central nervous system (CNS). Summary: All neurologic inpatients in a hospital over a 12-year period were reviewed. Nine patients met both the ACR 1990 traditional format criteria for the classification of GPA and the Chapel Hill nomenclature mandatory criteria for GPA. We focused on the clinical presentation, serological data, biopsy reports, disease activities [as assessed by the Birmingham Vasculitis Activity Score (BVAS)], electrophysiology, and brain images. Nine patients met the diagnostic criteria for GPA. The neurological signs of the initial manifestation of GPA were found in 6/9 (67%) patients. Eight patients had GPA-related CNS involvement, including four patients with chronic hypertrophic pachymeningitis, with either diffuse or focal thickening; three had intracranial hemorrhages and two had orbital mass lesions with optic nerve compression. In addition, six patients showed PNS involvement, including three with asymmetric sensorimotor polyneuropathy, two with symmetric sensorimotor polyneuropathy, and one with bilateral mononeuropathy. Key Messages: Neurological manifestation is not uncommon and can be the first clinical sign of GPA. The involvement of both CNS and PNS raises the possibility of GPA in hospitalized neurologic patients.

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“…Cerebral haemorrhage, in particular, is anecdotally reported [18,19], whereas ischemic cerebrovascular events secondary to vasculitis, seizures, diffuse encephalopathy, pituitary gland involvement, chronic hypertrophic pachymeningitis and cranial neuropathies are the most common manifestations [20,21].…”

Section: Discussionmentioning

confidence: 99%