Meningeal Involvement in Wegener's Granulomatosis Confirmed and Monitored by Positive Circulating Antineutrophil Cytoplasm in Cerebrospinal Fluid

Abstract

MRI and CSF investigations revealed meningeal involvement in a 29-year-old patient with biopsy-confirmed Wegener's granulomatosis. The intracranial manifestation of Wegener's granulomatosis was supported by the detection of pathologic circulating antineutrophil cytoplasm (c-ANCA) in the CSF. We monitored disease activity by c-ANCA measurement in the CSF. After repeated cycles of intrathecal administration of methotrexate and corticoids, progression of meningeal infiltration stopped, and CSF c-ANCA titers became negative.

Wegener granulomatosis; multisystem involvement Wegenergranulomatozis; multisistem tutulumu

“…These may produce peripheral neuropathy or cerebral infarcts and hemorrages, dural thickening may be diffuse, nodular or plaque like (15). Meningeal involvement , seen as enhancement, is less common but is reported (14,16). In our case there was direct invasion from granuloma in the posterior ethmoidal cell, primary nodular frontal granulomatous lesion related to dura and intensive content enhancement in tentorium cerebelli secondary to dural granulomatous thickening.…”

“…Splenic infarcts secondary to vasculitis may occur in WG (15)(16)(17). In our case splenic infarcts were established with performing US and CT.…”

A Rare Case of Stroke in Wegener’s Granulomatosis

“…Hypertrophy of the meninges has also been described [8]. Extension of WG to involve the meninges from a previously involved area is extremely uncommon [9,10]. Chronic meningitis has several presentations including chronic headaches, cranial neuropathies, seizures, encephalopathy and meningism [10,11].…”

“…Extension of WG to involve the meninges from a previously involved area is extremely uncommon [9,10]. Chronic meningitis has several presentations including chronic headaches, cranial neuropathies, seizures, encephalopathy and meningism [10,11]. Headache was the most common presentation in almost all of these cases and may also be the sole presenting symptom [10].…”

“…Chronic meningitis has several presentations including chronic headaches, cranial neuropathies, seizures, encephalopathy and meningism [10,11]. Headache was the most common presentation in almost all of these cases and may also be the sole presenting symptom [10]. In summation, although CNS abnormalities in WG are uncommon, they must always be considered in known WG patients presenting with new-onset symptoms and also in patients presenting for the first time with a CNS complaint unresponsive to conventional medical therapy.…”