Leptomeningeal form of Immunoglobulin G4-related hypertrophic meningitis with perivascular spread: a case report and review of the literature

Abstract

PurposeImmunoglobulin G4 (IgG4)-related disease represents a spectrum of fibro-inflammatory disorders that affects various organ systems, including the central nervous system.MethodsHere we present the case of lgG4-related hypertrophic meningitis with exclusively leptomeningeal involvement and spread via perivascular spaces.ResultsA 58-year-old male patient presented with complex partial seizures. Initial computed tomography examination showed left frontal sulcal hyperdensity. Subsequent magnetic resonance examination revealed FLAIR hyperintensity in the central sulcus, with post-contrast enhancement in the form of “dotted line.” Physical examination, routine laboratory, and cerebrospinal fluid analyses were unremarkable. Meningeal biopsy confirmed IgG4-related meningitis. After corticosteroid treatment, a complete resolution of imaging findings was observed. Two months later, the patient presented with relapsing neurological symptoms and radiological findings in postcentral, precentral, and temporal sulci, resembling the form of “dotted line” contrast enhancement. In addition, linear intraparenchymal enhancement that followed perivascular spaces was seen in the left parietal lobe. After repeated steroid therapy, all lesions resolved completely.ConclusionWe reported the first case of isolated IgG4-related leptomeningeal involvement with a “dotted line” enhancement and perivascular intraparenchymal spread. Although IgG4-related meningitis represents a rare disease, both clinicians and radiologists should include this condition in the differential diagnosis of unclear leptomeningeal disease.

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

“…Three steroid-refractory HP patients treated with RTX for 4 weeks showed clinical improvements and exhibited prominent decreases in dural thickness (61). Thus, RTX has been suggested to be a second-line therapy for steroid-refractory HP, especially for IgG4-RD (52)(53)(54)(55)(56)(57)(58)(59)(60)(61)(62)(63)(64)(65).…”

“…Necrotizing vasculitis of small arteries located in the dura and cerebral surfaces has also been reported ( 12 ). In our study, only one patient underwent a biopsy, which was mainly conducted to confirm the diagnosis of IgG4-RD ( 54 – 56 ). In fact, the biopsy rates were low in both China and other countries because of the potential risks of the procedure.…”

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

“…Necrotizing vasculitis of small arteries located in the dura and cerebral surfaces has also been reported [12]. In our study, only one patient underwent a biopsy, which was mainly conducted to confirm the diagnosis of IgG4-RD [48][49][50]. In fact, the biopsy rates were low both in China and other countries because of the potential risks of the procedure.…”

Chronic Meningitis

“…132 Leptomeningeal involvement has also been reported but is rarer than IgG4 infiltration of the dura mater. 135 Patients most commonly present with headache and multiple cranial neuropathies from mechanical compression. 134 All patients should have their serum IgG 4 level checked, which is elevated in multisystem disease, although if CNS disease occurs in isolation, the serum level may be normal.…”