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

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meningitis 83 Title: NeuroradiologyLeptomeningeal form of Immunoglobulin G4-related hypertrophic meningitis with perivascular spread: a case report and review of the literatureJasmina BobanSelin ArdalıMajda
meningitis 548 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
meningitis 1088 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
meningitis 1775 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
meningitis 2563 nervous system (CNS) involvement is relatively rare and mostly seen as hypophysitis. Hypertrophic pachy meningitis is a focal or diffuse thickening of the intracranial and/or spinal meninges and is a recently recognized
meningitis 2751 and is a recently recognized part of the IgG4-related disease spectrum [[3], [4]]. Hypertrophic pachy meningitis can be observed in a wide range of diseases, including malignant, immunological, infectious, and vasculitic
meningitis 2989 also be idiopathic. Many of the cases previously described as idiopathic hypertrophic cranial pachy meningitis (IHCPM) might actually belong to the IgG4-related disease spectrum [[5]].To date, several cases of pachymeningitis—but
meningitis 3104 (IHCPM) might actually belong to the IgG4-related disease spectrum [[5]].To date, several cases of pachy meningitis —but only three cases of leptomeningeal involvement in IgG4-related disease have been described [[6]].
meningitis 3438 spread has been described. Here, we present the case of histologically proven lgG4-related hypertrophic meningitis with exclusively leptomeningeal involvement and spread via the perivascular spaces.Case presentationA
meningitis 5061 positive for IgG4 antibodies on immunohistochemistry, thus indicating the diagnosis of IgG4-related meningitis . Serum IgG and IgG4 levels were within normal range. The workup for immunologic diseases and malignancy
meningitis 7749 the orbit, salivary glands, lymph nodes, lungs, kidneys, and other organs [[2]]. Hypertrophic pachy meningitis is a rare neurologic manifestation of the IgG4-related disease spectrum characterized by localized or
meningitis 7939 characterized by localized or diffuse thickening of the dura mater [[3], [4]]. Recently, IgG4-related pachy meningitis has been suggested to represent some of the cases that were previously diagnosed as idiopathic hypertrophic
meningitis 8071 represent some of the cases that were previously diagnosed as idiopathic hypertrophic cranial pachy meningitis (IHCPM) [[5]].IgG4-related pachymeningitis generally occurs in men during the fifth and sixth decades.
meningitis 8114 previously diagnosed as idiopathic hypertrophic cranial pachymeningitis (IHCPM) [[5]].IgG4-related pachy meningitis generally occurs in men during the fifth and sixth decades. Typical clinical symptoms include headache,
meningitis 8423 neuropathies, vessel occlusions, and dural venous sinus occlusions [[6]]. On CT/MR imaging, hypertrophic pachy meningitis is characterized by diffuse or mass-like thickening of the dura over the cerebral hemispheres and/or
meningitis 8736 described [[9]].To date, to the best of our knowledge, no cases of isolated leptomeningeal IgG4-related meningitis with perivascular spread have been described.In our patient, an isolated leptomeningeal involvement
meningitis 11555 establishing the diagnosis of IgG4-related disease [[15]]. Diagnostic criteria for IgG4-related pachy meningitis are based on three characteristic histological findings: lymphoplasmacytic infiltration of IgG4-positiveplasma
meningitis 12747 have implied moderate to severe damage to the blood-brain barrier, as in other forms of inflammatory meningitis . A higher elevation of CSF IgG4 levels in patients with IgG4-related pachymeningitis when compared with
meningitis 12832 of inflammatory meningitis. A higher elevation of CSF IgG4 levels in patients with IgG4-related pachy meningitis when compared with patients with infectious, neoplastic, and inflammatory meningitis has also been reported
meningitis 12917 IgG4-related pachymeningitis when compared with patients with infectious, neoplastic, and inflammatory meningitis has also been reported [[16]].Serum IgG4 level may or may not be elevated [[17]]. In our patient, and
meningitis 13214 within normal ranges.Although currently there is no consensus about the treatment of IgG4-related pachy meningitis , glucocorticoid therapy is the first choice. Recurrent disease after corticosteroid therapy rarely occurs.

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