Introduction
IgG4 related disease immunoglobulin G4 (IgG4)-related disease is recently recognized as a multi-systemic syndrome characterized by an immune-mediated fibroinflammatory condition with unique clinical, serological, and pathological features (1). It can involve any organ system and has the tendency to form multiple stupefactive lesions (1). It was first reported in the pancreas in early 2000 but gradually has been reported in other sites (2, 3). Pancreatic salivary glands, bile duct, and lacrimal glands are typically affected. The diagnosis of IgG4-RD requires the identification of characteristic findings upon biopsy of affected tissue which is interpreted in the context of clinical, serologic, and radiologic data. Ig4RD is a steroid-responsive disease and early recognition, and treatment is important because of the indolent nature of the condition and the risk of progression from an inflammatory stage to poorly responsive fibrotic disease and serious organ damage. Most patients experience disease flares during or after glucocorticoid tapers (4). Thus, additional immunosuppressive therapy may also be required.
Neurological presentation is less common and usually presents as hypophysitis followed by hypertrophic pachymeningitis (HP) (3). Hypertrophic pachymeningitis is a rare inflammatory disorder that causes localized or diffuse thickening of intracranial dura matter and has a broad range of neoplastic, chronic inflammatory, infectious, and hemorrhagic conditions associated with it and can therefore be a diagnostic challenge (1, 3). When no cause is found it is termed as idiopathic hypertrophic pachymeningitis. A dural biopsy is usually essential for a definitive diagnosis. Clinical manifestations of the disease are subject to localization or complications due to the compression and/or infiltration of neurologic and/or vascular adjacent structures. It has been suggested that IgG4-related sclerosing disease represents a subset of cases previously diagnosed as idiopathic hypertrophic pachymeningitis (5). Headache, cranial nerve palsy, and ataxia are the most common presentations (6). Seizures as chief presentations are uncommonly reported in the literature (5, 6).
Here we report highlights a unique, intricate case of a young male presented to our hospital with presentations imitating a variety of pathological conditions After undergoing a diagnostic workup, he was eventually diagnosed to have histologically proven Ig4RD systemic disease with meningeal involvement, which is a rare entity.