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.