A rare case report on hypertrophic pachymeningitis: Serum IgG4-related disease

Abstract

IgG4-related disease (IgG4-RD) is an immune-mediated inflammatory condition of unknown etiology characterized by invasion of tissue by IgG4-producing plasma cells. It can affect almost any organ system, but central nervous system involvement is a rare occurrence. A careful clinicopathological correlation is required to establish the diagnosis. The condition is highly treatable with glucocorticoids, but it is likely that it is underdiagnosed. Although IgG4-related disease responds quickly to glucocorticoids, if left untreated, can lead to end-stage organ failure and even death. We present a case of a 46-year-old female patient who presented with headache, tingling, numbness, flickering movement in her left lower limb gradually extending to torso and head, and loss of consciousness. After radiological and immunohistochemical studies, the diagnosis of IgG4-related hypertrophic pachymeningtis was confirmed. Corticosteroid therapy was administered, and the patient symptomatically improved. Clinicians should be aware of this rare condition, and the importance of early diagnosis and appropriate corticosteroid therapy should be emphasized.

Keywords

• IgG4-related disease
• Hypertrophic pachymeningitis
• Methylprednisolone
• Autoimmune
• Antinuclear antibody