IgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. The diagnosis requires consideration of clinical, radiographic, serological, and pathological evidence. Neurological involvement by IgG4-RD is relatively uncommon and is being increasingly recognized and reported with hypertrophic pachymeningitis and hypophysitis as the most frequent manifestations. IgG4-related involvement of brain parenchyma is rare, but isolated case reports exist. Here, we report a case of a young boy who presented to us with bilateral optic neuropathy and bilateral hippocampal bleed-related neurological involvement in a possible IgG4-RD, a rare entity.
Keywords: IgG4-related disease; hippocampal bleed; optic neuropathy.