Neurosurgery and Neuroscience

Objectives: Central nervous system (CNS) involvement in IgG4-related disease (IgG4-RD) is a relatively recent discovery, first reported in 2006. The disease can present a diagnostic challenge, as the clinical and neurological findings can mimic multiple oncological and inflammatory conditions. Here, we review our center’s experience of IgG4-RD.

 

Design: A retrospective study of patients presenting to our centre between 2013 and 2018.

 

Subjects: All patients with a histopathologically suspected diagnosis of IgG4-RD.

 

Methods: Review of our center’s neuropathology database.

 

Results: We identified 10 patients (5 males and 5 females, age range: 26-73 years) with a histopathological diagnosis of IgG4-RD. Median follow-up from diagnosis was 36 months. All patients presented with CNS manifestation of

IgG4-RD, and none had any manifestations of the disease. Two patients presented with cranial or peripheral nerve involvement and seizures, and the remaining 8 with clinical evidence of hypophysitis. All had biochemical evidence of panhypopituitarism at the time of presentation, and none had visual impairment. All patients underwent biopsy/ debulking) and five were subsequently treated with steroids/immunosuppressants. At last follow-up, all were alive.

 

Conclusions: IgG4-RD is a rare cause of neurological and endocrine dysfunction and requires individualized management. Once confirmation of the diagnosis has been obtained, some patients can be managed conservatively, although some require treatment with steroids and immunosuppressants, for example rituximab.