Queen Elizabeth University Hospital, UK
Scientific Tracks Abstracts: J Neurol Neurophysiol
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 centerÔ??s experience of
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 centerÔ??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.
Samih Hassan has completed his neurosurgical training in London between St Georges Hospital and the National Hospital of Neurology and Neurosurgery; Queen Square. He then did a fellow ship in pituitary surgery and neuroendoscopy at Queen Square Hospital. He is currently a Consultant Neurosurgeon at Queen Elizabeth University Hospital in Glasgow.
E-mail: [email protected]