{
  "abstract": "Background Immunoglobulin G 4 (IgG4) related disease (RD) is a multisystem, immunologically mediated disease discovered within the last two decades. Within the nervous system, a broad range of both central and peripheral nervous system involvement has been reported. We aimed to systematically review the neurological manifestations of IgG4-RD.Aim To identify the clinical presentation, radiological findings, diagnostic methods, treatment and outcomes for neurological manifestations of IgG 4-RD.Methods We systematically reviewed the literature to identify all reports of neurological manifestations of IgG 4-RD. Data on neurological manifestations, non-neurological manifestations, clinical presentation, radiological findings, diagnostic methods, treatment modalities and outcomes were extracted.Results We identified 393 cases from 297 publications meeting the inclusion criteria. Hypertrophic pachymeningitis, IgG 4-related orbital disease and hypophysitis are the most common neurological manifestations of IgG4-RD. Diagnostic evaluation involves testing for concomitant non-neurological manifestations, an MRI with gadolinium contrast, measurement of serum IgG4 levels and a biopsy with specific staining for IgG4 positive plasma cells. Treatment with corticosteroids leads to favourable outcomes.Conclusions IgG 4-RD is an emerging neurological disease that can manifest in multiple ways within the nervous system. It is important to recognise as treatment is often successful.",
  "authors": [
    {
      "affiliations": [
        "Neurology, Royal Hobart Hospital, Hobart, Tasmania, Australia"
      ],
      "name": "Yau Zane Justin Ng"
    },
    {
      "affiliations": [
        "Royal Hobart Hospital, Hobart, Tasmania, Australia",
        "Neurology, St Vincent’s Hospital Melbourne, Fitzroy, Victoria, Australia"
      ],
      "name": "Scarlett Bowen"
    },
    {
      "affiliations": [
        "Royal Hobart Hospital, Hobart, Tasmania, Australia",
        "Box Hill Hospital, Box Hill, Victoria, Australia"
      ],
      "name": "Jenna Phillips"
    },
    {
      "affiliations": [
        "Radiology, Royal Hobart Hospital, Hobart, Tasmania, Australia"
      ],
      "name": "Mugil Rajasekaran"
    },
    {
      "affiliations": [
        "Radiology, The Royal Melbourne Hospital, Melbourne, Victoria, Australia"
      ],
      "name": "Shi Sheng Lu"
    },
    {
      "affiliations": [
        "Menzies Research Institute, University of Tasmania, Hobart, Tasmania, Australia"
      ],
      "name": "Bruce V Taylor"
    }
  ],
  "title": "Neurological manifestations of immunoglobulin G4 related disease: a systematic review of 393 cases",
  "uid": "a805b862-35a8-52ee-a5c8-8f11bb217341"
}