J Neurol Sci 2021 Mar 27:117420. Epub 2021 Mar 27.
Division of Neuro-Ophthalmology, Department of Ophthalmology, Massachusetts Eye & Ear Infirmary/Harvard Medical School, Boston, MA, USA; Department of Neurology, Massachusetts General Hospital/Harvard Medical School, Boston, MA, USA. Electronic address:
Purpose Of Review: IgG4-related disease (IgG4-RD) is emerging as a fibro-inflammatory entity affecting multiple organs, including manifold neurologic manifestations. This review discusses general characteristics of IgG4-RD neurologic disease including epidemiology, histology, clinical picture and treatment approaches.
Recent Findings: IgG4-RD is increasingly recognized as an important underlying pathophysiology in multiple disorders of neurologic interest, including orbital inflammation, infundibulo-hypophysitis, hypertrophic pachymeningitis, and even in rare cases CNS parenchymal disease and cranial vascular involvement. Read More