IgG4-related Disease Presenting With Ataxia
Abstract Background:Immunoglobulin G4 related disease is a relatively rare multi-system disorder which can present with diverse manifestations including mass-lesions and, or, organ dysfunction. Although the orbits, salivary glands and sinuses are comparatively common sites of involvement there are few reports of isolated intracranial presentation. Case presentation:Although large vessel IgG4-RD vasculitis is an uncommon entity we report a case of a 84-year old male presenting with cerebellar ataxia. CT at presentation demonstrated bilateral low-density change in the middle cerebellar peduncles, which corelated to areas of elevated T2 signal demonstrating restricted diffusion on MRI. CT angiogram demonstrated marked thickening of the common carotid arteries and branches extending to the skull base. In addition, the vertebral arteries also demonstrated significant wall thickening and occlusive narrowing. Serum immunoglobulin IgG and IgA were elevated and in particular IgG immunoglobulin was extremely high.Conclusions:Bilateral symmetrical restricted diffusion in the middle cerebellar peduncles is a striking radiological feature and reported to occur with multiple aetiologies, we believe this is the first report to associate this with IgG4-RD and ataxia.