IgG4 Disease-Related Ataxia

Cerebellum. 2024 Jun;23(3):1231-1234. doi: 10.1007/s12311-023-01592-8. Epub 2023 Aug 9.

Abstract

We describe a male patient presenting with cerebellar ataxia and behavioural frontotemporal dementia in whom imaging showed cerebellar atrophy. He had significantly low N-acetyl aspartate to creatine (NAA/Cr) area ratio on MR spectroscopy of the cerebellum, primarily affecting the vermis. CT body scan showed extensive abnormal tissue within the mesentery, the retroperitoneum and perinephric areas. PET-CT showed increased tracer uptake within the wall of the aorta suggestive of an aortitis and within the perinephric tissue bilaterally. Biopsy of the perinephric tissue confirmed IgG4 disease. Treatment with steroids and mycophenolate improved his clinical state, but he developed symptoms attributed to pericardiac effusion that necessitated treatment initially with drainage and subsequently with pericardial window. After a course of rituximab, he had an episode of sepsis that did not respond to appropriate treatment and died as a result. Both the imaging findings and neurological presentation with cerebellar ataxia and behavioural frontotemporal dementia are novel in the context of IgG4 disease.

Keywords: Behavioural frontotemporal dementia; Cerebellar ataxia; IgG4 disease; Large vessel vasculitis.

Publication types

  • Case Reports

MeSH terms

  • Cerebellar Ataxia* / diagnostic imaging
  • Cerebellar Ataxia* / immunology
  • Cerebellar Ataxia* / pathology
  • Frontotemporal Dementia / diagnostic imaging
  • Frontotemporal Dementia / pathology
  • Humans
  • Immunoglobulin G4-Related Disease* / complications
  • Immunoglobulin G4-Related Disease* / diagnostic imaging
  • Immunoglobulin G4-Related Disease* / pathology
  • Male
  • Middle Aged