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- Title
IgG4 Disease-Related Ataxia.
- Authors
Hadjivassiliou, Marios; Blackburn, Daniel; O'Malley, Ronan; Hoggard, Nigel
- 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.
- Subjects
ATAXIA; CEREBELLAR ataxia; FRONTOTEMPORAL dementia; POSITRON emission tomography computed tomography; COMPUTED tomography; FRONTOTEMPORAL lobar degeneration; PERICARDIAL effusion
- Publication
Cerebellum, 2024, Vol 23, Issue 3, p1231
- ISSN
1473-4222
- Publication type
Case Study
- DOI
10.1007/s12311-023-01592-8