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CASE REPORT
Rapidly progressive global cerebral atrophy in the setting of anti-LGI1 encephalitis
  1. Anudeep Yelam1,
  2. Elanagan Nagarajan2 and
  3. Pradeep C Bollu1
  1. 1 Department of Neurology, University of Missouri Health Care, Columbia, Missouri, USA
  2. 2 Department of Neurology, Howard Hughes Medical Institute – University of Missouri Columbia School of Medicine, Columbia, Missouri, USA
  1. Correspondence to Dr Elanagan Nagarajan, nagarajane{at}health.missouri.edu

Abstract

A 47-year-old man presented with complaints of breakthrough seizures, psychiatric and behavioural changes and catatonic features. MRI of the brain showed mild cerebral and right hippocampal atrophy, while the electroencephalogram showed intermittent right temporal slowing. With a presumed diagnosis of autoimmune encephalitis, he was treated with intravenous immunoglobulin (IVIG) and methylprednisolone, which significantly improved the symptoms. Serological testing later was positive for antileucine-rich glioma inactivated 1 antibody. Two months after the initial presentation, patient had a relapse of the symptoms without any further episodes of seizures. Repeat MRI of the brain showed a significant rapidly progressive diffuse cortical atrophy and hippocampal atrophy, more prominent on the right side along with hydrocephalus ex vacuo when compared with the initial MRI. He is currently on monthly IVIG therapy. At 4 months follow-up from the second imagining study, the patient had persistent MRI findings.

  • epilepsy and seizures
  • hydrocephalus
  • neuro genetics
  • neuroimaging
  • immunology

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Footnotes

  • AY and EN contributed equally.

  • Contributors AY and EN conceived the original idea for the manuscript, drafting and revision of the manuscript. Both authors contributed equally to this manuscript. PCB revised the manuscript for intellectual content.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.