Global brain atrophy and metabolic dysfunction in LGI1 encephalitis: A prospective multimodal MRI study

J Neurol Sci. 2017 May 15:376:159-165. doi: 10.1016/j.jns.2017.03.020. Epub 2017 Mar 16.

Abstract

Background: Chronic cognitive deficits are frequent in leucin-rich glioma-inactivated 1 protein (LGI1) encephalitis. We examined structural and metabolic brain abnormalities following LGI1 encephalitis and correlated findings with acute and follow-up clinical outcomes.

Methods: Nine patients underwent prospective multimodal 3 Tesla MRI 33.1±18months after disease onset, including automated volumetry, diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS). Data were compared to 9 age- and sex-matched healthy controls.

Results: Although extratemporal lesions were not present on MRI in the acute stage, tract-based spatial statistics analyses of DTI during follow-up showed widespread changes in the cerebral and cerebellar white matter (WM), most prominent in the anterior parts of the corona radiata, capsula interna and corpus callosum. MRS revealed lower glutamine/glutamate WM levels compared to controls. Higher cerebellar gray matter volume was associated with better function at disease onset (measured by the modified Rankin Scale), and higher putaminal volume was associated with better cognition by Addenbrooke's Cognitive Examination test at 23.4±7.6months.

Conclusions: Poor clinical outcome following LGI1 encephalitis is associated with global brain atrophy and disintegration of white matter tracts. The pathological changes affect not only temporomesial structures but also frontal lobes and the cerebellum.

Keywords: Anti-LGI1 antibody; Cognition; Diffusion tensor imaging; Limbic encephalitis; MR-spectroscopy; Volumetry.

MeSH terms

  • Acute Disease
  • Atrophy
  • Autoimmune Diseases of the Nervous System / diagnostic imaging*
  • Autoimmune Diseases of the Nervous System / drug therapy
  • Autoimmune Diseases of the Nervous System / metabolism
  • Brain / diagnostic imaging*
  • Brain / metabolism
  • Chronic Disease
  • Diffusion Tensor Imaging*
  • Encephalitis / diagnostic imaging*
  • Encephalitis / drug therapy
  • Encephalitis / immunology
  • Female
  • Follow-Up Studies
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Magnetic Resonance Imaging*
  • Magnetic Resonance Spectroscopy*
  • Male
  • Middle Aged
  • Multimodal Imaging
  • Organ Size
  • Prospective Studies
  • Proteins / immunology
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Intracellular Signaling Peptides and Proteins
  • LGI1 protein, human
  • Proteins