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Hippocampal Neuronal Cultures to Detect and Study New Pathogenic Antibodies Involved in Autoimmune Encephalitis
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[Autoimmune limbic encephalitis with concurrent positivity for Caspr2 and GFAPα antibodies]

Akiko Hamamoto1, Tomohito Nakano1, Hisae Sumi1

  • 1Department of Neurology, Higashiosaka City Medical Center.

Rinsho Shinkeigaku = Clinical Neurology
|July 8, 2026
PubMed

Insights

This study reports a rare case of limbic encephalitis associated with contactin-associated protein 2 (Caspr2) antibodies, where glial fibrillary acidic protein alpha (GFAPα) antibodies likely contributed to white matter lesions. Immunotherapy showed significant clinical and radiological improvement.

Area of Science:

  • Neuroimmunology
  • Neurology
  • Autoimmune Encephalitis

Background:

  • Autoimmune limbic encephalitis is often associated with specific antibody targets.
  • Contactin-associated protein 2 (Caspr2) antibodies typically cause limbic encephalitis with medial temporal lobe involvement.
  • White matter lesions are an uncommon finding in Caspr2 antibody-associated encephalitis.

Purpose of the Study:

  • To describe a unique case of Caspr2 antibody-positive limbic encephalitis with prominent white matter involvement.
  • To investigate the potential role of glial fibrillary acidic protein alpha (GFAPα) antibodies in this presentation.
  • To evaluate the efficacy of immunotherapy in this rare condition.

Main Methods:

  • Clinical case presentation with detailed neurological examination.
  • Neuroimaging: Brain MRI and perfusion scintigraphy.
  • Electrophysiology: Electroencephalography (EEG).
  • Serological and cerebrospinal fluid (CSF) analysis for Caspr2 and GFAPα antibodies.

Main Results:

  • The patient presented with cognitive decline and status epilepticus.
  • MRI revealed abnormalities in medial temporal lobes and deep white matter with increased perfusion.
  • Positive Caspr2 antibodies in serum and CSF, and GFAPα antibodies in CSF.
  • Immunotherapy led to clinical and radiological improvement, including resolution of white matter lesions.

Conclusions:

  • This case highlights a rare presentation of Caspr2 antibody-positive limbic encephalitis with significant white matter lesions.
  • The presence of GFAPα antibodies may be associated with white matter involvement in this context.
  • Immunotherapy is effective in managing this complex autoimmune neurological disorder.
Keywords:
Caspr2 antibodyGFAPα antibodyautoimmune limbic encephalitis

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