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Immunotherapy in a case of low titre GAD65 antibody-associated spectrum neurological disorders
  1. Miao Wei,
  2. Firas Bannout,
  3. Mohammad Dastjerdi,
  4. Cattien Phan,
  5. Sanad Batarseh,
  6. Xiaofan Guo and
  7. Nancy Baker
  1. Neurology, Loma Linda University School of Medicine, Loma Linda, California, USA
  1. Correspondence to Dr Nancy Baker; NANBaker{at}llu.edu

Abstract

We present a rare case of low titre GAD65 antibody-associated autoimmune encephalitis and status epilepticus in a young woman. She initially presented with left arm dystonic movements, contractures and status epilepticus. Due to the concern of autoimmune encephalitis and seizures, the patient received intravenous immunoglobulin empirically. After the detection of low serum GAD65 antibodies, the patient underwent immunomodulation therapy with significant improvement. This case demonstrated that in autoimmune encephalitis, it is important to monitor serum GAD65 antibodies levels and consider immunotherapy, despite mildly elevated serum levels. The patient’s history of left arm dystonic movements without impaired awareness may have been due to limb dystonia, a presenting symptom of stiff person syndrome (SPS), despite SPS more commonly affecting axial muscles. This case further demonstrates that GAD65 antibody-related syndromes can manifest with different neurological phenotypes including co-occurrence of epilepsy with possible focal SPS despite low GAD65 antibodies titres.

  • Epilepsy and seizures
  • Neuroimaging
  • Immunology
  • Autoimmunity

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Footnotes

  • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content. MW and NB: conception and design, drafting and revising the article. MD and FB: acquisition of data, analysis and interpretation of data. CP, SB and XG: drafting and proofreading. The following authors gave final approval of the manuscript: MW, FB, MD, CP, SB, XG and NB.

  • 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.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

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