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High prevalence of electrocardiac abnormalities in anti-CASPR2 antibody-associated disease
  1. Qing Liu1,2,
  2. Jun Ma1,
  3. Yan Huang1,
  4. Hai Tao Ren1,
  5. Qiang Lu1,
  6. Jun Yi Zhang1,
  7. Bin Peng1,
  8. Yu Zhou Guan1,
  9. Hong Zhi Guan1,
  10. Li Ying Cui1,2
  1. 1 Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Beijing, China
  2. 2 Neuroscience Center, Chinese Academy of Medical Sciences (CAMS), Beijing, China
  1. Correspondence to Dr Qing Liu, Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Beijing 100730, China; drliuqing{at}126.com and Dr Hong Zhi Guan, Department of Neurology, Peking Union Medical College Hospital, Beijing 100730, China; pumchghz{at}126.com

https://doi.org/10.1136/jnnp-2017-317527

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    Introduction

    Antibodies directed against contactin-associated protein-like 2 (CASPR2) have been identified in the sera of patients with peripheral and central neurological syndromes, including neuromyotonia, Morvan syndrome (MoS) and autoimmune limbic encephalitis (LE).1 MoS is characterised by peripheral nervous hyperactivity, autonomic dysfunction and LE. Electrocardiac abnormalities, mostly sinus tachycardia, are manifestation of cardiac autonomic dysfunction in patients with MoS.2 Here we describe our observation that electrocardiac abnormalities may present with high prevalence and evolve with the disease courses in CASPR2 antibody (Ab)-positive MoS.

    Methods

    We observed severe arrhythmia and cardiac arrest in one patient with CASPR2 Ab-positive MoS and subsequently conducted a retrospective analysis of hospitalised patients with anti-CASPR2 Ab-associated neurological disorders between November 2014 and January 2017 at Peking Union Medical College Hospital. Samples were tested using brain immunohistochemistry and cell-based assays in parallel for the determination of anti-CASPR2 Abs and other antibodies (including anti-N-methyl D-aspartate (NMDA) receptor, LGI1, GABAA receptor and GABAB receptor). Patients were considered to have anti-CASPR2 Abs if both tests were recorded as strongly positive in either of the samples. Written informed consent was obtained from all the patients or their supervisors due to psychiatric and cognitive malfunctions.

    Results

    Case 1

    A 58-year-old man presented with insomnia and sleepwalking for 2 years. One month before admission to our hospital, he complained of aches all over the body and developed visual and auditory hallucinations, urinary retention, constipation and weight loss (8 kg). The symptoms worsened with episodic anxiety and agitation, accompanied by muscle cramps. On physical …

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    Footnotes

    • Contributors QL and HZG designed the study. QL contributed to data analysis and drafted the manuscript. JM collected patients’ materials and contributed to manuscript preparation. YH provided the important result of case observation. LYC offered advice and financial support on antibody tests. HTR performed antibody tests. QL, QLu, JYZ, BP and YZG evaluated patients and provided clinical information.

    • Funding This study was supported by CAMS innovation Fund for Medical Sciences (CIFMS, 2016-12M-1-002) and the National Key Research and Development Program of China (2016YFC0905100).

    • Competing interests None declared.

    • Patient consent Parental/guardian consent obtained.

    • Ethics approval Institutional review board of Peking Union Medical College Hospital.

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

    • Correction notice Since this paper was first published online, Hong Zhi Guan (pumchghz@126.com) has been added as an additional corresponding author.