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April 03, 2018; 90 (14) Video NeuroImages

Video NeuroImages: Paraneoplastic spinal myoclonus associated with Caspr2 antibodies

Harrison Hines, Nick M. Murray, Sarah Ahmad, Safwan Jaradeh, Carl A. Gold
First published April 2, 2018, DOI: https://doi.org/10.1212/WNL.0000000000005265
Harrison Hines
From Stanford University School of Medicine (H.H.); and Department of Neurology and Neurological Sciences (N.M.M., S.A., S.J., C.A.G.), Stanford University Medical Center, CA.
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Nick M. Murray
From Stanford University School of Medicine (H.H.); and Department of Neurology and Neurological Sciences (N.M.M., S.A., S.J., C.A.G.), Stanford University Medical Center, CA.
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Sarah Ahmad
From Stanford University School of Medicine (H.H.); and Department of Neurology and Neurological Sciences (N.M.M., S.A., S.J., C.A.G.), Stanford University Medical Center, CA.
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Safwan Jaradeh
From Stanford University School of Medicine (H.H.); and Department of Neurology and Neurological Sciences (N.M.M., S.A., S.J., C.A.G.), Stanford University Medical Center, CA.
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Carl A. Gold
From Stanford University School of Medicine (H.H.); and Department of Neurology and Neurological Sciences (N.M.M., S.A., S.J., C.A.G.), Stanford University Medical Center, CA.
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Citation
Video NeuroImages: Paraneoplastic spinal myoclonus associated with Caspr2 antibodies
Harrison Hines, Nick M. Murray, Sarah Ahmad, Safwan Jaradeh, Carl A. Gold
Neurology Apr 2018, 90 (14) 660-661; DOI: 10.1212/WNL.0000000000005265

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A 42-year-old man with thymoma-associated myasthenia gravis presented with 6 weeks of abnormal leg movements. Examination revealed myoclonus in the legs bilaterally (video, links.lww.com/WNL/A322). Chest CT showed recurrence of metastatic thymoma. MRI spine revealed nonspecific hemosiderin deposition at the T9 level without metastases or vascular malformation. EMG demonstrated right leg and rectus abdominus myoclonus up to T6, most prominently at T9-L1 (figure). Serum anti-contactin-associated protein-like 2 (Caspr2) antibodies were positive. Chemotherapy led to resolution of the myoclonus. Caspr2 antibodies have been associated with limbic encephalitis and neuromyotonia,1,2 but our patient showed unusual Caspr2-associated spinal myoclonus.

From Stanford University School of Medicine (H.H.); and Department of Neurology and Neurological Sciences (N.M.M., S.A., S.J., C.A.G.), Stanford University Medical Center, CA.

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Figure EMG tracing from the T9 level of the right rectus abdominus

EMG demonstrates an unusual decrescendo firing pattern which, to our knowledge, has not been previously described. Myoclonic bursts varied between 600 milliseconds and 1.8 seconds in duration.

Author contributions

Harrison Hines: clinical patient care, acquisition of video data, drafting/revising the manuscript, accepts responsibility for conduct of research and final approval. Nick M. Murray and Sarah Ahmad: clinical patient care, critical revision of the manuscript. Safwan Jaradeh: clinical patient care, analysis of EMG studies. Carl A. Gold: clinical patient care, study supervision, critical revision of the manuscript.

Study funding

No targeted funding reported.

Disclosure

The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.

Acknowledgment

The authors thank Dr. Sarah Yang for help in obtaining and interpreting the EMG results.

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Video: links.lww.com/WNL/A322

  • © 2018 American Academy of Neurology

References

  1. 1.↵
    1. Van Sonderen A,
    2. Ariño H,
    3. Petit-pedrol M, et al
    . The clinical spectrum of Caspr2 antibody-associated disease. Neurology 2016;87:521–528.
    OpenUrlAbstract/FREE Full Text
  2. 2.↵
    1. Song J,
    2. Jing S,
    3. Quan C, et al
    . Isaacs syndrome with CASPR2 antibody: a series of three cases. J Clin Neurosci 2017;41:63–66.
    OpenUrl

Disputes & Debates: Rapid online correspondence

  • Tipton et al. response to author
    • Philip W. Tipton, Resident Physician, Mayo Clinic (Jacksonville, FL)
    • Jay A. van Gerpen, Physician, Mayo Clinic (Jacksonville, FL)
    • Robert Chen, Physician, University of Toronto
    Submitted July 12, 2018
  • Author response to Tipton et al.
    • Harrison Hines, Resident Physician, University of California, San Francisco Department of Neurology
    • Nick M. Murray, Resident Physician, Stanford University Medical Center, Department of Neurology and Neurological Sciences
    • Sarah Ahmad, Resident Physician, Stanford University Medical Center, Department of Neurology and Neurological Sciences
    • Safwan Jaradeh, Professor of Neurology, Stanford University Medical Center, Department of Neurology and Neurological Sciences
    • Carl A. Gold, Clinical Assistant Professor of Neurology and Neurological Sciencies, Stanford University Medical Center, Department of Neurology and Neurological Sciences
    Submitted June 19, 2018
  • Reader response: Paraneoplastic spinal myoclonus associated with Caspr2 antibodies
    • Philip W. Tipton, Neurology Resident, Mayo Clinic Florida
    • Jay A. van Gerpen, Neurologist, Mayo Clinic Florida
    • Robert Chen, Neurologist, University of Toronto
    Submitted May 24, 2018
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  • All Spinal Cord
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  • Myasthenia
  • Paraneoplastic syndrome
  • Myoclonus

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