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June 2021; 11 (3) Research

CASPR2-Related Morvan Syndrome

Autonomic, Polysomnographic, and Neuropsychological Observations

Panda Sudha Swayang, Atchayaram Nalini, Veeramani Preethish-Kumar, Kaviraja Udupa, Ravi Yadav, Seena Vengalil, Sheikh Sultana Reshma, Kiran Polavarapu, Saraswati Nashi, T.N. Sathyaprabha, Priya Treesa Thomas, Bhat Maya, Rajeshwaran Jamuna, Anita Mahadevan, View ORCID ProfileM. Netravathi
First published October 6, 2020, DOI: https://doi.org/10.1212/CPJ.0000000000000978
Panda Sudha Swayang
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Atchayaram Nalini
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Veeramani Preethish-Kumar
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Kaviraja Udupa
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Ravi Yadav
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Seena Vengalil
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Sheikh Sultana Reshma
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Kiran Polavarapu
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Saraswati Nashi
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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T.N. Sathyaprabha
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Priya Treesa Thomas
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Bhat Maya
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Rajeshwaran Jamuna
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Anita Mahadevan
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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M. Netravathi
Departments of Neurology (PSS, AN, VP-K, KU, RY, SV, SSR, KP, SN, MN), Neurophysiology (TNS), Psychiatric Social Work (PTT), Neuroimaging & Interventional Neuroradiology (NIIR) (BM), Neuropsychology (RJ), and Neuropathology (AM), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.
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Citation
CASPR2-Related Morvan Syndrome
Autonomic, Polysomnographic, and Neuropsychological Observations
Panda Sudha Swayang, Atchayaram Nalini, Veeramani Preethish-Kumar, Kaviraja Udupa, Ravi Yadav, Seena Vengalil, Sheikh Sultana Reshma, Kiran Polavarapu, Saraswati Nashi, T.N. Sathyaprabha, Priya Treesa Thomas, Bhat Maya, Rajeshwaran Jamuna, Anita Mahadevan, M. Netravathi
Neurol Clin Pract Jun 2021, 11 (3) e267-e276; DOI: 10.1212/CPJ.0000000000000978

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Abstract

Objective Morvan syndrome is characterized by central, autonomic, and peripheral hyperexcitability due to contactin-associated protein 2 (CASPR2) antibody. Our objective was to study the clinical spectrum, electrophysiologic, autonomic, polysomnographic, and neuropsychological profile in patients with CASPR2–related Morvan syndrome.

Methods Serum and CSF samples that were CASPR2 antibody positive from 2016 to 2019 were assessed. Among them, patients with Morvan syndrome diagnosed based on clinical and electrophysiologic basis were included.

Results Fourteen (M:F = 10:4) patients with Morvan syndrome were included with age at onset of 37.1 ± 17.5 years. The clinical features were muscle twitching (12), insomnia (12), pain (11), paresthesias (9), hyperhidrosis (7), hypersalivation (6), double incontinence (3), spastic speech (2), dysphagia (2), behavioral disturbances (2), seizures (1), and cold intolerance (1). Neurologic examination revealed myokymia (12), hyperactive tendon reflexes (10), and tremor (6). EMG revealed neuromyotonia (12) and increased spontaneous activity (7). Autonomic function tests conducted in 8 patients revealed definite autonomic dysfunction (4), orthostatic hypotension (2), early dysfunction (1), and postural orthostatic tachycardia syndrome (1). Polysomnography findings in 6 patients revealed insomnia (3), absence of deep sleep (1), high-frequency beta activity (1), REM behavior disorder (1), and periodic leg movements (1). Neuropsychological evaluation showed subtle involvement of the left frontal and temporal lobe. Malignancy workup was negative. All patients were treated with steroids. There was complete neurologic resolution in follow-up with persistent neuropathic pain in 5 patients.

Conclusions This study has contributed to the growing knowledge on CASPR2-related Morvan syndrome. It is important for an increased awareness and early recognition as it is potentially treatable by immunotherapy.

Footnotes

  • Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp.

  • Received May 23, 2020.
  • Accepted August 7, 2020.
  • © 2021 American Academy of Neurology
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