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Breis et al. Neuroimmunol Neuroinflammation 2020;7:330-4 I http://dx.doi.org/10.20517/2347-8659.2020.14 Page 333
[19]
[4]
and generalized seizures are less frequent . Chandra et al. described 12 patients with anti-VGKC
encephalitis; all of them had cognitive decline, sleep disturbance and symptoms of panic, 9 had agitation
and 3 had faciobrachial dystonic seizures. Catatonia was observed in anti-NMDA related encephalitis, but
[19]
not in anti-VGKC .
Neuropathic pain is frequently found in patients with CASPR 2 antibodies, likely due to CASPR 2 large
[13]
expression in peripheral nervous system .
[13]
CASPR2 antibodies were positive in 10% of patients with idiopathic ataxia . Orthostatism and emotions
are triggers to episodic ataxia related to anti-CASPR2 antibodies .
[23]
First-line therapy consists in steroids, IV immunoglobulin and plasma exchange; while second line consists
in rituximab, cyclosporine, mycophenolate mofetil and cyclophosphamide. Immunotherapy leads to better
outcomes than no immune therapy .
[24]

In conclusion, the great variability of syndromes that can be caused by anti-CASPR2 antibodies, despite
its rarity, should be remembered in clinical practice as a differential diagnosis of diseases like limbic
encephalitis, epilepsy especially in older patients, new onset psychiatry symptoms, neuropathic pain,
idiopathic ataxia, because the immunotherapy has been associated with improved outcomes in patients.

DECLARATIONS
Authors’ contributions
Performed data acquisition and data analysis as well helping with the writing in the text: Breis LC,
Schlindwein MAM
Made substancial contribuitions to conception and design of the study, as well as provided technical
support: Gonçalves MVM

Availability of data and material
Not applicable.

Financial support and sponsorship
None.

Conflicts of interest
All authors declared that there are no conflicts of interest.

Ethical approval and consent to participate
Not applicable.

Consent for publication
Not applicable.

Copyright
© The Author(s) 2020.

REFERENCES
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family involved in several human diseases. Eur J Neurosci 2018;48:1906-23.
2. Arancibia-Carcamo IL, Attwell D. The node of Ranvier in CNS pathology. Acta Neuropathol 2014;128:161-75.