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Autoimmune and limbic encephalitis: case series with some atypical variables in clinical practice

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Abstract

Autoimmune and limbic encephalitides are still rare diseases characterized by rapid diagnostics and treatment development in recent years. The incidence of anti-N-methyl-D- aspartate receptor [NMDAR] encephalitis is about 1.5 per million person per year, and the incidence of paraneoplastic neurological syndromes [PNS] including limbic encephalitis [LE] is about 1.22 per 100 000 person per year (Vogrig et al. J Neurol 267:26–35, 1; Dalmau et al. Ann Neurol 61:25–36, 2). The diagnostic criteria of anti-NMDAR encephalitis are already well established (Zuliani et al. Neurol Sci 40:2017–2030, 10). We provide immunological and clinical characteristics of anti-NMDAR encephalitis case series emphasizing unusual association with colon tumour in one case and complete recovery in two cases. Then we report two cases of onconeural and cell surface antibody negative limbic encephalitis [LE] associated with tumours, seizures, cognitive and behavioural changes resulting in severe cerebellar syndrome and fatal outcome. The clinical characteristics and results of selected paraclinical examinations as electroencephalography [EEG], magnetic resonance imaging [MRI] and cerebrospinal fluid [CSF] analysis are reviewed.

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Abbreviations

CSF:

Cerebrospinal fluid

MRI:

Magnetic resonance imaging

EEG:

Electroencephalography

IVIG:

Intravenous immunoglobulins

PE:

Plasma exchange

CLL:

Chronic lymphatic leukemia

RS:

Rankin scale

NMDAR:

N-methyl-D-aspartate-receptor

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Funding

This work was supported by the Ministry of Health of the Czech Republic, ref.-RVO (FNBr,65269705).

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Authors and Affiliations

  1. Department of Neurology, University Hospital Brno and Faculty of Medicine, Masaryk University, Jihlavská 340/20, 625 00, Brno, Czech Republic

    Pavel Štourač

  2. Department of Clinical Microbiology, University Hospital Brno and Faculty of Medicine, Masaryk University, Jihlavská 340/20, 625 00, Brno, Czech Republic

    Jana Bednářová

  3. Department of Neurology, General Hospital, J. E. Purkyně 365, 686 68, Uherské Hradiště, Czech Republic

    Pavel Zicháček

  4. Department of Clinical Biochemistry, University Hospital Brno and Faculty of Medicine, Masaryk University, Jihlavská 340/20, 625 00, Brno, Czech Republic

    Zdena Čermáková

  5. Department of Neurology, University Hospital Hradec Králové and Faculty of Medicine, Charles University, Sokolská 581, 500 05, Hradec Králové, Czech Republic

    Zbyšek Pavelek & Martin Vališ

Authors
  1. Pavel Štourač
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  2. Jana Bednářová
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  3. Pavel Zicháček
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  4. Zdena Čermáková
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  5. Zbyšek Pavelek
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  6. Martin Vališ
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Corresponding author

Correspondence to Pavel Štourač.

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The project was approved by Ethical Committee of University Hospital Brno, Czech Republic.

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Štourač, P., Bednářová, J., Zicháček, P. et al. Autoimmune and limbic encephalitis: case series with some atypical variables in clinical practice. Neurol Sci 43, 687–690 (2022). https://doi.org/10.1007/s10072-021-05563-x

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  • DOI: https://doi.org/10.1007/s10072-021-05563-x

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