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Volume 12
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10.3390/children12111513
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Review

Seronegative Immune-Mediated Cerebellar Ataxia in Children: Autoimmune Encephalitis Spectrum Disorder or a Distinct Entity?

by
Gontika Maria
1,2,*,
Tsimakidi Chrysanthi
1,
Salamou Eudokia
1,
Prattos Theofanis
1,
Kallias Nikolaos
1,
Kilidireas Constantinos
3,4,
Tzartos John
5,6 and
Gkougka Dionysia
1
1
Neurological Department, Penteli’s Children Hospital, 11528 Penteli, Greece
2
Medical School of National, Kapodistrian University of Athens, 11527 Athens, Greece
3
First Department of Neurology, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece
4
Department of Neurology, Henry Dunant Hospital Center, 11526 Athens, Greece
5
Second Department of Neurology, “Attikon” University Hospital, School of Medicine, National, Kapodistrian University of Athens, 12462 Athens, Greece
6
Tzartos NeuroDiagnostics, 11523 Athens, Greece
*
Author to whom correspondence should be addressed.
Children 2025, 12(11), 1513; https://doi.org/10.3390/children12111513 (registering DOI)
Submission received: 19 October 2025 / Revised: 3 November 2025 / Accepted: 7 November 2025 / Published: 8 November 2025
(This article belongs to the Special Issue Current Updates on the Diagnosis and Management of Immune-Mediated Neurological Disorders in Children)
Versions Notes

Abstract

Pediatric seronegative immune-mediated cerebellar ataxia (IMCA) remains a poorly defined and often under-recognized diagnosis, particularly in young children, where symptoms are frequently misattributed to self-limited post-infectious processes. We report the case of a 2.5-year-old girl who presented with acute-onset ataxia (mSARA score: 14). Cerebrospinal fluid analysis revealed pleocytosis and positive oligoclonal bands, while serial brain imaging and extensive autoantibody panels were unremarkable. However, indirect immunohistochemistry (TIIF/IHC) demonstrated a positive intracellular signal in cerebellar Purkinje cells, supporting the diagnosis of isolated seronegative IMCA. The patient showed sustained clinical improvement with prolonged corticosteroid therapy (mSARA score: 1). To date, only a few similar cases have been reported in the literature. It remains unclear whether these presentations fall within the spectrum of autoimmune encephalitis (AIE) or represent a distinct pediatric phenotype, potentially expanding the age range of primary autoimmune cerebellar ataxia previously described in adults. We recommend incorporating TIIF/IHC into the diagnostic workup of both isolated and combined pediatric cerebellar ataxia syndromes to support diagnosis and guide individualized treatment. Additionally, neurofilament light chain (NfL) and glial fibrillary acidic protein (GFAP) are emerging as promising biomarkers in this context and warrant further investigation.
Keywords: pediatric; seronegative; cerebellar ataxia; TIIF/IHC; biomarkers pediatric; seronegative; cerebellar ataxia; TIIF/IHC; biomarkers

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MDPI and ACS Style

Maria, G.; Chrysanthi, T.; Eudokia, S.; Theofanis, P.; Nikolaos, K.; Constantinos, K.; John, T.; Dionysia, G. Seronegative Immune-Mediated Cerebellar Ataxia in Children: Autoimmune Encephalitis Spectrum Disorder or a Distinct Entity? Children 2025, 12, 1513. https://doi.org/10.3390/children12111513

AMA Style

Maria G, Chrysanthi T, Eudokia S, Theofanis P, Nikolaos K, Constantinos K, John T, Dionysia G. Seronegative Immune-Mediated Cerebellar Ataxia in Children: Autoimmune Encephalitis Spectrum Disorder or a Distinct Entity? Children. 2025; 12(11):1513. https://doi.org/10.3390/children12111513

Chicago/Turabian Style

Maria, Gontika, Tsimakidi Chrysanthi, Salamou Eudokia, Prattos Theofanis, Kallias Nikolaos, Kilidireas Constantinos, Tzartos John, and Gkougka Dionysia. 2025. "Seronegative Immune-Mediated Cerebellar Ataxia in Children: Autoimmune Encephalitis Spectrum Disorder or a Distinct Entity?" Children 12, no. 11: 1513. https://doi.org/10.3390/children12111513

APA Style

Maria, G., Chrysanthi, T., Eudokia, S., Theofanis, P., Nikolaos, K., Constantinos, K., John, T., & Dionysia, G. (2025). Seronegative Immune-Mediated Cerebellar Ataxia in Children: Autoimmune Encephalitis Spectrum Disorder or a Distinct Entity? Children, 12(11), 1513. https://doi.org/10.3390/children12111513

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