Case report: PCA-2-associated encephalitis with different clinical phenotypes: a two-case series and literature review

Purkinje cell cytoplasmic antibody type 2 (PCA-2), identified in 2000, targets the widely distributed microtubule-associated protein 1B in the central and peripheral nervous systems, leading to diverse clinical phenotypes of neurological disorders. We report two cases of PCA-2-associated encephaliti...

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Bibliographic Details
Published inFrontiers in immunology Vol. 15; p. 1431585
Main Authors Li, Xiaona, Lang, Yue, Ma, Di, Bai, Jing, Shen, Pingping, Wang, Xinyu, Cui, Li
Format Journal Article
LanguageEnglish
Published Switzerland Frontiers Media S.A 12.07.2024
Subjects
Aged
Antigens, Neoplasm - immunology
Autoantibodies - blood
Autoantibodies - immunology
autoimmune cerebellitis
Encephalitis - diagnosis
Encephalitis - immunology
Female
Humans
limbic encephalitis
Limbic Encephalitis - diagnosis
Limbic Encephalitis - immunology
Magnetic Resonance Imaging
Male
MAP1B
Middle Aged
paraneoplastic neurologic syndrome
PCA-2
PET-CT
Phenotype
Positron Emission Tomography Computed Tomography
autoimmune cerebellitis
PET-CT
limbic encephalitis
PCA-2
MAP1B
paraneoplastic neurologic syndrome
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Summary:Purkinje cell cytoplasmic antibody type 2 (PCA-2), identified in 2000, targets the widely distributed microtubule-associated protein 1B in the central and peripheral nervous systems, leading to diverse clinical phenotypes of neurological disorders. We report two cases of PCA-2-associated encephalitis, each presenting with distinct onset forms and clinical manifestations, thereby illustrating the phenotypic variability of PCA-2-related diseases. The first patient was diagnosed with PCA-2-associated autoimmune cerebellitis and undifferentiated small cell carcinoma with metastasis in mediastinal lymph nodes of unknown primary origin. The second patient was diagnosed with PCA-2-associated limbic encephalitis. Our findings underscore the superior sensitivity of positron emission tomography-computed tomography over brain magnetic resonance imaging in the early detection of PCA-2-associated encephalitis. Given the high risk of relapse and suboptimal response to traditional immunotherapy in PCA-2-related neurological disorders, this study highlights the need for a deeper understanding of their pathogenesis to develop more effective treatments to control symptoms and improve patient prognosis.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
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ISSN:1664-3224
1664-3224
DOI:10.3389/fimmu.2024.1431585