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

## TL;DR

This case report describes two patients with PCA-2-associated encephalitis showing different symptoms and highlights the importance of advanced imaging and better treatment strategies.

## Contribution

The study contributes two new cases of PCA-2-associated encephalitis with distinct clinical presentations and emphasizes the need for improved diagnostic and therapeutic approaches.

## Key findings

- PCA-2-associated encephalitis can present with diverse clinical phenotypes, such as autoimmune cerebellitis and limbic encephalitis.
- Positron emission tomography-computed tomography is more sensitive than MRI for early detection of PCA-2-related neurological disorders.
- PCA-2-related diseases often have a high risk of relapse and limited response to traditional immunotherapy.

## Abstract

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.