Clinical Characteristics and Predictors of Outcome for Onconeural Antibody-Associated Disorders: A Retrospective Analysis.

Shaohua Liao,Xiaoling Wang,Bing Niu,Huaiqiang Hu,Bingzhen Cao,Hongwei Guo,Shuai Miao,Ying Qian,Chuanfen Li

doi:10.3389/fneur.2017.00584

Abstract

To describe and analyze the clinical characteristics, laboratory data, management, and outcome of patients with onconeural antibody-associated disorders (OAAD) and identify predictors for poor outcome. This was a retrospective review of all patients with potential OAAD, who were hospitalized in Jinan General Hospital between September 2009 and July 2017. We clarified the diagnosis, collected comprehensive information and categorized patients into three groups: paraneoplastic neurological disorders (PNDs), autoimmune encephalitis (AE), and possible OAAD. Within the three groups, we analyzed a range of clinical and laboratory parameters and used univariate and multivariate regression analysis to identify predictors for poor outcome [modified Rankin Scale (mRS) = 3-6]. From 158 patients, we identified 70 who fulfilled the criteria for OAAD, including 44 men (62.9%) and 26 women (37.1%). There were 38 patients (54.3%) in the PNDs group, 14 patients (20%) in the AE group, and 18 patients (25.7%) in the possible OAAD group. After the last follow-up, 14 (36.8%), 9 (64.2%), and 12 (66.7%) had a good outcome (mRS = 0-2). However, 6 (15.8%), 2 (14.3%), and 3 (16.7%) died, respectively. Univariate analysis showed that duration prior to the hospital (p = 0.0224) and urinary incontinence/retention (p = 0.0043) were associated with poor outcome (mRS = 3-6). After multivariate regression analysis, urinary incontinence/retention (p = 0.0388) and an immunocompromised state (p = 0.0247) remained as significant factors for poor outcome. Urinary incontinence/retention and an immunocompromised state represent significant predictors of a worse prognosis for patients with OAAD. By contrast, the results showed that [corrected] cerebrospinal fluid analysis, serum autoantibodies and tumor markers, [corrected] the function of crucial organs, electrophysiology, and radiological findings were not associated with a poor outcome.

Highlights

Onconeural antibody-associated disorders (OAADs) are a type of heteroplasmic neurological syndrome and are drawing increasing attention from neurologists
From a total of 158 patients, we identified 70 patients who fulfilled the criteria for OAAD, including 44 men (62.9%) and 26 women (37.1%) with a median age of 55 years
Univariate analysis showed that duration prior to the hospital (p = 0.0224) and urinary incontinence/retention (p = 0.0043) were factors associated with poor outcome

Summary

Introduction

Onconeural antibody-associated disorders (OAADs) are a type of heteroplasmic neurological syndrome and are drawing increasing attention from neurologists. OAADs can be broadly divided into two clinical categories: classic paraneoplastic neurological syndromes (PNDs) and autoimmune encephalitis (AE) [4]. Paraneoplastic neurological disorders are mostly accepted as neurological syndromes that are immune mediated and triggered when systemic tumors express certain neuronal antigens [3]. Previous reports have indicated that approximately 1 in 10,000 patients with cancer eventually develop PNDs [8], showing that this disease is associated with a low prevalence. Patients were preselected using clinical criteria and serological screening; these data showed that 163/649 (25%) cases were serologically positive for antibodies associated with PNDs during a 23-month period [4]. The predominant cohort of patients for AE includes those with anti-NMDA-receptor encephalitis (4%) and voltage-gated potassium channels (VGKC)-complex antibody positive encephalitis (3%) [9]

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Conclusion