Clinical Characteristics and Outcomes of Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorder With Brainstem Lesions as Heralding Prodrome

Qiling Ji; Huiqing Dong; Hangil Lee; Zheng Liu; Yanna Tong; Kenneth Elkin; Yazeed Haddad; Xiaokun Geng; Yuchuan Ding

doi:10.3389/fneur.2022.836337

Clinical Characteristics and Outcomes of Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorder With Brainstem Lesions as Heralding Prodrome

Front Neurol. 2022 May 9:13:836337. doi: 10.3389/fneur.2022.836337. eCollection 2022.

Authors

Qiling Ji¹, Huiqing Dong², Hangil Lee³, Zheng Liu², Yanna Tong¹, Kenneth Elkin³, Yazeed Haddad³, Xiaokun Geng^{1

3}, Yuchuan Ding³

Affiliations

¹ Department of Neurology, Beijing Luhe Hospital, Capital Medical University, Beijing, China.
² Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China.
³ Department of Neurosurgery, Wayne State University School of Medicine, Detroit, MI, United States.

Abstract

Objective: The present study sought to differentiate multiple sclerosis and neuromyelitis optica spectrum disorder patients at their first attack by describing and distinguishing their clinical features, radiographic characteristics, and immunologic characteristics of serum and cerebrospinal fluid.

Methods: We retrospectively studied 58 patients with multiple sclerosis (MS) and 52 patients with neuromyelitis optica spectrum disorder (NMOSD) by referencing brainstem lesions as the prodromal events. Their demographics and presentation at the time of the first attack was evaluated including their gender, age, clinical features of the first attack, the expanded disability status scale (EDSS), brainstem lesion(s) by head MRI, and immunological indices of serum and cerebrospinal fluid.

Results: The NMOSD group had more female patients (4.8 vs. 1.9, p < 0.05), and was older than the MS group (37.81 ± 16.60 vs. 27.57 ± 11.17, p <0.001). NMOSD patients also had a significantly higher association with autoimmune diseases or positive autoimmune antibodies (p < 0.01). There was no significant difference in the EDSS scores between the two groups (p = 0.420). Central hiccups, vomiting, and pyramidal tract signs were more common in the NMOSD group than the MS group (p < 0.001, p < 0.001, p < 0.01), while eye movement abnormalities were more common with MS (p < 0.01). There were no significant differences in other clinical manifestations such as vertigo, diplopia, limb weakness, numbness, and eating difficulty. MS patients were more likely to have midbrain and pons imaging lesions (p < 0.001, p < 0.001), while NMOSD patients had more lesions in the medulla oblongata (p < 0.001). The lesions in the MS group were mostly located in the periphery, while those in the NMOSD group were centrally located (p < 0.001, p < 0.001). Patchy lesions were more common in MS patients (p < 0.001), while large lesions were more common in the NMOSD group (p < 0.001). Finally, serum AQP4 Ab was found only in the NMOSD group (p < 0.001).

Conclusion: Patients with MS and NMOSD have differentiating clinical manifestations at the time of their first brainstem lesions which include central hiccups, vomiting, pyramidal tract signs, and abnormal eye movements. Additionally, distinct imaging manifestations such as lesion location(s) and morphology may also aid in the development of pathognomonic criteria leading to timely initial diagnosis of MS and NMOSD.

Keywords: brainstem; demyelination; image characterization; multiple sclerosis; neuromyelitis optica spectrum disease.