Lucas Shoena, Lalive Patrice H, Lascano Agustina M
Faculty of Medicine, University of Geneva, Geneva, Switzerland.
Division of Neurology, Department of Clinical Neuroscience, Geneva University Hospitals, Geneva, Switzerland.
eNeurologicalSci. 2023 Jan 13;30:100443. doi: 10.1016/j.ensci.2023.100443. eCollection 2023 Mar.
Recently, an association between painful tonic spasms (PTS) and Neuromyelitis Optica Spectrum Disorder (NMOSD) was established.
To describe the clinical characteristics of PTS in NMOSD based on a video recording and to provide a literature review on the topic.
We report a case of a 38 years-old woman with a diagnosis of NMOSD and positive aquaporin-4 IgG antibody status who developed PTS five weeks after an episode of longitudinal extensive transverse myelitis (LETM).
Repetitive, brief, and painful episodes of muscle contraction were observed on the patient's left hand, spreading to the left arm, and then extending to the four limbs. While pregabalin and topiramate had no influence on these episodes, the patient responded to carbamazepine (CBZ), without symptom recurrence after one year.
PTS in association with LETM can be considered typical for NMOSD. Although the exact mechanism is unknown, ephaptic transmission after spinal cord damage and excitatory soluble factors released during acute inflammation responses are sought to be involved. Symptomatic treatment with CBZ achieved remission of spams in our case.
最近，疼痛性强直性痉挛（PTS）与视神经脊髓炎谱系障碍（NMOSD）之间建立了关联。
基于视频记录描述NMOSD中PTS的临床特征，并提供关于该主题的文献综述。
我们报告了一例38岁女性，诊断为NMOSD且水通道蛋白-4 IgG抗体状态呈阳性，在一次纵向广泛横贯性脊髓炎（LETM）发作五周后出现PTS。
观察到患者左手出现重复性、短暂性且疼痛的肌肉收缩发作，蔓延至左臂，然后扩展到四肢。虽然普瑞巴林和托吡酯对这些发作没有影响，但患者对卡马西平（CBZ）有反应，一年后症状未复发。
与LETM相关的PTS可被认为是NMOSD的典型症状。尽管确切机制尚不清楚，但脊髓损伤后的触突传递以及急性炎症反应期间释放的兴奋性可溶性因子被认为与之有关。在我们的病例中，用CBZ进行对症治疗使痉挛得到缓解。
