Carnero Contentti E, Leguizamón F, Hryb J P, Celso J, Pace J L Di, Ferrari J, Knorre E, Perassolo M B
Consultorio de Neuroinmunología, Servicio de Neurología, Hospital General de Agudos Dr. Carlos G. Durand, Buenos Aires, Argentina.
Servicio de Neurología, Hospital General de Agudos Dr. Teodoro Álvarez, Buenos Aires, Argentina.
Neurologia. 2016 Oct;31(8):511-5. doi: 10.1016/j.nrl.2014.12.001. Epub 2015 Feb 3.
Paroxysmal painful tonic spasms (PPTS) were initially described in multiple sclerosis (MS) but they are more frequent in neuromyelitis optica (NMO). The objective is to report their presence in a series of cases of NMO and NMO spectrum disorders (NMOSD), as well as to determine their frequency and clinical features.
We conducted a retrospective assessment of medical histories of NMO/NMOSD patients treated in 2 hospitals in Buenos Aires (Hospital Durand and Hospital Álvarez) between 2009 and 2013.
Out of 15 patients with NMOSD (7 with definite NMO and 8 with limited NMO), 4 presented PPTS (26.66%). PPTS frequency in the definite NMO group was 57.14% (4/7). Of the 9 patients with longitudinally extensive transverse myelitis (LETM), 44.44% (9/15) presented PPTS. Mean age was 35 years (range, 22-38 years) and all patients were women. Mean time between NMO diagnosis and PPTS onset was 7 months (range, 1-29 months) and mean time from last relapse of LETM was 30 days (range 23-40 days). LETM (75% cervicothoracic and 25% thoracic) was observed by magnetic resonance imaging (MRI) in all patients. Control over spasms and pain was achieved in all patients with carbamazepine (associated with gabapentin in one case). No favourable responses to pregabalin, gabapentin, or phenytoin were reported.
PPTS are frequent in NMO. Mean time of PPTS onset is approximately one month after an LETM relapse, with extensive cervicothoracic lesions appearing on the MRI scan. They show an excellent response to carbamazepine but little or no response to pregabalin and gabapentin. Prospective studies with larger numbers of patients are necessary in order to confirm these results.
阵发性疼痛性强直性痉挛（PPTS）最初在多发性硬化症（MS）中被描述，但在视神经脊髓炎（NMO）中更为常见。目的是报告其在一系列视神经脊髓炎及视神经脊髓炎谱系障碍（NMOSD）病例中的存在情况，并确定其发生率和临床特征。
我们对2009年至2013年间在布宜诺斯艾利斯的两家医院（杜兰德医院和阿尔瓦雷斯医院）接受治疗的NMO/NMOSD患者的病历进行了回顾性评估。
在15例NMOSD患者中（7例确诊为NMO，8例为局限性NMO），4例出现PPTS（26.66%）。确诊NMO组中PPTS的发生率为57.14%（4/7）。在9例纵向广泛横贯性脊髓炎（LETM）患者中，44.44%（9/15）出现PPTS。平均年龄为35岁（范围22 - 38岁），所有患者均为女性。NMO诊断至PPTS发作的平均时间为7个月（范围1 - 29个月），自LETM最后一次复发的平均时间为30天（范围23 - 40天）。所有患者通过磁共振成像（MRI）观察到LETM（75%为颈胸段，25%为胸段）。所有患者使用卡马西平（1例联合加巴喷丁）后痉挛和疼痛得到控制。未报告对普瑞巴林、加巴喷丁或苯妥英有良好反应。
PPTS在NMO中很常见。PPTS发作的平均时间约为LETM复发后一个月，MRI扫描显示颈胸段广泛病变。它们对卡马西平反应良好，但对普瑞巴林和加巴喷丁反应很小或无反应。有必要进行更大规模患者的前瞻性研究以证实这些结果。
