Matsumoto Hideyuki, Uchio Naohiro, Hao Akihito, Haga Mari, Abe Chiaki, Sakamoto Yuuri, Ugawa Yoshikazu
Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan.
Department of Clinical Laboratory, Mitsui Memorial Hospital, Tokyo, Japan.
Case Rep Neurol. 2020 Nov 17;12(3):447-451. doi: 10.1159/000510395. eCollection 2020 Sep-Dec.
The cortical silent period (CSP) induced by transcranial magnetic stimulation (TMS) has been reported to be prolonged in 2 Creutzfeldt-Jakob disease (CJD) patients who presented with periodic myoclonus. Herein, we will show a prominent prolongation of TMS-induced CSP in a patient with CJD who did not have periodic myoclonus. The patient was a 66-year-old woman who developed rapidly progressive dementia. No myoclonic jerks were observed. Brain magnetic resonance imaging showed high-intensity lesions in the cerebral cortex, basal ganglia, and thalamus on diffusion-weighted images. Electroencephalography (EEG) showed diffuse and continuous slow waves, but no periodic synchronous discharges (PSDs). A TMS study revealed that the duration of CSP was prominently prolonged: the duration of CSP (370 ms) equaled that of the mean + 6.5 SD of the normal value. One month after admission, the patient exhibited akinetic mutism and developed periodic myoclonus in her limbs. The clinical course was compatible with CJD. To date, CSP has been measured in only 2 CJD patients. The common findings in both cases were marked prolongation of CSP, periodic myoclonus, and PSD on EEG. In short, we demonstrated that TMS-induced CSP was prominently prolonged even at the early stage of CJD without periodic myoclonus or PSD. In other disorders, the CSP has not been reported to be comparably prolonged to that of CJD patients. Therefore, we conclude that TMS-induced CSP could be prominently prolonged even in the early stage of CJD. The marked prolongation of the CSP might be an early biomarker of CJD.
据报道，在2例出现周期性肌阵挛的克雅氏病（CJD）患者中，经颅磁刺激（TMS）诱发的皮质静息期（CSP）延长。在此，我们将展示1例无周期性肌阵挛的CJD患者中TMS诱发的CSP显著延长。该患者为一名66岁女性，出现快速进展性痴呆。未观察到肌阵挛性抽搐。脑磁共振成像在扩散加权图像上显示大脑皮质、基底神经节和丘脑有高强度病变。脑电图（EEG）显示弥漫性和持续性慢波，但无周期性同步放电（PSD）。一项TMS研究显示，CSP的持续时间显著延长：CSP的持续时间（370毫秒）等于正常值的平均值+6.5标准差。入院1个月后，患者出现运动不能性缄默，并在四肢出现周期性肌阵挛。临床病程符合CJD。迄今为止，仅在2例CJD患者中测量过CSP。这两例的共同发现是CSP显著延长、周期性肌阵挛和脑电图上的PSD。简而言之，我们证明即使在CJD早期，无周期性肌阵挛或PSD时，TMS诱发的CSP也会显著延长。在其他疾病中，尚未报道CSP会像CJD患者那样延长。因此，我们得出结论，即使在CJD早期，TMS诱发的CSP也可能显著延长。CSP的显著延长可能是CJD的早期生物标志物。
