Matsunaga K, Uozumi T, Akamatsu N, Nagashio Y, Qingrui L, Hashimoto T, Tsuji S
Department of Neurology, University of Occupational and Environmental Health, School of Medicine, Yahatanishi-ku, Kitakyushu City, Japan.
Clin Neurophysiol. 2000 Mar;111(3):471-6. doi: 10.1016/s1388-2457(99)00277-1.
To describe electrophysiological findings in a patient with Creutzfeldt-Jakob disease (CJD) showing negative myoclonus.
We studied this CJD patient electrophysiologically, in comparison with two patients with cortical reflex positive myoclonus due to benign adult familial myoclonic epilepsy (BAFME). Spontaneous negative myoclonus was associated with periodic synchronous discharges (PSDs) on the electroencephalogram, but negative myoclonus could also be induced by electrical stimulation of the median nerve in the CJD patient. This patient showed giant somatosensory evoked potentials (SEPs) and enhanced C reflexes, and the duration of the induced EMG silences was found to be significantly correlated with the amplitude of cortical SEPs. The duration of silent periods (SPs) produced by magnetic stimulation of the motor cortex was extremely long. The study of recovery function of SEPs suggested that the excitability of the somatosensory cortex was decreased during a long post-stimulus period. These findings were clearly different from those of patients with BAFME.
This CJD patient had two types of negative myoclonus; one was associated with PSDs and the other was cortical reflex negative myoclonus. The long-lasting decrease in excitability of the sensorimotor cortices after stimulation could be related to the occurrence of both types of negative myoclonus.
描述1例患有克雅氏病（CJD）且表现为阴性肌阵挛的患者的电生理检查结果。
我们对该CJD患者进行了电生理研究，并与2例因良性成人家族性肌阵挛性癫痫（BAFME）导致皮质反射阳性肌阵挛的患者进行比较。自发性阴性肌阵挛与脑电图上的周期性同步放电（PSD）相关，但在该CJD患者中，正中神经电刺激也可诱发阴性肌阵挛。该患者出现巨大体感诱发电位（SEP）和C反射增强，并且发现诱发的肌电图静息期持续时间与皮质SEP的波幅显著相关。运动皮质磁刺激产生的静息期（SP）持续时间极长。SEP恢复功能研究表明，在刺激后的长时间内体感皮质的兴奋性降低。这些发现与BAFME患者的明显不同。
该CJD患者有两种类型的阴性肌阵挛；一种与PSD相关，另一种是皮质反射阴性肌阵挛。刺激后感觉运动皮质兴奋性的长期降低可能与两种类型阴性肌阵挛的发生有关。
