Takeuchi H, Touge T, Miki H, Yamada A, Deguchi K, Nishioka M
Third Department of Internal Medicine, Kagawa Medical School, Japan.
Electromyogr Clin Neurophysiol. 1992 Mar;32(3):143-54.
Reflex myoclonus displays symptomatological heterogeneity involving the cortical and brain stem types that seem to originate above the spinal cord. Three cases of generalized myoclonus proved to be spontaneous and stimulus-sensitive, and increased with action. Segmental spinal myoclonus was spontaneous, stimulus-sensitive and rhythmical and decreased with action. Two cases of post-anoxic myoclonus seemed to be of the reticular reflex in which myoclonus was manifested in all muscles, particularly the proximal ones, and for which the EEG showed no spikes preceding myoclonus. The evoked electromyogram showed a long-loop reflex (LLR) of high amplitude, with no giant somatosensory evoked potential (SEP). Pharmacological examinations showed that the thyrotropin-releasing hormone (TRH) enhanced the onset of myoclonus, shortened the latency of the LLR and increased its amplitude, but caused no remarkable changes in SEP. These results indicate that TRH stimulates the medullary reticular neuron, thereby enhancing reticular reflex myoclonus. The myoclonus of a 3rd case was believed to be cortical reflex myoclonus on the basis of the emergence of giant SEP, increased LLR and the onset of spikes in the EEGs preceding myoclonic jerks, as ascertained by jerk-locked averaging analysis with muscular discharge. Pharmacologically, LLR, SEP and myoclonus showed no definite changes in response to TRH. Segmental myoclonus which seemed to have a spinal origin, showed no giant SEP, enhanced LLR or cortical spikes in the electrophysiological studies. No definite clinical or electrophysiological changes in response to TRH were observed. We believe the TRH administration test may be useful in the differential diagnosis of stimulus-sensitive myoclonus. In addition, the origins and nature of these types of reflex myoclonus are discussed.
反射性肌阵挛表现出症状学异质性,包括皮质型和脑干型,似乎起源于脊髓以上部位。3例全身性肌阵挛被证明是自发性且对刺激敏感的,并且随动作而加重。节段性脊髓肌阵挛是自发性的、对刺激敏感且有节律的,随动作而减轻。2例缺氧后肌阵挛似乎是网状反射性的,其中肌阵挛出现在所有肌肉中,尤其是近端肌肉,脑电图显示在肌阵挛之前无棘波。诱发肌电图显示高幅长环反射(LLR),无巨大体感诱发电位(SEP)。药理学检查表明,促甲状腺激素释放激素(TRH)可增强肌阵挛的发作,缩短LLR的潜伏期并增加其幅度,但对SEP无显著影响。这些结果表明,TRH刺激延髓网状神经元,从而增强网状反射性肌阵挛。根据巨大SEP的出现、LLR增加以及通过肌肉放电的抽搐锁定平均分析确定的脑电图中在肌阵挛性抽搐之前出现的棘波,第3例患者的肌阵挛被认为是皮质反射性肌阵挛。在药理学上,LLR、SEP和肌阵挛对TRH无明确反应。似乎起源于脊髓的节段性肌阵挛在电生理研究中未显示巨大SEP、增强的LLR或皮质棘波。未观察到对TRH有明确的临床或电生理变化。我们认为TRH给药试验可能有助于对刺激敏感性肌阵挛进行鉴别诊断。此外,还讨论了这些类型反射性肌阵挛的起源和性质。