Dokkyo Medical University Repository / 獨協医科大学リポジトリ
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Recording Methods of Neuronal Cell Activity Using Microelectrodes
Get PDFTechnological advancements in microelectrode recording have contributed to the field of neurophysiology. Herein, we first review the historical background of the invention of microelectrodes. Next, epoch-making neurophysiological studies using microelectrodes, such as resting potentials, action potentials, inhibitory postsynaptic potentials, and single ion channel currents, are presented. In addition, we describe the molecular mechanisms of synapses involved in memory, mapping of the visual cortex, and functions of place and grid cells for spatial navigation, which were revealed using microelectrodes. We further describe recently developed technologies that combine electrophysiology and molecular biology, including those that contributed to identification of receptor proteins for thermal or mechanical stimuli. When combined with molecular biology, electrophysiology using microelectrodes remains useful.journal articl
A Case of Idiopathic Scoliosis with Intraoperative Neurophysiological Monitoring Abnormalities Leading to the Diagnosis of Charcot-Marie-Tooth Disease 1B
Get PDFThe current case report describes the clinical and genetic characteristics of a 16-year-old female proband. She did not have any subjective neurological symptoms preoperatively and who was incidentally diagnosed due to abnormal intraoperative neurophysiological monitoring (IONM) using transcranial electrical stimulation motor evoked potentials (TES-MEP) and somatosensory evoked potentials (SEP) for idiopathic scoliosis, leading to the diagnosis of Charcot-Marie-Tooth disease (CMT) 1B. There was no similar disease in her family history. Nerve conduction velocity testing revealed decreased conduction velocity of the median nerve, and genetic testing indicated myelin protein zero (MPZ) mutation (c242A > G), leading to the diagnosis of demyelinating type CMT1B. The parents had no genetic mutation, and this was a case of de novo mutation. CMT1B is an important differential diagnosis because, similar to our case, there may not be any clinical symptoms. The disease was discovered during a careful evaluation of the patient's scoliosis and other complications. TES-MEP was more useful than SEP for IONM of scoliosis with CMT1B.journal articl
Progress in Growth Hormone Treatment for Short Stature Children
Get PDF低身長の原因である成長ホルモン分泌不全/成長ホルモン欠損症（growth hormone （GH） deficiency：GHD）の治療のために，ヒト下垂体から抽出した GH 製剤（pituitary-derived human GH：phGH）が日本では 1975年に承認されたが，供給量は限られており，すべての GHD による低身長児に投与することはできなかった．しかし，1988 年に遺伝子組換え hGH （recombinant hGH：rhGH）が開発され，それ以降 rhGH が十分に供給されるようになり，GH 治療の状況は劇的に改善した．また，GH-IGF-I （Insulin-like growth factor-I） 軸の成長調節機構がより解明されたため, rhGH は GHD に対する補充投与だけでなく，薬理学的治療として高用量 GH が一部の GH 分泌不全のない非 GHD 低身長児の治療にも使用されるようになった．
GH 治療の安全性への懸念としては，GH の抗インスリン作用と細胞増殖活性に関連する 2 型糖尿病と白血病の発生であるが，長期調査の結果，現在，小児における rhGH の投与は安全と考えられている．GH 治療の将来については，2021 年に週 1 回の投与ですむ持続型 GH 製剤が開発され，毎日の自己注射が不要となった．その結果，成長ホルモン治療のアドヒアランスが向上し，良好な治療効果が得られることが期待される．
本総説では小児の低身長に対する GH 治療の進歩について述べた．journal articl
