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1. 成人における詳細な神経学的検査the detailed neurologic examination in adults [show details]
… feature distinguishing a central lesion from a peripheral one is muscle tone . A central lesion is characterized by spasticity, whereas tone is normal or reduced with a peripheral lesion. For patients with …
2. 筋緊張低下や脱力感のある乳児へのアプローチapproach to the infant with hypotonia and weakness [show details]
… for a few seconds when the sitting position is reached. Normal muscle tone is decreased in preterm compared with term infants. Flexor tone in preterm infants diminishes with decreasing gestational age …
3. 筋力低下の評価における筋力検査muscle examination in the evaluation of weakness [show details]
…dermatomyositis. Increased muscle tone or rigidity is present in Parkinson disease, a disorder in which weakness or decreased motor function may be an initial complaint. Increased tone also occurs in upper motor …
4. 閉塞性睡眠時無呼吸の小児に行うCPAP（持続陽圧呼吸療法）cpap for pediatric obstructive sleep apnea [show details]
…resolution of events is often higher if the child is supine, and may be higher in REM sleep when muscle tone is the lowest. Therefore, the final prescription should reflect the pressure required to treat …
5. 慢性腎臓病患者の骨粗鬆症：マネージメントosteoporosis in patients with chronic kidney disease management [show details]
…systemic condition of muscle mass deficiency and poor muscle strength, receiving more attention by the National Institutes of Health and various professional societies . Improving muscle tone and strength and …

English Journal

A case report on middle cerebral artery aneurysm treated by rapid ventricular pacing: A CARE compliant case report.

Ping Y, Gu H.
Medicine. 2018 Nov;97(48)e13320.
Cerebral aneurysm is a common cause of intracranial hemorrhage, stroke, and death. It is treated with vascular surgeries, such as coil embolism and artery clipping. However, surgery itself is a risk factor that may cause rupture of aneurysm, and leads to irreversible brain damage, and even death. Ra
PMID 30508924

A Madras Motor Neuron Disease Patient With Cerebellar Atrophy: A New Clinical Feature.

Long L, Cai X, Liu J, Kang Z, Li J, Huang Z, Guo R, Zou Y, Lu Z.
Frontiers in neuroscience. 2018 ;12()722.
A 34-year-old Chinese Han female complaining of general muscle weakness and wasting for 9 years. She was admitted for aggravation of her symptoms caused by respiratory distress. She also suffered from bulbar palsy. She had no hearing loss, visual problems, or cerebellar signs. Her parents had a cons
PMID 30349455

A prospective natural history study of Krabbe disease in a patient cohort with onset between 6 months and 3 years of life.

Bascou N, DeRenzo A, Poe MD, Escolar ML.
Orphanet journal of rare diseases. 2018 08;13(1)126.
Krabbe disease is a rare neurodegenerative disorder caused by a deficiency in the lysosomal enzyme galactocerebrosidase. Patients with Krabbe disease present with a variable disease course depending on their age of onset. The purpose of this prospective cohort study was to characterize the natural p
PMID 30089515

Japanese Journal

高度の頸部後屈を呈した晩期の進行性核上性麻痺患者への治療―ボツリヌス療法が頸部後屈に奏功した1例―

林眞弘,小林克治
日本プライマリ・ケア連合学会誌 41(3), 125-128, 2018
<p>進行性核上性麻痺（以下PSP）は，精神・神経・身体症状を呈する多彩な疾患であり，晩期には運動機能障害から臥床状態を呈する．PSPは，頸部や体幹のジストニア・筋緊張を特徴とし，それらが痛みの原因にもなり，進行期には頸部後屈を呈する．高度の頸部後屈から頻回な肺炎が起こり，生命予後に影響を与える．今回，高度の頸部後屈（頸部ジストニア）を呈し，嚥下性肺炎を繰り返したPSP患者に対して，ボ …
NAID 130007490486

前頭側頭型認知症を伴う筋萎縮性側索硬化症が疑われたミトコンドリア病の1例

石川知子,中村憲一郎,島﨑里恵,後藤勝政,梅原藤雄
臨床神経学 58(1), 15-20, 2018
<p>症例は76歳女性である．2014年10月に歌声の低音化で発症し，左腕の筋力低下，著明な左優位の前頭側頭葉萎縮を認めた．構音・嚥下障害，四肢筋力低下が悪化し，筋生検にてミトコンドリア異常および筋組織ミトコンドリアDNA（mitochondrial DNA; mtDNA）多重欠失を認め，ミトコンドリア病と診断された．臨床的には前頭側頭型認知症（frontotemporal demen …
NAID 130006320310

ワールデンブルグ症候群を有する小児内耳埋め込み術の全身麻酔経験

櫛方哲也,外崎充,野口智子,松本杏菜,佐々木亮,廣田和美
日本臨床麻酔学会誌 37(2), 168-171, 2017
<p>ワールデンブルグ症候群は神経堤の分化異常による難聴，毛髪・皮膚・虹彩の色素異常を有する常染色体優性遺伝疾患である．麻酔管理上の問題点は，難聴や痙攣，筋緊張異常，低栄養である．麻酔症例報告は5例に満たず確固とした見解がいまだない．本症例は37週正常分娩で出生した1歳8カ月の男児．人工内耳埋め込み術の全身麻酔管理が依頼された．麻酔前投薬は投与せず，亜酸化窒素とセボフルラン，酸素で導入 …
NAID 130006855299