著者

野本 信篤 紺野 晋吾 村田 眞由美 中空 浩志 根本 博 藤岡 俊樹

出版者

日本神経学会

雑誌

臨床神経学 (ISSN:0009918X)

巻号頁・発行日

vol.50, no.6, pp.415-417, 2010 (Released:2010-06-24)

参考文献数

7

被引用文献数

3 3

---

症例は48歳男性である.微熱,食欲低下が1週間続いた後に,頸部の激痛と右側上肢の脱力をみとめた.第3病日に両上肢の脱力,激痛が出現した.肝逸脱酵素の急激な上昇をともなった.右上肢は完全麻痺,左側上肢はMMT3で,深部反射は消失し,神経痛性筋萎縮症と診断した.髄液細胞数2/mm3,蛋白105mg/dl と蛋白細胞解離をみとめ,IgMおよびIgG抗GT1a抗体が陽性であった.抗GT1a抗体は咽頭頸部上腕型のGuillain-Barré症候群との関与が指摘されており,腕神経叢の障害を呈した本症例の臨床所見と一致した.抗GT1a抗体は一次的に病態と関わった可能性と二次的変化である可能性の両者が考えられた.

著者

渡邉 典子 相馬 ひとみ 吉野 彩香 和田 麻季子 藤岡 俊樹

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.39, no.4, pp.723-726, 2022 (Released:2022-12-27)

参考文献数

12

---

症例は90歳男性.10年前にAlzheimer病と血管性認知症と診断され,薬剤治療を受けていたが,重症心不全を発症してICUに入院.心不全状態は改善したがせん妄症状と,自発性の低下,不眠,日中の傾眠,食事摂取不良をきたしたため,睡眠薬,抗認知症薬,鎮静薬などの投与に加え,認知症ケアチームが介入した.患者は認知症発症以前から音楽を趣味としていたため,好きな楽曲を中心に週3回,リハビリテーションの時に聴かせ,歌う様に促したところ,1ヶ月後には自発的にリズムをとり歌うようになった.同時にABC認知症スケールは50点から60点に改善,日中の傾眠も改善し自宅退院できた.音楽療法の効果は薬物療法と違いエビデンスは低いとされているが本例では音楽療法の開始に伴い日常生活動作の改善が明らかとなった.今後,音楽療法の科学的根拠を求めた検討が必要と思われた.

著者

藤岡 俊樹

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.35, no.3, pp.198-202, 2018 (Released:2018-12-25)

参考文献数

29

---

CIDP (chronic inflammatory demyelinating polyneuritis) is the most common immune–mediated chronic polyneuropathy that comprises typical CIDP (symmetrical demyelinating sensory–motor neuropathy) and several subtypes ; i.e., MADSAM (multifocal acquired demyelinating sensory and motor) neuropathy (Lewis Sumner Syndrome), DADS (distal acquired demyelinating symmetric) neuropathy and multifocal motor neuropathy (MMN). Typical CIDP is usually treated by either intravenous immunoglobulin (IVIg), corticosteroid (CS) or plasma exchange (PLEX). These three therapies are thought as a first line therapy (FLT). Among FLT, IVIg and CS are very often employed because of their simplicity during treatment, however, the efficacy is almost same in three modalities of FLT. If one treatment of FLT failed to lead a good response, other treatment of FLT should be tried. Finally, FLT results in remission in more than 80% of typical CIDP cases. Second line treatment for FLT–resistant cases is still vague although numerous experiences especially about immunosuppressive agents (ISA) have been reported. Among ISA, cyclophosphamide (intravenous pulse) and rituximab seem to be effective although randomized controlled trial is required. IVIg treatment requires maintenance treatment (IVIg every three weeks). Recently efficacy of subcutaneous immunoglobulin treatment (SCIg) in maintenance treatment was proven although it is not approved in Japan. Merit of SCIg is that it can be done by patients in their home. Moreover, SCIg is proven effective as an initial treatment for typical CIDP. This may help patients suffering from CIDP reduce economical, physical or time burden during treatment.The standard treatment for MADSAM neuropathy is still vague. FLT in typical CIDP should be tried as well. IVIg–responding case needs to adhere maintenance IVIg or SCIg although later is not approved in Japan.MMN responds to IVIg well, however, delayed diagnosis or treatment results in poor response.Recently, neuropathy with antibodies against proteins localized in paranode of peripheral nerve is discovered, called as “paranodopathy”.Most of these cases have IgG4 antibodies against Neurofascin 155, 140, 186, contactin–1, or contactin–associated protein 1 (Caspr). They present unique signs compared to typical CIDP, i.e., relatively rapid progression, remarkable ataxia or tremor. Most of these cases present poor response for IVIg. Rituximab seems to be a hopeful therapeutical candidate in the future although strong evidence is not available at this moment.