今日の臨床サポート

ギラン・バレー症候群

著者: 三井良之 近畿大学 医学部

著者: 楠進 近畿大学 医学部

監修: 永山正雄 国際医療福祉大学大学院医学研究科 脳神経内科学

著者校正済:2021/08/18
現在監修レビュー中
参考ガイドライン:
  1. 日本神経学会:ギラン・バレー症候群、フィッシャー症候群診療ガイドライン2013
患者向け説明資料

概要・推奨   

  1. 歩行困難のあるGBS患者の治療として、血漿交換療法(plasma exchange, PE)が推奨される(推奨度1)。
  1. 歩行困難のあるGBS患者の治療としてガンマグロブリン大量静注療法(Intravenous ImmunoglobulinIVIg)が推奨される(推奨度1
  1. 歩行困難のあるGBS患者の治療として、免疫吸着療法(Immunoadsorption Plasmapheresis、IAPP)、二重膜濾過法(Double filtration Plasmapheresis、DFPP)が推奨されるが、PEに比べるとエビデンスレベルは低い(推奨度2)
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となりま
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧
  1. 重症度は、GBS disability score[1]()を用いることが多く、治療適応や経過観察の指標として利用されている。予後予測スケールとして、modified Erasmus GBS Outcome Score[2](以下mEGOS,)やErasmus GBS Respiratory Insufficiency Score[3](以下EGRIS,)が、長期の機能予後や人工呼吸器装着が必要となる症例の予測に役立つ。mEGOSは、半年後の独歩可能性を、入院当日、7日目の臨床症状から予測するスコアであり、簡便に施行できる。本邦でもその有用性が確認されている[4]。また、EGRISは5点以上で人工呼吸管理が必要となる確率が高くなる(推奨度2)。
  1. 一般に、肺活量12~15mL/kg以下の場合、あるいは予測値よりも30~40%低下している場合、室内空気でPO270mmHg以下、4~6時間にわたって肺活量低下傾向のあるとき、高度の球麻痺があり誤嚥が認められる場合などは、気管挿管、人工呼吸器管理が推奨される(推奨度2)。
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
三井良之 : 特に申告事項無し[2021年]
楠進 : 特に申告事項無し[2021年]
監修:永山正雄 : 未申告[2021年]

改訂のポイント:
  1. 定期レビューを行い、重症化および人工呼吸器装着の予測因子について加筆・整理した。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. ギラン・バレー症候群(GBS)とは、先行感染などにより惹起された自己免疫機序による急性炎症性ニューロパチーである。
  1. 10万人あたりの年間発症率は0.6~1.9人、男女比は1.67:1くらいで、男性に多い。高齢になるほど発症率が上昇する。
  1. 臨床症侯は急性進行性の運動障害を主体とするが、感覚神経障害、自律神経障害も伴う。先行感染から1週間程度で発症することが多い。4週以内に症侯のピークを迎え、その後、自然回復傾向を示す。多くの症例では2週以内にピークとなる。自然回復も期待できるが、重症度は軽微な筋力低下にとどまる例から、呼吸筋麻痺を来たし人工呼吸器装着を必要とする例までさまざまである。
  1. Zika Virus感染症で、GBS発症率が増加したとの報告がある[5]
  1. 予後予測ツールとして、modified Erasmus GBS Outcome Score[2](以下mEGOS、<図表>)やErasmus GBS Respiratory Insufficiency Score[3](以下EGRIS,<図表>)を用いることが多い。治療方針を決定する際の参考とする。
  1. 肺活量測定が困難な状況では、人工呼吸器装着の判断にPeak flow検査 250 mL/min未満であることが役立つ可能性がある[6]
  1. 脳神経障害の合併率は報告により幅があるが、顔面神経麻痺(約50%)が最も多く、次いで球麻痺(約30%)、眼球運動麻痺(約20%)の順となる。特に両側性顔面神経麻痺ではGBSを疑うべきである。
  1. 自律神経障害は、軽微なものを含めると50%以上に合併するとされるが、臨床的に問題になるのは、不整脈、起立性低血圧、膀胱直腸障害などである。
  1. 臨床症侯とともに、神経伝導検査、血清ガングリオシド抗体測定、脳脊髄液検査を参考にする。特に神経伝導検査が重要である。
  1. エビデンスの確立した急性期治療は、ガンマグロブリン大量静注療法、血液浄化療法である。
  1. 回復期には適切なリハビリテーションを行う。
 
問診・診察のポイント  
  1. 通常、日単位で、急速進行例では時間単位で進行する四肢運動麻痺を来す場合にGBSを想起する。

今なら12か月分の料金で14ヶ月利用できます(個人契約、期間限定キャンペーン)

11月30日(火)までにお申込みいただくと、
通常12ヵ月の使用期間が2ヶ月延長となり、14ヵ月ご利用いただけるようになります。

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文献 

著者: W H Geerts, J A Heit, G P Clagett, G F Pineo, C W Colwell, F A Anderson, H B Wheeler
雑誌名: Chest. 2001 Jan;119(1 Suppl):132S-175S.
Abstract/Text
PMID 11157647  Chest. 2001 Jan;119(1 Suppl):132S-175S.
著者: A K Asbury, D R Cornblath
雑誌名: Ann Neurol. 1990;27 Suppl:S21-4.
Abstract/Text Criteria for the diagnosis of Guillain-Barré syndrome are reaffirmed. Electrodiagnostic criteria are expanded and specific detail added.

PMID 2194422  Ann Neurol. 1990;27 Suppl:S21-4.
著者: Fabienne Krauer, Maurane Riesen, Ludovic Reveiz, Olufemi T Oladapo, Ruth Martínez-Vega, Teegwendé V Porgo, Anina Haefliger, Nathalie J Broutet, Nicola Low, WHO Zika Causality Working Group
雑誌名: PLoS Med. 2017 Jan;14(1):e1002203. doi: 10.1371/journal.pmed.1002203. Epub 2017 Jan 3.
Abstract/Text BACKGROUND: The World Health Organization (WHO) stated in March 2016 that there was scientific consensus that the mosquito-borne Zika virus was a cause of the neurological disorder Guillain-Barré syndrome (GBS) and of microcephaly and other congenital brain abnormalities based on rapid evidence assessments. Decisions about causality require systematic assessment to guide public health actions. The objectives of this study were to update and reassess the evidence for causality through a rapid and systematic review about links between Zika virus infection and (a) congenital brain abnormalities, including microcephaly, in the foetuses and offspring of pregnant women and (b) GBS in any population, and to describe the process and outcomes of an expert assessment of the evidence about causality.
METHODS AND FINDINGS: The study had three linked components. First, in February 2016, we developed a causality framework that defined questions about the relationship between Zika virus infection and each of the two clinical outcomes in ten dimensions: temporality, biological plausibility, strength of association, alternative explanations, cessation, dose-response relationship, animal experiments, analogy, specificity, and consistency. Second, we did a systematic review (protocol number CRD42016036693). We searched multiple online sources up to May 30, 2016 to find studies that directly addressed either outcome and any causality dimension, used methods to expedite study selection, data extraction, and quality assessment, and summarised evidence descriptively. Third, WHO convened a multidisciplinary panel of experts who assessed the review findings and reached consensus statements to update the WHO position on causality. We found 1,091 unique items up to May 30, 2016. For congenital brain abnormalities, including microcephaly, we included 72 items; for eight of ten causality dimensions (all except dose-response relationship and specificity), we found that more than half the relevant studies supported a causal association with Zika virus infection. For GBS, we included 36 items, of which more than half the relevant studies supported a causal association in seven of ten dimensions (all except dose-response relationship, specificity, and animal experimental evidence). Articles identified nonsystematically from May 30 to July 29, 2016 strengthened the review findings. The expert panel concluded that (a) the most likely explanation of available evidence from outbreaks of Zika virus infection and clusters of microcephaly is that Zika virus infection during pregnancy is a cause of congenital brain abnormalities including microcephaly, and (b) the most likely explanation of available evidence from outbreaks of Zika virus infection and GBS is that Zika virus infection is a trigger of GBS. The expert panel recognised that Zika virus alone may not be sufficient to cause either congenital brain abnormalities or GBS but agreed that the evidence was sufficient to recommend increased public health measures. Weaknesses are the limited assessment of the role of dengue virus and other possible cofactors, the small number of comparative epidemiological studies, and the difficulty in keeping the review up to date with the pace of publication of new research.
CONCLUSIONS: Rapid and systematic reviews with frequent updating and open dissemination are now needed both for appraisal of the evidence about Zika virus infection and for the next public health threats that will emerge. This systematic review found sufficient evidence to say that Zika virus is a cause of congenital abnormalities and is a trigger of GBS.

PMID 28045901  PLoS Med. 2017 Jan;14(1):e1002203. doi: 10.1371/journal・・・
著者: Inés González-Suárez, Irene Sanz-Gallego, Francisco Javier Rodríguez de Rivera, Javier Arpa
雑誌名: BMC Neurol. 2013 Jul 22;13:95. doi: 10.1186/1471-2377-13-95. Epub 2013 Jul 22.
Abstract/Text BACKGROUND: Guillain-Barre syndrome (GBS) is characterized by acute onset and progressive course, and is usually associated with a good prognosis. However, there are forms of poor prognosis, needing ventilatory support and major deficits at discharge. With this study we try to identify the factors associated with a worse outcome.
METHODS: 106 cases of GBS admitted in our hospital between years 2000-2010 were reviewed. Epidemiological, clinical, therapeutical and evolutionary data were collected.
RESULTS: At admission 45% had severe deficits, percentage which improves throughout the evolution of the illness, with full recovery or minor deficits in the 87% of patients at the first year review. Ages greater than 55 years, severity at admission (p < 0.001), injured cranial nerves (p = 0.008) and the needing of ventilator support (p = 0.003) were associated with greater sequels at the discharge and at the posterior reviews in the following months. 17% required mechanical ventilation (MV). Values < 250 L/min in the Peak Flow-test are associated with an increased likelihood of requiring MV (p < 0.001).
CONCLUSIONS: Older age, severe deficits at onset, injured cranial nerves, requiring MV, and axonal lesion patterns in the NCS were demonstrated as poor prognostic factors. Peak Flow-test is a useful predictive factor of respiratory failure by its easy management.

PMID 23876199  BMC Neurol. 2013 Jul 22;13:95. doi: 10.1186/1471-2377-1・・・
著者: James J Sejvar, Katrin S Kohl, Jane Gidudu, Anthony Amato, Nandini Bakshi, Roger Baxter, Dale R Burwen, David R Cornblath, Jan Cleerbout, Kathryn M Edwards, Ulrich Heininger, Richard Hughes, Najwa Khuri-Bulos, Rudolf Korinthenberg, Barbara J Law, Ursula Munro, Helena C Maltezou, Patricia Nell, James Oleske, Robert Sparks, Priscilla Velentgas, Patricia Vermeer, Max Wiznitzer, Brighton Collaboration GBS Working Group
雑誌名: Vaccine. 2011 Jan 10;29(3):599-612. doi: 10.1016/j.vaccine.2010.06.003. Epub 2010 Jun 18.
Abstract/Text
PMID 20600491  Vaccine. 2011 Jan 10;29(3):599-612. doi: 10.1016/j.vacc・・・
著者: T W Ho, B Mishu, C Y Li, C Y Gao, D R Cornblath, J W Griffin, A K Asbury, M J Blaser, G M McKhann
雑誌名: Brain. 1995 Jun;118 ( Pt 3):597-605.
Abstract/Text Guillain-Barré syndrome has been considered to be primarily an acute inflammatory demyelinating polyneuropathy (AIDP). Our experience with Guillain-Barré syndrome in northern China differs from the traditional concept. Electrophysiologically and pathologically, most of our patients have motor axonal degeneration with minimal cellular inflammation, which we have termed 'acute motor axonal neuropathy' (AMAN). The current studies were undertaken to characterize prospectively the clinical, electrophysiological, and serological features of Guillain-Barré syndrome, defined clinically, in northern China. In 1991 and 1992, we characterized by electrodiagnostic criteria 129 Chinese patients with Guillain-Barré syndrome. The AMAN form was present in 65% of patients, the AIDP form in 24% and 11% were unclassifiable. For the 38 patients who presented from January to October, 1992, we performed serological assays for antibodies to Campylobacter jejuni and to glycolipids. Of these 38 patients, 55% had AMAN, 32% had AIDP and 13% were unclassifiable. Sixty-six percent of the 38 had serological evidence of recent C. jejuni infection as compared with 16% of village controls (P = 0.001). Seventy-six percent of AMAN patients and 42% of AIDP patients were seropositive. IgG anti-GM1 antibodies were more frequent in Guillain-Barré syndrome patients compared with village controls (42% versus 6%; P < 0.01). However, no statistically significant correlations were found between the pattern of disease, AMAN or AIDP, anti-glycolipid antibodies, or C. jejuni antibodies. Based on electrophysiological criteria, Guillain-Barré syndrome in northern China can be divided into two predominant forms: AIDP and AMAN. The AMAN form is more common and predominates in the yearly summer outbreaks of Guillain-Barré syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)

PMID 7600081  Brain. 1995 Jun;118 ( Pt 3):597-605.
著者: M Färkkilä, E Kinnunen, E Haapanen, M Iivanainen
雑誌名: Neurology. 1987 May;37(5):837-40.
Abstract/Text A prospective, controlled study with quantitative measurement of hand muscle-force for plasma exchange treatment in acute Guillain-Barré polyradiculitis was done. Of the 29 patients with severe symptoms, every second patient was selected to the plasma exchange group and all others to the control group. The muscle forces increased and CSF protein decreased significantly more in the plasma exchange group than in the control group, but there were no differences in hospitalization or recovery periods.

PMID 3553986  Neurology. 1987 May;37(5):837-40.
著者:
雑誌名: Neurology. 1985 Aug;35(8):1096-104.
Abstract/Text We compared plasmapheresis with conventional therapy in 245 patients with the Guillain-Barré syndrome of recent onset. Statistically significant differences, favoring the plasmapheresis group, were found in terms of improvement at 4 weeks, time to improve one clinical grade, time to independent walking, and outcome at 6 months. Plasmapheresis was not effective for all patients, but was particularly effective for patients who received this treatment within 7 days of onset and for patients who required mechanical ventilation after entry into the study. Plasmapheresis appears to be of benefit in patients with Guillain-Barré syndrome of recent onset.

PMID 4022342  Neurology. 1985 Aug;35(8):1096-104.
著者:
雑誌名: Ann Neurol. 1987 Dec;22(6):753-61. doi: 10.1002/ana.410220612.
Abstract/Text The goals of this multicenter controlled trial were: (1) to study the short-term effect of plasma exchange in the Guillain-Barré syndrome when applied alone within 17 days of onset of the disease; and (2) to compare two replacement fluids, diluted albumin and fresh frozen plasma (FFP) with regard to efficacy and morbidity. Two hundred twenty patients were included, 111 in the control group, 109 in the plasma exchange group, 57 of whom were assigned to receive albumin and 52 to receive fresh frozen plasma. Treatment consisted of four plasma exchanges of two plasma volumes each, initiated on the day of randomization and repeated on alternate days. Significant short-term benefits appeared in the group that received plasma exchange as demonstrated by the reduction in the proportion of patients who required assisted ventilation after randomization, and the decrease in time before beginning weaning from ventilator, time to onset of motor recovery, and time to walk with and without assistance. No statistically significant difference was found between the group that received albumin and the group that received fresh frozen plasma. Incidents during exchanges and complications related to the plasma exchange were more frequent in patients who received fresh frozen plasma. Plasma exchange is beneficial in Guillain-Barré syndrome when it is carried out early in the course of the disease. Given its risks and the lack of its clear superiority over albumin, however, we recommend that fresh frozen plasma be abandoned in the treatment of Guillain-Barré syndrome.

PMID 2893583  Ann Neurol. 1987 Dec;22(6):753-61. doi: 10.1002/ana.410・・・
著者:
雑誌名: Ann Neurol. 1997 Mar;41(3):298-306. doi: 10.1002/ana.410410304.
Abstract/Text Plasma exchange (PE) is the standard treatment in Guillain-Barré syndrome (GBS) patients who have lost the ability to walk. The effect of exchanges before this stage and the optimal number of exchanges for the other patients are still unknown. We randomized 556 GBS patients according to severity and number of exchanges as follows: Zero versus 2 PEs for patients who could walk-with or without aid-but not run, or who could stand up unaided (mild group); 2 versus 4 PEs for patients who could not stand up unaided (moderate group); and 4 versus 6 PEs for mechanically ventilated patients (severe group). In the mild group, 2 PEs were more effective than none for time to onset of motor recovery (median, 4 vs 8 days, respectively). In the moderate group, 4 PEs were more beneficial than 2 for time to walk with assistance (median, 20 vs 24 days) and for 1-year full muscle-strength recovery rate (64% vs 46%). Six PEs were no more beneficial than 4 in the severe cases. Patients with mild GBS on admission should receive 2 PEs. Patients with moderate and severe forms should benefit from 2 further exchanges.

PMID 9066350  Ann Neurol. 1997 Mar;41(3):298-306. doi: 10.1002/ana.41・・・
著者: H S Patwa, V Chaudhry, H Katzberg, A D Rae-Grant, Y T So
雑誌名: Neurology. 2012 Mar 27;78(13):1009-15. doi: 10.1212/WNL.0b013e31824de293.
Abstract/Text OBJECTIVE: To assess the evidence for the efficacy of IV immunoglobulin (IVIg) to treat neuromuscular disorders.
METHODS: The MEDLINE, Web of Science, and EMBASE databases were searched (1966-2009). Selected articles were rated according to the American Academy of Neurology's therapeutic classification of evidence scheme; recommendations were based on the evidence level.
RESULTS AND RECOMMENDATIONS: IVIg is as efficacious as plasmapheresis and should be offered for treating Guillain-Barré syndrome (GBS) in adults (Level A). IVIg is effective and should be offered in the long-term treatment of chronic inflammatory demyelinating polyneuropathy (Level A). IVIg is probably effective and should be considered for treating moderate to severe myasthenia gravis and multifocal motor neuropathy (Level B). IVIg is possibly effective and may be considered for treating nonresponsive dermatomyositis in adults and Lambert-Eaton myasthenic syndrome (Level C). Evidence is insufficient to support or refute use of IVIg in the treatment of immunoglobulin M paraprotein-associated neuropathy, inclusion body myositis, polymyositis, diabetic radiculoplexoneuropathy, or Miller Fisher syndrome, or in the routine treatment of postpolio syndrome or in children with GBS (Level U). IVIg combined with plasmapheresis should not be considered for treating GBS (Level B). More data are needed regarding IVIg efficacy as compared with other treatments/treatment combinations. Most studies concluded IVIg-related serious adverse effects were rare. Given the variable nature of these diseases, individualized treatments depending on patient need and physician judgment are important.

PMID 22454268  Neurology. 2012 Mar 27;78(13):1009-15. doi: 10.1212/WNL・・・
著者: V Bril, W K Ilse, R Pearce, A Dhanani, D Sutton, K Kong
雑誌名: Neurology. 1996 Jan;46(1):100-3.
Abstract/Text We compared intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) in the treatment of 50 patients with Guillain-Barré syndrome (GBS). Standard outcome measures did not differ for the two groups. Sixty-one percent of the PLEX-treated group and 69% of the IVIG-treated group improved by one disability grade at 1 month. The complication rate was higher in the PLEX-treated group. We conclude that the efficacy of IVIG in the treatment of GBS is comparable with that of PLEX and that it can be used safely, although we had a small number of patients. We did not observe a higher relapse rate with IVIG. The usefulness of combination therapy is unknown at this time.

PMID 8559353  Neurology. 1996 Jan;46(1):100-3.
著者:
雑誌名: Lancet. 1997 Jan 25;349(9047):225-30.
Abstract/Text BACKGROUND: The relative efficacy of plasma exchange (PE) and intravenous immunoglobulin (IVIg) for the treatment of Guillain-Barré syndrome has not been established. We compared PE with IVIg, and with a combined regimen of PE followed by IVIg, in an international, multicentre, randomised trial of 383 adult patients with Guillain-Barré syndrome.
METHODS: The patients were randomly assigned PE (five 50 mL/kg exchanges over 8-13 days), IVIg (Sandoglobulin, 0.4 g/kg daily for 5 days), or the PE course immediately followed by the IVIg course. The inclusion criteria were severe disease (aid needed for walking) and onset of neuropathic symptoms within the previous 14 days. Patients were followed up for 48 weeks.
FINDINGS: Four patients were excluded because they did not meet the randomisation criteria. All the remaining 379 patients were assessed for the major outcome criterion-change on a seven-point disability grade scale-by an observer unaware of treatment assignment, 4 weeks after randomisation. At that time, the mean improvement was 0.9 (SD 1.3) in the 121 PE-group patients, 0.8 (1.3) in the 130 IVIg-group patients, and 1.1 (1.4) in the 128 patients who received both treatments (intention-to-treat analysis). None of the differences between the groups for this major outcome criterion was significant. The difference between PE alone and IVIg alone was so small that a 0.5 grade difference was excluded at the 95% level of confidence. There was no significant difference between any of the treatment groups in the secondary outcome measures: time to recovery of unaided walking, time to discontinuation of ventilation, and trend describing the recovery from disability up to 48 weeks. There was a non-significant trend towards a more favourable outcome on some outcome measures with combined treatment.
INTERPRETATION: In treatment of severe Guillain-Barré syndrome during the first 2 weeks after onset of neuropathic symptoms, PE and IVIg had equivalent efficacy. The combination of PE with IVIg did not confer a significant advantage.

PMID 9014908  Lancet. 1997 Jan 25;349(9047):225-30.
著者: S Kuwabara, M Nakajima, I Moroo, K Hirayama
雑誌名: Rinsho Shinkeigaku. 1996 Feb;36(2):289-92.
Abstract/Text We studied the effect of double filtration and immunoadsorption plasmapheresis in Guillain-Barré syndrome retrospectively in 70 patients. Thirty three patients had plasmapheresis, of whom 23 had double filtration and 10 immunoadsorption. The rest of 37 patients consisted of a control group. Clinical disability was evaluated using the functional grading scale by Hughes. In patients having a maximum disability of grade 4 and 5, the double filtration plasmapheresis group showed statistically significant improvement at 3 months after the treatment. There was no significant difference in prognoses between the immunoadsorption plasmapheresis and control groups. In mildly-illed patients having grade 2 and 3, the prognoses were not different between the three groups. In Guillain-Barré syndrome, double filtration plasmapheresis, as well as well-established plasma exchange, had benefit for severely-illed patients.

PMID 8752682  Rinsho Shinkeigaku. 1996 Feb;36(2):289-92.
著者: T Araki, H Nakata, S Kusunoki, Y Arai, Y Katayama
雑誌名: Rinsho Shinkeigaku. 2000 Oct;40(10):979-85.
Abstract/Text Immunoadsorption therapy (IAT) using TR-350 was performed for 14 patients with Guillain-Barré syndrome (GBS). Presence of serum antiganglioside antibodies (AGA) was investigated in all the patients in the acute phase. In 14 patients studied, 6 men and 8 women, ages from 24 to 74 years(mean, 42.5 years), 7 patients had suffered from common cold and 3 from diarrhea before neurological onset. Ophthalmoplegia was seen in 6 patients, facial palsy in 6, bulbar palsy in 3 and cerebellar sign in 2. Functional grade scores (FGS) by Hughes et al. of the patients were from 5 to 1 (mean, 3.3). In 4 patients, whose FGS were 1 or 2, IAT were performed, because of worsting of bulbar palsy, bilateral facial palsy and limb weakness. Relapse occurred in one patient. IAT was started from 2 to 18 days (mean, 8.4) after neurological onset and performed 3 to 14 times (mean, 7.5) for each patient. Mean FGS improved from 3.3 to 2.1 after IAT. The mean time to improve 1 grade was 10.3 days and mean time to improve 2 grades was 39.0 days. The mean FGS after 1 month was 1.4 and that after 3 months was 0.4. Some of 14 patients had elevated titers of AGA in sera in the acute phase. Four patients had anti-GQ1b IgG antibody and showed external ophthalmoplegia. One patient with anti-GD1b IgG antibody had cerebellar signs as well as peripheral neuropathy. Those AGAs decreased after IAT in parallel with improvement. IAT is an effective treatment in acute phase of GBS.

PMID 11296374  Rinsho Shinkeigaku. 2000 Oct;40(10):979-85.
著者: N K Singh, A Gupta
雑誌名: J Assoc Physicians India. 1996 Jan;44(1):22-4.
Abstract/Text Forty-six patients of Guillain-Barre' Syndrome were randomized to receive either prednisolone (40 mg daily for 2 weeks and then tapered off) or placebo. The patients were followed up for 6 months and were assessed on an objective scale of disability. The improvement in mean disability grade was significantly better at 2 weeks and 4 weeks in the placebo group as compared to those who received corticosteroids. The difference persisted at 24 weeks, but was statistically insignificant. A greater proportion of patients in the placebo group had improved by at least 1 disability grade at all points of time. The group of patients treated with steroids took twice as long to improve by 1 disability grade as compared to those in the placebo group. At 6 months, 41.7% of the patients in the steroid group had recovered almost completely (good outcome) as compared to 54.5% of the patients is the placebo group. Corticosteroids, therefore, do not appear to benefit GBS patients, and may in fact, delay the recovery from acute illness.

PMID 8773088  J Assoc Physicians India. 1996 Jan;44(1):22-4.
著者:
雑誌名: Lancet. 1993 Mar 6;341(8845):586-90.
Abstract/Text Steroids have been beneficial in the treatment of demyelinating diseases with features similar to those of Guillain-Barré syndrome (GBS). However, steroid treatment of GBS has been disappointing; in an earlier trial oral prednisolone was ineffective, although the dose was low and the sample small. We assessed the benefit of a high-dose steroid regimen in a large sample of patients with GBS in a multicentre, randomised, double-blind trial. 242 adult patients were randomised to receive intravenous methylprednisolone (IVMP) 500 mg (124 patients) or a placebo (118) daily for 5 days. Patients were diagnosed by standard clinical criteria and entered the trial within 15 days of onset of neurological symptoms. All patients were too weak to run. Some patients received plasma exchange depending on the practice of their centre. Disability was graded on a scale from 0 (healthy) to 6 (dead) at intervals for 48 weeks. There was no significant difference in any outcome variable between patients treated with IVMP and those given placebo. The most important outcome was the difference between the groups in disability grade 4 weeks after randomisation, which was only a 0.06 grade (95% Cl -0.23 to 0.36) greater improvement in the IVMP than the placebo group. The 39 patients in the IVMP group who required ventilation did so for a median of 18 days, 9 days fewer than the 44 patients who had a placebo and required ventilation (95% Cl -9.6 to 27.6). Median time to walk unaided was 38 days in the IVMP patients and 50 days in the placebo patients (difference 12 days, (95% Cl -21.3 to 45.3). A short course of high-dose IVMP given early in GBS is ineffective.

PMID 8094828  Lancet. 1993 Mar 6;341(8845):586-90.
著者: R van Koningsveld, P I M Schmitz, F G Avander Meché, L H Visser, J Meulstee, P A van Doorn, Dutch GBS study group
雑誌名: Lancet. 2004 Jan 17;363(9404):192-6.
Abstract/Text BACKGROUND: Despite available treatment with intravenous immunoglobulin (IVIg), morbidity and mortality are considerable in patients with Guillain-Barré syndrome (GBS). Our aim was to assess whether methylprednisolone, when taken with IVIg, improves outcome when compared with IVIg alone.
METHODS: We did a double-blind, placebo-controlled, multicentre, randomised study, to which we enrolled patients who were unable to walk independently and who had been treated within 14 days after onset of weakness with IVIg (0.4 g/kg bodyweight per day) for 5 days. We assigned 233 individuals to receive either intravenous methylprednisolone (500 mg per day; n=116) or placebo (n=117) for 5 days within 48 h of administration of first dose of IVIg. Because age is an important prognostic factor, we split treatment groups into two age-groups-ie, younger than age 50 years, or 50 years and older. Our primary outcome was an improvement from baseline in GBS disability score of one or more grades 4 weeks after randomisation. Analysis was by intention to treat.
FINDINGS: We analysed 225 patients. GBS disability scores increased by one grade or more in 68% (76 of 112) of patients in the methylprednisolone group and in 56% (63 of 113) of controls (odds ratio [OR] 1.68, 95% CI 0.97-2.88; p=0.06). After adjustment for age and degree of disability at entry, treatment OR was 1.89 (95% CI 1.07-3.35; p=0.03). Side-effects did not differ greatly between groups.
INTERPRETATION: We noted no significant difference between treatment with methylprednisolone and IVIg and IVIg alone. Because of the relevance of prognostic factors and the limited side-effects of methylprednisolone, the potential importance of combination treatment with the drug and IVIg, however, warrants further investigation.

PMID 14738791  Lancet. 2004 Jan 17;363(9404):192-6.
著者: M Tripathi, S Kaushik
雑誌名: Crit Care Med. 2000 Mar;28(3):655-8.
Abstract/Text OBJECTIVE: To evaluate carbamezapine (CBZ) for neuritic pain relief in Guillain-Barré syndrome (GBS) patients in the intensive care unit (ICU).
DESIGN: Prospective, double-blind, randomly allocated cross-over study days.
SETTING: ICU in a tertiary care university hospital.
PARTICIPANTS: Twelve consecutive, conscious adult (22-54 yrs) patients with GBS during recovery from the muscular weakness and receiving pressure-support ventilation in the ICU. All patients complained of severe backache and/or leg cramps and tenderness in muscles, and they required opioids for pain relief.
INTERVENTIONS: CBZ (100 mg every 8 hrs) or equivalent placebo was given to nursing staff in coded powder form. Medication was given to patients through a nasogastric feeding tube. The same coded medicine was given for 3 days, and after a 1-day omission, a second set of coded powder was given for the next 3 days in a randomized, double-blind, crossover fashion. Pethidine (1 mg x kg(-1)) was given intravenously in between, if the pain score was >2. Group 1 (n = 6) patients were given a placebo on the first 3 days, followed by CBZ. Group 2 (n = 6) patients were given CBZ on the first 3 days, followed by a placebo.
MEASUREMENTS AND MAIN RESULTS: In these two study periods of different medications, we observed and scored pain (1, no pain; 5, severe pain), sedation (1, alert; 6, asleep, does not respond to verbal command), and total pethidine requirement per day. In group 1 patients, a significant (p < .001) improvement in the sedation score and a low requirement for pethidine was observed 3 days later, when CBZ was started. However, in group 2 patients, a gradual increase in the pethidine requirement and a high sedation score were noteworthy in the later days of placebo medication. Observations were also analyzed for CBZ days vs. placebo days. Overall, the pain score (1.7 +/- 0.8) during the CBZ period of both regimens was significantly (p < .001) lower than during the placebo days (3.1 +/- 0.9). Significantly higher doses of pethidine (3.7 +/- 0.9 mg/kg/day) were used on the placebo days than on the CBZ days (1.7 +/- 1.0 mg/kg/day).
CONCLUSION: The pain in GBS has a dual origin, and we recommend CBZ as an adjuvant to treat pain in GBS patients, during the recovery phase in the ICU, to reduce the narcotic requirement.

PMID 10752810  Crit Care Med. 2000 Mar;28(3):655-8.
著者: N Attal, G Cruccu, R Baron, M Haanpää, P Hansson, T S Jensen, T Nurmikko, European Federation of Neurological Societies
雑誌名: Eur J Neurol. 2010 Sep;17(9):1113-e88. doi: 10.1111/j.1468-1331.2010.02999.x. Epub 2010 Apr 9.
Abstract/Text BACKGROUND AND OBJECTIVES: This second European Federation of Neurological Societies Task Force aimed at updating the existing evidence about the pharmacological treatment of neuropathic pain since 2005.
METHODS: Studies were identified using the Cochrane Database and Medline. Trials were classified according to the aetiological condition. All class I and II randomized controlled trials (RCTs) were assessed; lower class studies were considered only in conditions that had no top-level studies. Treatments administered using repeated or single administrations were considered, provided they are feasible in an outpatient setting.
RESULTS: Most large RCTs included patients with diabetic polyneuropathies and post-herpetic neuralgia, while an increasing number of smaller studies explored other conditions. Drugs generally have similar efficacy in various conditions, except in trigeminal neuralgia, chronic radiculopathy and HIV neuropathy, with level A evidence in support of tricyclic antidepressants (TCA), pregabalin, gabapentin, tramadol and opioids (in various conditions), duloxetine, venlafaxine, topical lidocaine and capsaicin patches (in restricted conditions). Combination therapy appears useful for TCA-gabapentin and gabapentin-opioids (level A).
CONCLUSIONS: There are still too few large-scale comparative studies. For future trials, we recommend to assess comorbidities, quality of life, symptoms and signs with standardized tools and attempt to better define responder profiles to specific drug treatments.

PMID 20402746  Eur J Neurol. 2010 Sep;17(9):1113-e88. doi: 10.1111/j.1・・・
著者: Sibel Ozbudak Demir, Füsun Köseoğlu
雑誌名: Disabil Rehabil. 2008;30(8):593-9. doi: 10.1080/09638280701352626.
Abstract/Text PURPOSE: To compare the health-related quality of life (HRQOL) in control subjects and patients with severe Guillain-Barré syndrome (GBS) 6 months after rehabilitation. To determine the relationship of several sociodemographic and medical factors with the HRQOL of the GBS survivors.
METHODS: Thirty-one patients with severe GBS and 31 control subjects were included in the study. Demographic and medical variables were recorded. The functional outcome was measured using the Functional Independence Measure (FIM), both at admission and discharge and also at the 6-month follow-up examination. The HRQOLs were assessed by the Nottingham Health Profile (NHP) at the 6-month follow-up examination.
RESULTS: There were significant improvements in functional status as measured by the FIM at discharge and also at 6 months. The scores of all of the NHP dimensions of the GBS patients were significantly higher than in the control subjects. Functional disability scores were highly related to the energy level, physical mobility and emotional reactions of the NHP domains. Education, gender, employment, mechanical ventilation and tendency to depression were the factors most related to the NHP domains. Age and marital status showed no significant correlation with the NHP scores.
CONCLUSION: The HRQOL of the GBS patients remains lower than that of the control subjects. In addition to functional scores, several sociodemographic and medical variables, such as education, psychological factors, gender, mechanical ventilation and employment may play a crucial role in determining the quality of life in persons with GBS.

PMID 17852306  Disabil Rehabil. 2008;30(8):593-9. doi: 10.1080/0963828・・・

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