今日の臨床サポート

多発ニューロパチー

著者: 辻浩史 筑波大学 神経内科

監修: 庄司進一 筑波大学

著者校正/監修レビュー済:2019/04/05
患者向け説明資料

概要・推奨   

  1. 糖尿病性ニューロパチーの治療は、血糖値の正常化が最も重要である。
  1. Guillain-Barré症候群(GBS)の治療には単純血漿交換療法(PE)もしくは免疫グロブリン大量療法(IVIG)が有効である。
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:
辻浩史 : 特に申告事項無し[2021年]
監修:庄司進一 : 特に申告事項無し[2021年]

改訂のポイント:
  1. 定期レビューを行った(変更なし)。

病態・疫学・診察

疫学情報・病態・注意事項  
  1. 多発ニューロパチーは、典型的には左右対称で四肢の遠位側優位に末梢神経が障害される疾患の総称である。
  1. 末梢神経には運動神経、感覚神経、自律神経の3つの機能が存在し、障害される神経により臨床症状が異なる。
  1. 原因は免疫性、遺伝性、中毒性、感染性、全身疾患に伴うニューロパチーなどがあり原疾患により治療法、予後が異なる。
  1. 病理学的には脱髄障害、軸索障害に分類される。
 
神経伝導検査の模式図

脱髄性ニューロパチーでは潜時が延長し、神経伝導速度が遅くなる(b)。軸索障害性ニューロパチーでは活動電位が低下する(c)。

出典

img1:  Peripheral neuropathy.
 
 BMJ. 2002 Feb 23;324(7335):466-9.
 
  1. ニューロパチーの有病率は人口10万人当たり2,400人から8,000人である(推奨度1)。
  1. まとめ:多発ニューロパチーの頻度を文献検索した。
  1. 代表事例の説明:イタリアで行われた疫学調査では、55歳以上の多発ニューロパチーの有病率は人口10万人当たり8,000人であった[1]。またインドのボンベイにおいても、その有病率は2,400人であった[2]
  1. 結論:ニューロパチーはありふれた疾患である。
  1. 追記:わが国での疫学調査研究はPubMedにて検索されなかった。
 
問診・診察のポイント  
 
  1. 末梢神経には運動神経、感覚神経、自律神経の3つの機能が混在し、障害される神経により症状が異なる。それぞれの臨床症状が混在し、運動感覚ニューロパチーと称することもある。

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文献 

著者:
雑誌名: Neurology. 1995 Oct;45(10):1832-6.
Abstract/Text The prevalence and general characteristics of chronic symmetric symptomatic polyneuropathy were assessed in two elderly populations living in Varese, northern Italy, and San Giovanni Rotondo, southern Italy. We interviewed 4,191 subjects (3,027 in Varese and 1,164 in San Giovanni Rotondo), 55 years and older, seen in office consultations by 27 general practitioners. A neurologist examined 734 patients who had two or more symptoms of polyneuropathy. A diagnosis of possible polyneuropathy (screening neuropathic symptoms and one of the following findings: bilateral impairment of strength; bilateral impairment of sensation; bilateral impairment of deep tendon reflexes) was made in 213 patients (7.0%) in Varese and 94 (8.1%) in San Giovanni Rotondo. Probable polyneuropathy (screening symptoms and at least two of the physical findings) was present in 111 Varese patients (3.7%) and 40 San Giovanni Rotondo patients (3.4%). The age- and sex-adjusted prevalence rate of probable polyneuropathy was 3.6 per 100 in Varese and 3.3 per 100 in San Giovanni Rotondo. The disease was more prevalent in women in Varese and in men in San Giovanni Rotondo and was significantly correlated with age in Varese. Diabetes was found in association with probable polyneuropathy in 43.7% of patients. Muscle cramps and distal paresthesia were the main symptoms. In general, polyneuropathy was mild to moderate, impairment of deep tendon reflexes and sensation being the most common findings.

PMID 7477977  Neurology. 1995 Oct;45(10):1832-6.
著者: N E Bharucha, A E Bharucha, E P Bharucha
雑誌名: Neurology. 1991 Aug;41(8):1315-7.
Abstract/Text We carried out a door-to-door survey to screen for neurologic diseases, including peripheral neuropathy, in a community of 14,010 Parsis living in housing colonies in Bombay, India. The most common neurologic disorder was peripheral neuropathy with 334 cases (2,384 cases/100,000 population). The most common neuropathy was compressive, with diabetes the most common noncompressive etiology. There was no leprosy, and nutritional neuropathies were rare.

PMID 1650932  Neurology. 1991 Aug;41(8):1315-7.
著者:
雑誌名: Ann Intern Med. 1995 Apr 15;122(8):561-8.
Abstract/Text OBJECTIVE: To examine whether intensive therapy designed to achieve glycemic levels as close to normal as possible prevents or slows the progression of neuropathy when compared with conventional therapy in patients with insulin-dependent diabetes mellitus in the Diabetes Control and Complications Trial.
DESIGN: Multicenter, randomized, controlled clinical trial.
SETTING: 29 U.S. and Canadian clinical centers.
PARTICIPANTS: 1441 patients aged 13 to 39 years, of whom 726 had had insulin-dependent diabetes mellitus for 1 to 5 years and had no retinopathy at baseline (primary prevention cohort); 715 had had diabetes for 1 to 15 years and had minimal to moderate nonproliferative retinopathy at baseline (secondary intervention cohort).
INTERVENTION: Intensive therapy with three or more daily insulin injections or continuous subcutaneous insulin infusion guided by four or more glucose tests per day compared with conventional therapy with one or two daily insulin injections.
RESULTS: Intensive therapy reduced the development of confirmed clinical neuropathy (defined as a history or physical examination consistent with clinical neuropathy confirmed by either abnormal nerve conduction or autonomic nervous system testing) by 64% (95% CI, 45% to 76%) in the combined cohorts after 5 years of follow-up (5% of the intensive therapy group compared with 13% of the conventional therapy group). The prevalence of abnormal nerve conduction and abnormal autonomic nervous system function were reduced by 44% (CI, 34% to 53%) and 53% (CI, 24% to 70%), respectively (26% of the intensive treatment group developed abnormal nerve conduction compared with 46% of the conventional treatment group; 4% of the intensive treatment group had abnormal autonomic nervous system function compared with 9% of the conventional treatment group). Finally, nerve conduction velocities generally remained stable with intensive therapy but decreased significantly with conventional therapy.
CONCLUSION: Intensive diabetes therapy markedly delays or prevents the development of clinically manifest diabetic polyneuropathy as confirmed by objective nerve function testing in patients with insulin-dependent diabetes mellitus.

PMID 7887548  Ann Intern Med. 1995 Apr 15;122(8):561-8.
著者: J C Raphaël, S Chevret, R A Hughes, D Annane
雑誌名: Cochrane Database Syst Rev. 2002;(2):CD001798. doi: 10.1002/14651858.CD001798.
Abstract/Text BACKGROUND: Guillain-Barré syndrome is an acute symmetric, usually ascending and usually paralysing illness, due to inflammation of peripheral nerves. It is thought to be caused by autoimmune factors, such as antibodies. Plasma exchange removes antibodies and other potentially injurious factors from the blood stream. It involves connecting the patient's blood circulation to a machine which exchanges the plasma for a substitute solution, usually albumin. Several studies have evaluated plasma exchange for Guillain-Barré syndrome.
OBJECTIVES: To systematically review the evidence concerning the efficacy of plasma exchange for treating Guillain-Barré syndrome.
SEARCH STRATEGY: Search of the Cochrane Neuromuscular Disease Trial Register for randomised trials concerning plasma exchange in Guillain-Barré syndrome, search of the bibliographies of identified papers and enquiry from the authors of the papers.
SELECTION CRITERIA: Randomised and quasi-randomised trials of plasma exchange versus sham exchange or supportive treatment.
DATA COLLECTION AND ANALYSIS: Potentially relevant papers were scrutinised by two reviewers and the selection of eligible studies was agreed by them and a third reviewer. Data were extracted by one reviewer and checked by a second reviewer. Some missing data were obtained from the authors of studies.
MAIN RESULTS: Six eligible trials concerning 649 patients were identified, all comparing plasma exchange versus supportive treatment alone. Primary outcome measures ~bullet~Time to recover walking with aid In the only two trials for which this measure was reported, the median time to recover this ability was faster in the plasma exchange than the control group. ~bullet~Time to onset of motor recovery in mildly affected patients In the one trial for which this measure was available, the time was significantly shortened in the plasma exchange group. Secondary outcome measures ~bullet~Improvement in disability grade at four weeks In five trials, there were significantly more patients who had improved by one disability grade or more in the plasma exchange group as compared to the control group. Patients treated with plasma exchange fared significantly better in the following secondary outcome measures: time to recover walking without aid, percentage of patients requiring artificial ventilation, duration of ventilation, full muscle strength recovery after one year, and severe sequelae after one year. There were less patients with infectious events and cardiac arrhythmias in the plasma exchange than the control group. Subgroup analyses Plasma exchange was beneficial in patients with mild, moderate and severe (needing ventilation) Guillain-Barré syndrome. It was beneficial in patients with a disease duration of seven or less days and also in those with disease lasting more than seven days. However, in the only trial that enrolled patients up to 30 days from disease onset, the benefit of plasma exchange in patients treated after seven days was less apparent. Type of treatment Single studies showed that two plasma exchanges were significantly superior to none for mild Guillain-Barré syndrome and four to two for moderate Guillain-Barré syndrome, but that six were not superior to four for severe Guillain-Barré syndrome requiring ventilation. One study suggested that continuous flow plasma exchange was significantly superior to intermittent flow. Another study found no significant difference between the two techniques. The same study found a significantly higher rate of adverse events with fresh frozen plasma as the replacement fluid than albumin. Plasma exchange compared with cerebrospinal fluid filtration A single trial comparing these two treatments did not show any difference in outcomes but was too small to demonstrate equivalence.
REVIEWER'S CONCLUSIONS: Plasma exchange is the first and only treatment that has been proven to be superior to supportive treatment alone in Guillain-Barré syndrome. Consequently, plasma exchange should be regarded as the treatment against which new treatments, such as intravenous immunoglobulin, should be judged. In mild Guillain-Barré syndrome two sessions of plasma exchange are superior to none. In moderate Guillain-Barré syndrome four sessions are superior to two. In severe Guillain-Barré syndrome six sessions are no better than four. Continuous flow plasma exchange machines may be superior to intermittent flow machines and albumin to fresh frozen plasma as the exchange fluid. Plasma exchange is more beneficial when started within seven days after disease onset rather than later, but was still beneficial in patients treated up to 30 days after disease onset. The value of plasma exchange in children less than 12 years old is not known. There is insufficient evidence to determine whether cerebrospinal fluid filtration is equivalent to plasma exchange.

PMID 12076424  Cochrane Database Syst Rev. 2002;(2):CD001798. doi: 10.・・・
著者: R A C Hughes, J C Raphaël, A V Swan, P A Doorn
雑誌名: Cochrane Database Syst Rev. 2004;(1):CD002063. doi: 10.1002/14651858.CD002063.pub2.
Abstract/Text BACKGROUND: Guillain-Barré syndrome is an acute, paralysing, inflammatory peripheral nerve disease. Intravenous immunoglobulin purified from donated blood is beneficial in other autoimmune diseases.
OBJECTIVES: We aimed to determine the efficacy of intravenous immunoglobulin for treating Guillain-Barré syndrome.
SEARCH STRATEGY: We searched the Cochrane Neuromuscular Disease Group register (search updated 11 February 2003), MEDLINE and EMBASE (from January 2000 to February 2003) using Guillain-Barré syndrome and acute polyradiculoneuritis as the search terms. We also searched bibliographies of trials and made contact with their authors and other experts.
SELECTION CRITERIA: We included all randomised and quasi-randomised trials.
DATA COLLECTION AND ANALYSIS: Two reviewers examined the titles and abstracts of all the papers retrieved by the search, extracted the data and assessed the quality of the trials independently.
MAIN RESULTS: Two trials comparing intravenous immunoglobulin with supportive treatment were inadequate to establish its value. Another Cochrane systematic review has shown that plasma exchange hastens recovery. We found six randomised trials that compared intravenous immunoglobulin with plasma exchange. In a meta-analysis of five trials involving 536, mostly adult, participants who were unable to walk unaided and had been ill for less than two weeks. The primary outcome measure in this review was the change in a seven grade disability scale four weeks after randomisation. The weighted mean difference of this measure was not statistically significant, being only 0.04 (95% CI -0.26 to 0.19) of a disability grade more improvement in the intravenous immunoglobulin group than the plasma exchange group. There were also no statistically significant differences in time to walk unaided, mortality, and proportion of participants unable to walk without aid after a year. One trial involving 249 participants compared plasma exchange followed by intravenous immunoglobulin with plasma exchange alone, and another involving 37 participants compared immunoabsorption followed by intravenous immunoglobulin with immunoabsorption alone. Neither revealed significant extra benefit from intravenous immunoglobulin. One study of only 39 participants showed a trend towards more improvement with high-dose compared with low-dose intravenous immunoglobulin.
REVIEWER'S CONCLUSIONS: Although there are no adequate comparisons with placebo, intravenous immunoglobulin hastens recovery from Guillain-Barré syndrome as much as plasma exchange. Giving intravenous immunoglobulin after plasma exchange is not significantly better than plasma exchange alone. Randomised trials are needed to decide the effect of intravenous immunoglobulin in children, in adults with mild disease and in adults who start treatment after more than two weeks.

PMID 14973982  Cochrane Database Syst Rev. 2004;(1):CD002063. doi: 10.・・・

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