今日の臨床サポート

しびれ・感覚障害

著者: 興津雅人 東京都立神経病院 脳神経内科

著者: 漆葉章典 東京都立神経病院 脳神経内科

監修: 野口善令 豊田地域医療センター 総合診療科

著者校正済:2022/08/17
現在監修レビュー中
患者向け説明資料

概要・推奨   

  1. しびれは日常的には多彩な症状を含む表現で、一般的には感覚・知覚の症状を指すことが多いが、患者によっては運動麻痺の表現として用いることもあり注意が必要である。
  1. しびれは神経疾患のほか、血管疾患、内分泌疾患、精神疾患、産婦人科疾患など多様な原因で生じる症状であり、症状経過、背景などからまず原因疾患のカテゴリーを特定する必要がある。
  1. 急性発症のしびれでは脳梗塞・出血、脊髄梗塞・出血・脊髄炎などの脊髄病変、血管炎性ニューロパチー、ギラン・バレー症候群といった緊急対応が必要な病態を鑑別する必要がある。
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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。
※同効薬・小児・妊娠および授乳中の注意事項等は、海外の情報も掲載しており、日本の医療事情に適応しない場合があります。
※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、実際の保険適応の査定において保険適応及び保険適応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応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:
興津雅人 : 未申告[2022年]
漆葉章典 : 特に申告事項無し[2022年]
監修:野口善令 : 特に申告事項無し[2022年]

改訂のポイント:
  1. 定期レビューを行い、全面的な書き直しを行った。

病態・疫学・診察

疾患情報(疫学・病態)  
ポイント:
  1. 「しびれ」は、日常的にはさまざまな意味で用いられる言葉である。したがって、診察の際にはできる限りその意味を把握し、適切な医学カテゴリーに分類することが必要になる。特に患者によっては「しびれ」を感覚・知覚の症状でなく運動麻痺の表現として用いることもあり、その場合は必要なアプローチが大きく異なるので注意が必要である。本稿では感覚障害に対応する内容として、「しびれ」について扱う。本稿では感覚障害に対応する内容として、「しびれ」について扱う。
  1. なお、四肢のしびれを訴える患者の割合は人口比で10万人に2,400人、年齢の上昇に伴って8,000人にまで増加するとの報告がある[1]。顔面の神経障害については別項「 顔面神経麻痺(Bell麻痺、Hunt症候群:耳性帯状疱疹) 」を参照にしてほしい。
 
しびれの医学的カテゴリー:
  1. 「しびれ」は一般的には「ジンジンする」「ビリビリする」「チクチクする」と表現される外的刺激に拠らない自発的な感覚を指し、その場合には「異常感覚」の用語が対応する。一方で、外界から与えられた刺激を異なった性質で感じる「錯感覚」、与えられた刺激を強く感じる「感覚過敏(hyperesthesia)」、痛みを起こさないような軽微な刺激を強い痛みとして感じる「異痛症(allodynia)」、さらに与えられた刺激を鈍く感じる「感覚鈍麻(hyp(o)esthesia)」、あるいは与えられた刺激を感じない「感覚消失(anesthesia)」などの場合もしばしば「しびれ」と表現される。
  1. なお「異常感覚」、「錯感覚」に関連して英語ではdysesthesia、paresthesiaという単語があるがこの定義と使い分けについては英語圏でも本邦でも様々な考えがあり統一されていないため、日本神経学会が作成した神経学用語集 第3版では「dysesthesia、paresthesiaに対し、異常感覚、錯感覚のいずれかを対応させることはしない」と記載されている[2][3][4]
 
知覚の伝導路:
  1. 「しびれ」の原因評価は知覚伝導路に沿って考察するとよい。知覚の伝導は、末梢の受容器にて把握された刺激が電気的興奮となって脊髄後根神経を介して脊髄に達した後、位置覚・振動覚は、同側の後索を上昇し内側毛帯となって対側の視床に向かい大脳皮質の知覚野に達する。一方、温痛覚は、後根を介して脊髄に入った後にすぐに対側の外側脊髄視床路になって上行し視床を経て大脳皮質に向かう。大脳では、これらの情報は、中心後回のブロードマン第1-3野が一次中枢となり、その後連合野などにて情報処理がされる。
  1. 「しびれ」の原因は上述の知覚伝導路のいずれの部位でも生じ得るため、原因疾患として、中枢神経疾患(脳血管障害など)、脊髄・神経根疾患(頚椎症、横断性脊髄炎、椎間板ヘルニアなど)、末梢神経疾患(単神経炎、多発神経炎など)などが考えられる。また注意すべき点として、感覚神経以外に病変の主座を有する神経疾患(筋萎縮性側索硬化症、Parkinson病)においても、感覚症状として「しびれ」の訴えが比較的高頻度にあることが近年報告されている[5]。ほかに、全身疾患として、血管疾患(閉塞性動脈硬化症など)、内分泌疾患(甲状腺機能低下症など)、精神疾患(パニック発作、過換気症候群など)、産婦人科疾患(更年期障害)、電解質異常(低Ca血症、低Mg血症、アルカローシスなど)も原因として考慮され、脳神経内科、脳神経外科、整形外科、内分泌内科、血管外科、精神科など多くの科に専門がまたがっている。
 
神経線維の種類:
  1. 神経には有髄のA線維(太い方からα:位置覚、β:触覚・圧覚、γ:位置覚、δ:温痛覚の4群)とB線維(自律神経)、無髄のC線維(交感神経、温痛覚)があり、Aδは 速い痛み(チクッとする痛み:体性痛)と温度覚を、最も細いC線維は遅い痛み(ジーンとする痛み:内臓痛・関連痛)を伝達する。
  1. 患者の訴える「しびれ」が疼痛性か、非疼痛性かによって、主に障害されている神経が前者であれば小径線維系(Aδ、C線維)、後者であれば大径線維系かを大まかに判断することができる[5]
問診・診察のポイント  
問診のポイント:
  1. 問診の際には、しびれという言葉の意味や、しびれの分布、しびれの発症機転、背景因子を確認することが診断に有用である。

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文献 

C N Martyn, R A Hughes
Epidemiology of peripheral neuropathy.
J Neurol Neurosurg Psychiatry. 1997 Apr;62(4):310-8.
Abstract/Text
PMID 9120441
Adrià Arboix, Cristòbal García-Plata, Luis García-Eroles, Joan Massons, Emili Comes, Montserrat Oliveres, Cecilia Targa
Clinical study of 99 patients with pure sensory stroke.
J Neurol. 2005 Feb;252(2):156-62. doi: 10.1007/s00415-005-0622-5.
Abstract/Text OBJECTIVE: We report a clinical description of pure sensory stroke based on data collected from a prospective acute stroke registry.
METHODS: From 2500 acute stroke patients included in a hospital-based prospective stroke registry over a 12-year period, 99 were identified as having pure sensory stroke.
RESULTS: Pure sensory stroke accounted for 4.7% of all acute stroke patients, 5.4% of acute ischemic stroke, and 17.4% of lacunar syndromes. Complete hemisensory syndrome was present in 80 patients and incomplete hemisensory syndrome in 19 (cheiro-oral syndrome 12, cheiro-oral-pedal 6, isolated oral syndrome 1). The lacunar hypothesis was fulfilled in 88% of patients. Atherothrombotic infarction occurred in 8 patients, intracerebral hemorrhage in 3, and stroke of undetermined cause in 1. Hemorrhagic pure sensory stroke was diagnosed in 1% of all cases of hemorrhagic stroke (n = 270) in the database. Outcome was good (in-hospital mortality 0%, symptom-free at discharge 41.5%). After multivariate analysis, absence of disability at discharge, hypertension, diabetes, hyperlipidemia, and thalamic (56.5%) and corona radiata (4%) locations were clinical and topographic variables significantly associated with pure sensory stroke.
CONCLUSIONS: Pure sensory stroke is an infrequent cerebrovascular syndrome, in which the lacunar hypothesis is supported. Most patients had thalamic lacunar infarction. Incomplete hemisensory syndromes were also caused by a lacunar infarct in 84% of patients. Hemorrhagic pure sensory stroke accounted only for 3% of the cases. The prognosis is good with striking similarity to other lacunar strokes. There are important differences between pure sensory stroke and nonlacunar strokes.

PMID 15729520
Nicholas L Zalewski, Alejandro A Rabinstein, Karl N Krecke, Robert D Brown, Eelco F M Wijdicks, Brian G Weinshenker, Timothy J Kaufmann, Jonathan M Morris, Allen J Aksamit, J D Bartleson, Giuseppe Lanzino, Melissa M Blessing, Eoin P Flanagan
Characteristics of Spontaneous Spinal Cord Infarction and Proposed Diagnostic Criteria.
JAMA Neurol. 2019 Jan 1;76(1):56-63. doi: 10.1001/jamaneurol.2018.2734.
Abstract/Text Importance: Spinal cord infarction (SCI) is often disabling, and the diagnosis can be challenging without an inciting event (eg, aortic surgery). Patients with a spontaneous SCI are often misdiagnosed as having transverse myelitis. Diagnostic criteria for SCI are lacking, hindering clinical care and research.
Objective: To describe the characteristics of spontaneous SCI and propose diagnostic criteria.
Design, Setting, and Participants: An institution-based search tool was used to identify patients evaluated at Mayo Clinic, Rochester, Minnesota, from January 1997 to December 2017 with a spontaneous SCI. Patients provided written consent to use their records for research. Participants were 18 years and older with a diagnosis of spontaneous SCI (n = 133), and controls were selected from a database of alternative myelopathy etiologies for validation of the proposed diagnostic criteria (n = 280).
Main Outcomes and Measures: A descriptive analysis of SCI was performed and used to propose diagnostic criteria, and the criteria were validated.
Results: Of 133 included patients with a spontaneous SCI, the median (interquartile range) age at presentation was 60 (52-69) years, and 101 (76%) had vascular risk factors. Rapid onset of severe deficits reaching nadir within 12 hours was typical (102 [77%]); some had a stuttering decline (31 [23%]). Sensory loss occurred in 126 patients (95%), selectively affecting pain/temperature in 49 (39%). Initial magnetic resonance imaging (MRI) spine results were normal in 30 patients (24%). Characteristic MRI T2-hyperintense patterns included owl eyes (82 [65%]) and pencil-like hyperintensity (50 [40%]); gadolinium enhancement (37 of 96 [39%]) was often linear and located in the anterior gray matter. Confirmatory MRI findings included diffusion-weighted imaging/apparent diffusion coefficient restriction (19 of 29 [67%]), adjacent dissection/occlusion (16 of 82 [20%]), and vertebral body infarction (11 [9%]). Cerebrospinal fluid showed mild inflammation in 7 of 89 patients (8%). Diagnostic criteria was proposed for definite, probable, and possible SCI of periprocedural and spontaneous onset. In the validation cohort (n = 280), 9 patients (3%) met criteria for possible SCI, and none met criteria for probable SCI.
Conclusions and Relevance: This large series of spontaneous SCIs provides clinical, laboratory, and MRI clues to SCI diagnosis. The diagnostic criteria proposed here will aid clinicians in making the correct diagnosis and ideally improve future care for patients with SCI. The validation of these criteria supports their utility in the evaluation of acute myelopathy.

PMID 30264146
Alexander E Ropper, Allan H Ropper
Acute Spinal Cord Compression.
N Engl J Med. 2017 Apr 6;376(14):1358-1369. doi: 10.1056/NEJMra1516539.
Abstract/Text
PMID 28379788
Zhan Liu, Qingfang Jiao, Jianguo Xu, Xiang Wang, Sanzhong Li, Chao You
Spontaneous spinal epidural hematoma: analysis of 23 cases.
Surg Neurol. 2008 Mar;69(3):253-60; discussion 260. doi: 10.1016/j.surneu.2007.02.019. Epub 2007 Sep 27.
Abstract/Text BACKGROUND: Spontaneous spinal epidural hematoma is a rare but disabling disease. To explore its characters and find out what factors influence the prognosis, we gave a retrospective analysis of 23 patients in our department in the past 8 years.
METHODS: Spontaneous spinal epidural hematoma was diagnosed by taking MRIs of patients without surgical management or by histopathological examination. We registered patient's case history, laboratory examination, radiological image, treatment, pathological result, and prognosis after 3 months and gave them nonparameter analysis.
RESULTS: Primary neurological status and progressive intervals have correlation with prognosis (P< .01), and the latter less than 12 hours predict worse prognosis (P= .032). Spinal edema in MRI predicts pessimistic prognosis (P= .013). Long hematoma predicts worse prognosis (P= .02). Preoperative interval, emphasized by other authors, has no statistical correlation with prognosis in this study (P= .832). Finally, patients with a single hematoma or hematoma mingled with other spinal disturbance have the same prognosis (P= .065).
CONCLUSIONS: The primary neurological status, progressive interval, spinal edema, and size of hematoma will influence the prognosis of the patient with SSEH. The major treatment is surgical intervention, and it should be operated as soon as possible to avoid the aggravation of neurological status. Conservative treatment is not considered unless patient's neurological deficiency has relieved in the early period.

PMID 17900669
Franz Blaes
Diagnosis and therapeutic options for peripheral vasculitic neuropathy.
Ther Adv Musculoskelet Dis. 2015 Apr;7(2):45-55. doi: 10.1177/1759720X14566617.
Abstract/Text Vasculitis can affect the peripheral nervous system alone (nonsystemic vasculitic neuropathy) or can be a part of primary or secondary systemic vasculitis. In cases of pre-existing systemic vasculitis, the diagnosis can easily be made, whereas suspected vasculitic neuropathy as initial or only manifestation of vasculitis requires careful clinical, neurophysiological, laboratory and histopathological workout. The typical clinical syndrome is mononeuropathia multiplex or asymmetric neuropathy, but distal-symmetric neuropathy can frequently be seen. Standard treatments include steroids, azathioprine, methotrexate and cyclophosphamide. More recently the B-cell antibody rituximab and intravenous immunoglobulins have shown to be effective in some vasculitic neuropathy types.

PMID 25829955
Vera Bril, Hans D Katzberg
Acquired immune axonal neuropathies.
Continuum (Minneap Minn). 2014 Oct;20(5 Peripheral Nervous System Disorders):1261-73. doi: 10.1212/01.CON.0000455882.83803.72.
Abstract/Text PURPOSE OF REVIEW: This article discusses the clinical features, pathophysiology, and management of primary and secondary acquired immune axonal neuropathies.
RECENT FINDINGS: Although there are many collagen vascular disorders associated with vasculitic neuropathy, a quarter of cases have been described to be due to nonsystemic vasculitis of the peripheral nervous system. Enhanced surveillance and aggressive treatment of conditions such as cryoglobulin-related vasculitic neuropathy with cyclophosphamide, rituximab, and alfa interferons has led to improved morbidity and mortality, however, many cases of immune axonal acquired neuropathy are still associated with poor outcomes. Acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) are well-characterized variants of Guillain-Barré syndrome.
SUMMARY: Characterizing the clinical and electrophysiologic phenotype can help diagnose conditions such as nonsystemic vasculitic neuropathy, AMAN, AMSAN, and immune small fiber neuropathy, while careful evaluation of systemic features is key to identifying secondary immune axonal neuropathies such as vasculitic neuropathy related to collagen vascular disease. Additional research is needed to determine the exact immune pathogenesis and optimized treatment regimens for all acquired immune axonal neuropathies.

PMID 25299281
Delia Tulbă, Bogdan Ovidiu Popescu, Emilia Manole, Cristian Băicuș
Immune Axonal Neuropathies Associated With Systemic Autoimmune Rheumatic Diseases.
Front Pharmacol. 2021;12:610585. doi: 10.3389/fphar.2021.610585. Epub 2021 Apr 14.
Abstract/Text Immune axonal neuropathies are a particular group of immune-mediated neuropathies that occasionally accompany systemic autoimmune rheumatic diseases such as connective tissue dissorders and primary systemic vasculitides. Apart from vasculitis of vasa nervorum, various other mechanisms are involved in their pathogenesis, with possible therapeutic implications. Immune axonal neuropathies have highly heterogeneous clinical presentation and course, ranging from mild chronic distal sensorimotor polyneuropathy to severe subacute mononeuritis multiplex with rapid progression and constitutional symptoms such as fever, malaise, weight loss and night sweats, underpinning a vasculitic process. Sensory neuronopathy (ganglionopathy), small fiber neuropathy (sensory and/or autonomic), axonal variants of Guillain-Barré syndrome and cranial neuropathies have also been reported. In contrast to demyelinating neuropathies, immune axonal neuropathies show absent or reduced nerve amplitudes with normal latencies and conduction velocities on nerve conduction studies. Diagnosis and initiation of treatment are often delayed, leading to accumulating disability. Considering the lack of validated diagnostic criteria and evidence-based treatment protocols for immune axonal neuropathies, this review offers a comprehensive perspective on etiopathogenesis, clinical and paraclinical findings as well as therapy guidance for assisting the clinician in approaching these patients. High quality clinical research is required in order to provide indications and follow up rules for treatment in immune axonal neuropathies related to systemic autoimmune rheumatic diseases.

Copyright © 2021 Tulbă, Popescu, Manole and Băicuș.
PMID 33935704
Sonja E Leonhard, Melissa R Mandarakas, Francisco A A Gondim, Kathleen Bateman, Maria L B Ferreira, David R Cornblath, Pieter A van Doorn, Mario E Dourado, Richard A C Hughes, Badrul Islam, Susumu Kusunoki, Carlos A Pardo, Ricardo Reisin, James J Sejvar, Nortina Shahrizaila, Cristiane Soares, Thirugnanam Umapathi, Yuzhong Wang, Eppie M Yiu, Hugh J Willison, Bart C Jacobs
Diagnosis and management of Guillain-Barré syndrome in ten steps.
Nat Rev Neurol. 2019 Nov;15(11):671-683. doi: 10.1038/s41582-019-0250-9. Epub 2019 Sep 20.
Abstract/Text Guillain-Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.

PMID 31541214
Alex Y Doets, Christine Verboon, Bianca van den Berg, Thomas Harbo, David R Cornblath, Hugh J Willison, Zhahirul Islam, Shahram Attarian, Fabio A Barroso, Kathleen Bateman, Luana Benedetti, Peter van den Bergh, Carlos Casasnovas, Guido Cavaletti, Govindsinh Chavada, Kristl G Claeys, Efthimios Dardiotis, Amy Davidson, Pieter A van Doorn, Tom E Feasby, Giuliana Galassi, Kenneth C Gorson, Hans-Peter Hartung, Sung-Tsang Hsieh, Richard A C Hughes, Isabel Illa, Badrul Islam, Susumu Kusunoki, Satoshi Kuwabara, Helmar C Lehmann, James A L Miller, Quazi Deen Mohammad, Soledad Monges, Eduardo Nobile Orazio, Julio Pardo, Yann Pereon, Simon Rinaldi, Luis Querol, Stephen W Reddel, Ricardo C Reisin, Nortina Shahrizaila, Soren H Sindrup, Waheed Waqar, Bart C Jacobs, IGOS Consortium
Regional variation of Guillain-Barré syndrome.
Brain. 2018 Oct 1;141(10):2866-2877. doi: 10.1093/brain/awy232.
Abstract/Text Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrophysiological subtype and outcome. Previous single country reports indicate that Guillain-Barré syndrome may differ among regions, but no systematic comparative studies have been conducted. Comparative studies are required to identify factors determining disease susceptibility, variation and prognosis, and to improve diagnostic criteria. The International Guillain-Barré Syndrome Outcome Study is a prospective, observational cohort study including all patients within the diagnostic spectrum, aiming to describe the heterogeneity of Guillain-Barré syndrome worldwide. The current study was based on the first 1000 inclusions with a follow-up of at least 1 year and confirmed the variation in clinical presentation, course and outcome between patients. The full clinical spectrum of Guillain-Barré syndrome was observed in patients from all countries participating in the International Guillain-Barré Syndrome Outcome Study, but the frequency of variants differed between regions. We compared three regions based on geography, income and previous reports of Guillain-Barré syndrome subtypes: 'Europe/Americas', 'Asia' (without Bangladesh), and 'Bangladesh'. We excluded 75 (8%) patients because of alternative diagnoses, protocol violations, or missing data. The predominant clinical variant was sensorimotor in Europe/Americas (n = 387/562, 69%) and Asia (n = 27/63, 43%), and pure motor in Bangladesh (n = 74/107, 69%). Miller Fisher syndrome and Miller Fisher-Guillain-Barré overlap syndrome were more common in Asia (n = 14/63, 22%) than in the other two regions (Europe/Americas: n = 64/562, 11%; Bangladesh: n = 1/107, 1%) (P < 0.001). The predominant electrophysiological subtype was demyelinating in all regions (Europe/Americas: n = 312/573, 55%; Asia: n = 29/65, 45%; Bangladesh: n = 38/94, 40%). The axonal subtype occurred more often in Bangladesh (n = 34/94, 36%) than in Europe/Americas (n = 33/573, 6%) and other Asian countries (n = 4/65, 6%) (P < 0.001). In all regions, patients with the axonal subtype were younger, had fewer sensory deficits, and showed a trend towards poorer recovery compared to patients with the demyelinating subtype. The proportion of patients able to walk unaided after 1 year varied between Asia (n = 31/34, 91%), Europe/Americas (n = 334/404, 83%) and Bangladesh (n = 67/97, 69%) (P = 0.003). A similar variation was seen for mortality, being higher in Bangladesh (n = 19/114, 17%) than in Europe/Americas (n = 23/486, 5%) and Asia (n = 1/45, 2%) (P < 0.001). This study showed that factors related to geography have a major influence on clinical phenotype, disease severity, electrophysiological subtype, and outcome of Guillain-Barré syndrome.

PMID 30247567
Christa Walgaard, Hester F Lingsma, Liselotte Ruts, Judith Drenthen, Rinske van Koningsveld, Marcel J P Garssen, Pieter A van Doorn, Ewout W Steyerberg, Bart C Jacobs
Prediction of respiratory insufficiency in Guillain-Barré syndrome.
Ann Neurol. 2010 Jun;67(6):781-7. doi: 10.1002/ana.21976.
Abstract/Text OBJECTIVE: Respiratory insufficiency is a frequent and serious complication of the Guillain-Barré syndrome (GBS). We aimed to develop a simple but accurate model to predict the chance of respiratory insufficiency in the acute stage of the disease based on clinical characteristics available at hospital admission.
METHODS: Mechanical ventilation (MV) in the first week of admission was used as an indicator of acute stage respiratory insufficiency. Prospectively collected data from a derivation cohort of 397 GBS patients were used to identify predictors of MV. A multivariate logistic regression model was validated in a separate cohort of 191 GBS patients. Model performance criteria comprised discrimination (area under receiver operating curve [AUC]) and calibration (graphically). A scoring system for clinical practice was constructed from the regression coefficients of the model in the combined cohorts.
RESULTS: In the derivation cohort, 22% needed MV in the first week of admission. Days between onset of weakness and admission, Medical Research Council sum score, and presence of facial and/or bulbar weakness were the main predictors of MV. The prognostic model had a good discriminative ability (AUC, 0.84). In the validation cohort, 14% needed MV in the first week of admission, and both calibration and discriminative ability of the model were good (AUC, 0.82). The scoring system ranged from 0 to 7, with corresponding chances of respiratory insufficiency from 1 to 91%.
INTERPRETATION: This model accurately predicts development of respiratory insufficiency within 1 week in patients with GBS, using clinical characteristics available at admission. After further validation, the model may assist in clinical decision making, for example, on patient transfer to an intensive care unit.

PMID 20517939
L Ruts, J Drenthen, B C Jacobs, P A van Doorn, Dutch GBS Study Group
Distinguishing acute-onset CIDP from fluctuating Guillain-Barre syndrome: a prospective study.
Neurology. 2010 May 25;74(21):1680-6. doi: 10.1212/WNL.0b013e3181e07d14. Epub 2010 Apr 28.
Abstract/Text OBJECTIVE: The aim of the study was to provide criteria that can help to distinguish between GBS-TRF and A-CIDP in the early phase of disease.
BACKGROUND: The distinction between Guillain-Barré syndrome (GBS) with fluctuations shortly after start of treatment (treatment-related fluctuations, or GBS-TRF) and chronic inflammatory demyelinating polyneuropathy with acute onset (A-CIDP) is difficult but important because prognosis and treatment strategy largely differ.
METHODS: Patients with GBS (n = 170) were included in a prospective longitudinal study. Patients with GBS-TRF (n = 16) and patients with A-CIDP (n = 8) were analyzed and compared. Extended clinical data, biologic material, and electrophysiologic data were collected during 1 year follow-up.
RESULTS: The first TRF in the GBS-TRF group always occurred within 8 weeks (median 18 days; range 10-54 days) from onset of weakness. In the GBS-TRF group, 5 (31%) patients had a second TRF and none had more TRFs. At all timepoints, patients in the A-CIDP group were less severely affected than patients with GBS-TRF, did not need artificial ventilation, rarely had cranial nerve dysfunction, and tended to have more CIDP-like electrophysiologic abnormalities. More GBS-TRF patients were severely affected and more patients had sensory disturbances when compared to the GBS group without fluctuations.
CONCLUSIONS: The diagnosis of acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP) should be considered when a patient thought to have Guillain-Barré syndrome deteriorates again after 8 weeks from onset or when deterioration occurs 3 times or more. Especially when the patient remains able to walk independently and has no cranial nerve dysfunction or electrophysiologic features likely to be compatible with CIDP, maintenance treatment for CIDP should be considered.

PMID 20427754
Peter Y K Van den Bergh, Pieter A van Doorn, Robert D M Hadden, Bert Avau, Patrik Vankrunkelsven, Jeffrey A Allen, Shahram Attarian, Patricia H Blomkwist-Markens, David R Cornblath, Filip Eftimov, H Stephan Goedee, Thomas Harbo, Satoshi Kuwabara, Richard A Lewis, Michael P Lunn, Eduardo Nobile-Orazio, Luis Querol, Yusuf A Rajabally, Claudia Sommer, Haluk A Topaloglu
European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision.
J Peripher Nerv Syst. 2021 Sep;26(3):242-268. doi: 10.1111/jns.12455. Epub 2021 Jul 30.
Abstract/Text To revise the 2010 consensus guideline on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Seventeen disease experts, a patient representative, and two Cochrane methodologists constructed 12 Population/Intervention/Comparison/Outcome (PICO) questions regarding diagnosis and treatment to guide the literature search. Data were extracted and summarized in GRADE summary of findings (for treatment PICOs) or evidence tables (for diagnostic PICOs). Statements were prepared according to the GRADE Evidence-to-Decision frameworks. Typical CIDP and CIDP variants were distinguished. The previous term "atypical CIDP" was replaced by "CIDP variants" because these are well characterized entities (multifocal, focal, distal, motor, or sensory CIDP). The levels of diagnostic certainty were reduced from three (definite, probable, possible CIDP) to only two (CIDP and possible CIDP), because the diagnostic accuracy of criteria for probable and definite CIDP did not significantly differ. Good Practice Points were formulated for supportive criteria and investigations to be considered to diagnose CIDP. The principal treatment recommendations were: (a) intravenous immunoglobulin (IVIg) or corticosteroids are strongly recommended as initial treatment in typical CIDP and CIDP variants; (b) plasma exchange is strongly recommended if IVIg and corticosteroids are ineffective; (c) IVIg should be considered as first-line treatment in motor CIDP (Good Practice Point); (d) for maintenance treatment, IVIg, subcutaneous immunoglobulin or corticosteroids are recommended; (e) if the maintenance dose of any of these is high, consider either combination treatments or adding an immunosuppressant or immunomodulatory drug (Good Practice Point); and (f) if pain is present, consider drugs against neuropathic pain and multidisciplinary management (Good Practice Point).

© 2021 European Academy of Neurology and Peripheral Nerve Society.
PMID 34085743
James C Watson, P James B Dyck
Peripheral Neuropathy: A Practical Approach to Diagnosis and Symptom Management.
Mayo Clin Proc. 2015 Jul;90(7):940-51. doi: 10.1016/j.mayocp.2015.05.004.
Abstract/Text Peripheral neuropathy is one of the most prevalent neurologic conditions encountered by physicians of all specialties. Physicians are faced with 3 distinct challenges in caring for patients with peripheral neuropathy: (1) how to efficiently and effectively screen (in less than 2 minutes) an asymptomatic patient for peripheral neuropathy when they have a disorder in which peripheral neuropathy is highly prevalent (eg, diabetes mellitus), (2) how to clinically stratify patients presenting with symptoms of neuropathy to determine who would benefit from specialty consultation and what testing is appropriate for those who do not need consultation, and (3) how to treat the symptoms of painful peripheral neuropathy. In this concise review, we address these 3 common clinical scenarios. Easily defined clinical patterns of involvement are used to identify patients in need of neurologic consultation, the yield of laboratory and other diagnostic testing is reviewed for the evaluation of length-dependent, sensorimotor peripheral neuropathies (the most common form of neuropathy), and an algorithmic approach with dosing recommendations is provided for the treatment of neuropathic pain associated with peripheral neuropathy.

Copyright © 2015 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.
PMID 26141332
M Camerlingo, R Nemni, B Ferraro, L Casto, T Partziguian, B Censori, A Mamoli
Malignancy and sensory neuropathy of unexplained cause: a prospective study of 51 patients.
Arch Neurol. 1998 Jul;55(7):981-4.
Abstract/Text OBJECTIVE: To investigate the frequency of cancer developing in patients with peripheral sensory neuropathy of unexplained cause.
DESIGN: Prospective study.
SETTING: A neurologic unit in a general hospital.
METHODS: Following the diagnosis of neuropathy, we searched for occult malignancy. This search was repeated together with neurologic evaluations every 6 months thereafter. Patient recruitment began January 1, 1988, and ended December 31, 1995. The end point of the study was December 31, 1996.
RESULTS: In the study period, we observed 363 patients with peripheral sensory neuropathy. Of these, 53 patients without any identified cause of neuropathy were invited to participate in the study. Of the 53, 2 patients refused. Thus, we examined and followed up 51 patients, 42 men and 9 women, with a mean age of 64.5 years (range, 19-80 years). The range between the onset of neurologic symptoms and the diagnosis of neuropathy was 2 to 72 months (mean, 13.9 months). The follow-up period ranged from 14 to 94 months (mean, 51.4 months). In 18 patients (35.3%) (16 men and 2 women) whose mean age at diagnosis of neuropathy was 66.5 years. malignant growths were found 3 to 72 months (mean, 27.4 months) after the onset of the neuropathy. The cancer was in the liver in 4 patients (all had a primary hepatoma), the bladder in 3, the lymph nodes in 3 (all with non-Hodgkin lymphoma), the prostate gland in 2, the lungs in 2 (small cell lung cancer in both), the breast in 1, the pancreas in 1, the sublingual gland in 1, and the bone in 1 (a metastatic sarcoma).
CONCLUSIONS: More than one third of the patients with peripheral sensory neuropathy of unexplained cause developed cancer without any predominating type of malignancy.

PMID 9678316
Nanna B Finnerup, Nadine Attal, Simon Haroutounian, Ewan McNicol, Ralf Baron, Robert H Dworkin, Ian Gilron, Maija Haanpää, Per Hansson, Troels S Jensen, Peter R Kamerman, Karen Lund, Andrew Moore, Srinivasa N Raja, Andrew S C Rice, Michael Rowbotham, Emily Sena, Philip Siddall, Blair H Smith, Mark Wallace
Pharmacotherapy for neuropathic pain in adults: a systematic review and meta-analysis.
Lancet Neurol. 2015 Feb;14(2):162-73. doi: 10.1016/S1474-4422(14)70251-0. Epub 2015 Jan 7.
Abstract/Text BACKGROUND: New drug treatments, clinical trials, and standards of quality for assessment of evidence justify an update of evidence-based recommendations for the pharmacological treatment of neuropathic pain. Using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE), we revised the Special Interest Group on Neuropathic Pain (NeuPSIG) recommendations for the pharmacotherapy of neuropathic pain based on the results of a systematic review and meta-analysis.
METHODS: Between April, 2013, and January, 2014, NeuPSIG of the International Association for the Study of Pain did a systematic review and meta-analysis of randomised, double-blind studies of oral and topical pharmacotherapy for neuropathic pain, including studies published in peer-reviewed journals since January, 1966, and unpublished trials retrieved from ClinicalTrials.gov and websites of pharmaceutical companies. We used number needed to treat (NNT) for 50% pain relief as a primary measure and assessed publication bias; NNT was calculated with the fixed-effects Mantel-Haenszel method.
FINDINGS: 229 studies were included in the meta-analysis. Analysis of publication bias suggested a 10% overstatement of treatment effects. Studies published in peer-reviewed journals reported greater effects than did unpublished studies (r(2) 9·3%, p=0·009). Trial outcomes were generally modest: in particular, combined NNTs were 6·4 (95% CI 5·2-8·4) for serotonin-noradrenaline reuptake inhibitors, mainly including duloxetine (nine of 14 studies); 7·7 (6·5-9·4) for pregabalin; 7·2 (5·9-9·21) for gabapentin, including gabapentin extended release and enacarbil; and 10·6 (7·4-19·0) for capsaicin high-concentration patches. NNTs were lower for tricyclic antidepressants, strong opioids, tramadol, and botulinum toxin A, and undetermined for lidocaine patches. Based on GRADE, final quality of evidence was moderate or high for all treatments apart from lidocaine patches; tolerability and safety, and values and preferences were higher for topical drugs; and cost was lower for tricyclic antidepressants and tramadol. These findings permitted a strong recommendation for use and proposal as first-line treatment in neuropathic pain for tricyclic antidepressants, serotonin-noradrenaline reuptake inhibitors, pregabalin, and gabapentin; a weak recommendation for use and proposal as second line for lidocaine patches, capsaicin high-concentration patches, and tramadol; and a weak recommendation for use and proposal as third line for strong opioids and botulinum toxin A. Topical agents and botulinum toxin A are recommended for peripheral neuropathic pain only.
INTERPRETATION: Our results support a revision of the NeuPSIG recommendations for the pharmacotherapy of neuropathic pain. Inadequate response to drug treatments constitutes a substantial unmet need in patients with neuropathic pain. Modest efficacy, large placebo responses, heterogeneous diagnostic criteria, and poor phenotypic profiling probably account for moderate trial outcomes and should be taken into account in future studies.
FUNDING: NeuPSIG of the International Association for the Study of Pain.

Copyright © 2015 Elsevier Ltd. All rights reserved.
PMID 25575710
Wayne A Ray, Sarah Meredith, Purushottam B Thapa, Kathi Hall, Katherine T Murray
Cyclic antidepressants and the risk of sudden cardiac death.
Clin Pharmacol Ther. 2004 Mar;75(3):234-41. doi: 10.1016/j.clpt.2003.09.019.
Abstract/Text BACKGROUND: Tricyclic and other related cyclic antidepressants (TCAs), used frequently for the treatment of depression and several other indications, have cardiovascular effects that may increase the risk of sudden cardiac death. We thus sought to quantify the risk of sudden cardiac death among TCA users, according to dose, as well as among users of selective serotonin reuptake inhibitors (SSRIs).
METHODS: We conducted a retrospective cohort study in Tennessee Medicaid, from Jan 1, 1988, through Dec 31, 1993, which included large numbers of antidepressant users and computer files describing medication use and comorbidity. The cohort included 1,282,091 person-years of follow-up for persons aged 15 to 84 years who were not in a nursing home and were free of life-threatening noncardiac illness. This included 58,956 person-years for current use of TCAs alone, 6291 person-years for SSRIs only, and 96,220 person-years for former use.
RESULTS: The cohort included 1487 confirmed sudden cardiac deaths occurring in the community. When compared with nonusers of antidepressants, current users of TCAs had a dose-related increase in the risk of sudden cardiac death. Rate ratios increased from 0.97 (95% confidence interval [CI], 0.72-1.29) for doses lower than 100 mg (amitriptyline or its equivalent) to 2.53 (95% CI, 1.04-6.12) for doses of 300 mg or more (P =.03, test for dose-response). The rate ratio for SSRIs was 0.95 (95% CI, 0.42-2.15). There was no evidence that TCA doses lower than 100 mg increased the risk of sudden cardiac death in subgroups defined by pre-existing cardiovascular disease, female sex, age 65 years or older, or use of amitriptyline.
CONCLUSIONS: Our data suggest that SSRI antidepressants and TCAs in doses of less than 100 mg (amitriptyline equivalents) did not increase the risk of sudden cardiac death. However, higher doses of TCAs were associated with increased relative risk, which suggests that such doses should be used cautiously, particularly in patients with an elevated baseline risk of sudden death.

PMID 15001975

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