今日の臨床サポート

対麻痺

著者: 福武敏夫 亀田メディカルセンター 脳神経内科

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:2021/01/28
現在監修レビュー中
患者向け説明資料

概要・推奨   

  1. 頚椎症性脊髄症の術後の転帰を判断するには、臨床的要素や感覚誘発電位検査をもとにすることが勧められる。
  1. 頚椎症性脊髄症患者では、経過、年齢、筋電図検査などによる症状把握が推奨される。
  1. 圧迫性頚髄症の術後予後判定に術前・術後のMRIが有用である。
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  1. 中年の脊髄障害患者では、ビタミンB12の測定が推奨される。
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  1. 血清ビタミンB12のカットオフ値にはコンセンサスがないが、脊髄障害患者では測定と早期治療が望まれる。
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:
福武敏夫 : 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、主に画像所見について加筆修正し、MOG抗体について追記した。

病態・疫学・診察

疫学情報・病態・注意事項  
  1. 対麻痺とは両下肢の、対称性の運動麻痺(筋力低下)のことであり、歩行障害として現れる。その意味では本来、症候を指すが、家族性痙性対麻痺のように、病名として用いられることもある。
  1. 病変部位は通常、頚髄下部から腰髄の脊髄にあり、例外的に大脳や末梢神経に求められる。筋原性の両下肢脱力は対麻痺とはいわない。
  1. 上位運動ニューロン、すなわち錐体路を構成する皮質脊髄路の障害では、腱反射の亢進や筋緊張の痙性(痙縮性)も現れ、下位運動ニューロン障害では、筋萎縮や筋緊張の弛緩性も現れる。
  1. 伴い得る主な合併症候は、さまざまの体性感覚症候や膀胱直腸障害である。
  1. 主な内科的原因には、感染性ないし炎症性脊髄炎、HTLV-I関連脊髄症、視神経脊髄炎(NMO)、多発性硬化症(MS)、ビタミンB12欠乏症(亜急性連合性脊髄変性症)、甲状腺機能亢進症(低K性周期性四肢麻痺)、家族性(遺伝性)痙性対麻痺がある。ときに銅欠乏性脊髄症、ヘモジデリン沈着症、アルコール性脊髄症、膵性脊髄症、血管内悪性リンパ腫症、ホモシスチン尿症、潜函病性脊髄症、両側前頭葉病変(大脳鎌髄膜腫、他)、ポリオ、なども原因となる。
  1. 手術もあり得る外科的な原因には、変形性脊椎症・椎間板ヘルニア、脊髄外傷、脊髄腫瘍、脊髄空洞症がある。脊髄血管障害には内科的病態と外科的病態が含まれる。
 
  1. 遺伝性痙性対麻痺は下肢の進行性の筋力低下と痙性によって特徴づけられる遺伝的に規定される疾患であり、大多数の臨床的・遺伝学的に不均一な疾患に含まれる。(参考文献:[1][2][3][4]
  1. 現在、59の対応する痙性対麻痺遺伝子を伴う79の異なる痙性歩行疾患の遺伝子座があり、すべての遺伝様式がある。SPG4が最も多い。
  1. 染色体劣性遺伝のものは多様な他の症状を伴い、複雑型といわれ、優性遺伝のものはたいてい純粋型である。
  1. 精神遅滞とMRI上の脳梁の非薄化はSPG11とSPG15を示唆する。
  1. 次世代シーケンスの導入により、他の神経変性疾患(筋萎縮性側索硬化症やニューロパチー、小脳性運動失調など)との遺伝子的・表現型的オーバーラップが明らかになっている。
  1. 神経病理学的検討は限られているが、軸索変性が皮質脊髄路と薄束の遠位端に及んでいる。
問診・診察のポイント  
  1. 最初の大きな目標は外科的原因かどうか、さらに緊急手術が必要かどうかを明らかにすることであるが、問診・診察で絞り込んだ後でMRI検査が必要となることが多い。

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文献 

著者: John K Fink
雑誌名: Semin Neurol. 2014 Jul;34(3):293-305. doi: 10.1055/s-0034-1386767. Epub 2014 Sep 5.
Abstract/Text Hereditary spastic paraplegia (HSP) refers to inherited disorders in which spastic gait is either the only feature or is a major syndrome feature. There are more than 70 genetic types of HSP. Neuropathological studies, albeit limited to only a few genetic types of HSP, have identified axon degeneration involving the distal ends of the corticospinal tracts and fasciculus gracilis fibers. In this review, the author highlights the clinical and genetic features of HSP.

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
PMID 25192507  Semin Neurol. 2014 Jul;34(3):293-305. doi: 10.1055/s-00・・・
著者: S Klebe, G Stevanin, C Depienne
雑誌名: Rev Neurol (Paris). 2015 Jun-Jul;171(6-7):505-30. doi: 10.1016/j.neurol.2015.02.017. Epub 2015 May 23.
Abstract/Text Hereditary spastic paraplegias (HSPs) are genetically determined neurodegenerative disorders characterized by progressive weakness and spasticity of lower limbs, and are among the most clinically and genetically heterogeneous human diseases. All modes of inheritance have been described, and the recent technological revolution in molecular genetics has led to the identification of 76 different spastic gait disease-loci with 59 corresponding spastic paraplegia genes. Autosomal recessive HSP are usually associated with diverse additional features (referred to as complicated forms), contrary to autosomal dominant HSP, which are mostly pure. However, the identification of additional mutations and families has considerably enlarged the clinical spectra, and has revealed a huge clinical variability for almost all HSP; complicated forms have also been described for primary pure HSP subtypes, adding further complexity to the genotype-phenotype correlations. In addition, the introduction of next generation sequencing in clinical practice has revealed a genetic and phenotypic overlap with other neurodegenerative disorders (amyotrophic lateral sclerosis, neuropathies, cerebellar ataxias, etc.) and neurodevelopmental disorders, including intellectual disability. This review aims to describe the most recent advances in the field and to provide genotype-phenotype correlations that could help clinical diagnoses of this heterogeneous group of disorders.

Copyright © 2015 Elsevier Masson SAS. All rights reserved.
PMID 26008818  Rev Neurol (Paris). 2015 Jun-Jul;171(6-7):505-30. doi: ・・・
著者: Adam, Ardinger, Pagon, Wallace, Bean, Stephens, Amemiya
Abstract/Text The purpose of this overview is to increase the awareness of clinicians regarding hereditary spastic paraplegia. The following are the goals of this overview. GOAL 1: Describe the clinical characteristics of hereditary spastic paraplegia and recommended treatment. GOAL 2: Review the causes of hereditary spastic paraplegia. GOAL 3: Consider the differential diagnosis of hereditary spastic paraplegia. GOAL 4: Provide an evaluation strategy to identify the genetic cause of hereditary spastic paraplegia in a proband. GOAL 5: Inform genetic counseling of family members of a proband with hereditary spastic paraplegia.

PMID 20301682  
著者: Samuel Shribman, Evan Reid, Andrew H Crosby, Henry Houlden, Thomas T Warner
雑誌名: Lancet Neurol. 2019 Dec;18(12):1136-1146. doi: 10.1016/S1474-4422(19)30235-2. Epub 2019 Jul 31.
Abstract/Text Hereditary spastic paraplegia (HSP) describes a heterogeneous group of genetic neurodegenerative diseases characterised by progressive spasticity of the lower limbs. The pathogenic mechanism, associated clinical features, and imaging abnormalities vary substantially according to the affected gene and differentiating HSP from other genetic diseases associated with spasticity can be challenging. Next generation sequencing-based gene panels are now widely available but have limitations and a molecular diagnosis is not made in most suspected cases. Symptomatic management continues to evolve but with a greater understanding of the pathophysiological basis of individual HSP subtypes there are emerging opportunities to provide targeted molecular therapies and personalised medicine.

Copyright © 2019 Elsevier Ltd. All rights reserved.
PMID 31377012  Lancet Neurol. 2019 Dec;18(12):1136-1146. doi: 10.1016/・・・
著者: Han Jo Kim, Lindsay A Tetreault, Eric M Massicotte, Paul M Arnold, Andrea C Skelly, Erika D Brodt, K Daniel Riew
雑誌名: Spine (Phila Pa 1976). 2013 Oct 15;38(22 Suppl 1):S78-88. doi: 10.1097/BRS.0b013e3182a7eb06.
Abstract/Text STUDY DESIGN: Literature review.
OBJECTIVE: To identify case series that have been confused with cervical spondylotic myelopathy (CSM) to develop a comprehensive differential diagnosis.
SUMMARY OF BACKGROUND DATA: Myelopathy can be caused by a number of different etiologies. In those patients with CSM, the presentation is not always clear. Distinct radiographical and clinical characteristics, which are not always obvious, aid in arriving at the correct diagnosis.
METHODS: A PubMed search was done to identify reports written in English describing conditions that may present in a manner similar to CSM to differentiate them from CSM. Material from review articles and relevant textbooks was also considered. Information regarding the number of patients, the specific diagnosis presenting as myelopathy, the diagnostic findings, and the method(s) for distinguishing CSM from the initial diagnosis was abstracted from included articles. Salient features of the conditions were summarized.
RESULTS: A total of 35 citations (totaling 474 patients) that reported on diagnoses confused with CSM based on clinical presentation were included. All were case reports or small case series. The differential diagnoses were organized into 7 categories: congenital/anatomic, degenerative, neoplastic, inflammatory/autoimmune, idiopathic, circulatory, and metabolic. The primary conditions in the differential included amyotrophic lateral sclerosis, multiple sclerosis, syringomyelia, and spinal tumors.
CONCLUSION: In the vast majority of cases, magnetic resonance imaging was an invaluable tool in determining the correct diagnosis. Electrodiagnostic studies, cerebrospinal fluid profile, unique symptomatology, and consideration of patient demographics can also aid in the diagnosis. Bilateral sensory complaints in the hands are suspicious for cervical cord pathology and MR imaging of the same should be done even if the electromyography/nerve conduction studies (NCS) suggest bilateral carpal tunnel syndrome. SUMMARY STATEMENTS: Physical exam findings are not always consistent with severity of disease in CSM; therefore, correlation to plain radiographs, MRI, and patient symptomatology is essential for arriving at the correct diagnosis. In some cases where these studies are still equivocal, use of other studies should be considered including electrodiagnostic studies as well as cerebrospinal fluid examination.

PMID 23962997  Spine (Phila Pa 1976). 2013 Oct 15;38(22 Suppl 1):S78-8・・・
著者: Chad E Cook, Eric Hegedus, Ricardo Pietrobon, Adam Goode
雑誌名: Phys Ther. 2007 Sep;87(9):1233-42. doi: 10.2522/ptj.20060150. Epub 2007 Jul 17.
Abstract/Text Physical therapists commonly use screening tests to identify upper motoneuron lesions such as cord compressive myelopathy (CCM), the presence of which necessitates appropriate medical referral. Signs and symptoms of CCM include sensory and ataxic changes of the lower extremities, poorly coordinated gait, weakness, tetraspasticity, clumsiness, spasticity, hyperreflexia, and primitive reflexes. Clinical tests and measures such as Hoffmann sign, clonus, Lhermitte sign, the grip and release test, the finger escape sign, the Babinski test, and the inverted supinator sign have historically been used as screens for CCM. For effectiveness as a screen, a clinical test or measure should demonstrate high sensitivity. Diagnostic accuracy studies have shown that clinical tests and measures for CCM often display low sensitivity, indicating that a negative finding may falsely suggest the absence of a condition or disease that actually is present. To counter the low levels of sensitivity, screening should include a combination of a thorough patient history, recognition of and appropriate referral for cauda equina symptoms, and clusters of any pertinent contributory tests and measures.

PMID 17636158  Phys Ther. 2007 Sep;87(9):1233-42. doi: 10.2522/ptj.200・・・
著者: P A Kempster, R D Rollinson
雑誌名: J Clin Neurosci. 2008 Apr;15(4):379-81. doi: 10.1016/j.jocn.2007.05.002. Epub 2008 Feb 14.
Abstract/Text Typical Lhermitte phenomenon (tingling sensations moving down the limbs or trunk on neck flexion) is a sign of intrinsic spinal cord pathology, most commonly cervical spinal cord demyelination. The phenomenon has several variant forms, and each has a different pathological significance. A delayed typical Lhermitte phenomenon can follow contusion of the spinal cord from neck trauma. Reverse Lhermitte phenomenon induced by neck extension is usually produced by extrinsic compression of the cervical spinal cord. Upward moving paraesthesia with neck flexion (inverse Lhermitte phenomenon) is relatively rare, and can be a sign of myelopathy from nitrous oxide inhalation.

PMID 18280165  J Clin Neurosci. 2008 Apr;15(4):379-81. doi: 10.1016/j.・・・
著者: W Qiu, J-S Wu, M-N Zhang, T Matsushita, J-i Kira, W M Carroll, F L Mastaglia, A G Kermode
雑誌名: J Neurol Neurosurg Psychiatry. 2010 Feb;81(2):209-12. doi: 10.1136/jnnp.2009.172973. Epub 2009 Aug 25.
Abstract/Text OBJECTIVES: To characterise West Australian cases of longitudinally extensive myelopathy (LEM).
METHODS: Twenty six patients with LEM were identified from a cohort of 983 patients with demyelinating disease. Clinical and MRI data and AQP4-IgG results were reviewed.
RESULTS: LEM cases were classified as conventional MS (CMS) 13, neuromyelitis optica (NMO) 7, and isolated LEM 6. LEM was the initial presentation in 13/26 cases. In CMS cases lesions were mainly in the lower cervical cord (C4-C7) whereas in NMO and isolated LEM they were more often thoracic and were longer. The severity of disability was highly variable but was greater in the NMO than the CMS group. Only one of 20 patients tested was seropositive for AQP4-IgG.
CONCLUSION: LEM occurred as part of CMS or NMO or in isolation. Patients with LEM had highly heterogeneous clinical characteristics and a low rate of AQP4-IgG seropositivity.

PMID 19710049  J Neurol Neurosurg Psychiatry. 2010 Feb;81(2):209-12. d・・・
著者: Mario Habek, Ivan Adamec, Goran Pavliša, Vesna V Brinar
雑誌名: Acta Neurol Belg. 2012 Mar;112(1):39-43. doi: 10.1007/s13760-012-0006-4. Epub 2012 Jan 26.
Abstract/Text The aim of this study is to present a diagnostic and therapeutic approach in patients with LETM. In a period between June 2008 and June 2010, all patients who fulfilled criteria for LETM were included in the study. All patients underwent a standardized protocol of investigations presented in this paper. Ten patients were included (5 male, 5 female, with the age distribution from 24 to 70 years). Four patients were diagnosed with NMO/spatially limited NMO spectrum disorder, three patients were diagnosed with spinal cord ADEM, two multiple sclerosis (MS) and one patient with copper deficiency myelopathy. Laboratory support for the diagnosis of NMO was positive NMO-IgG antibody; for the diagnosis of ADEM signs of peripheral nervous system involvement on electromyoneurography; and for the diagnosis of MS brain MRI lesions typical for MS, as well as positive oligoclonal bands (OCB) in the cerebrospinal fluid (CSF). All cases with inflammatory myelopathy were treated either with steroids or plasma exchange and copper replacement was started in the case of copper deficiency. The mean time from the first symptom until the final diagnosis was 16.3 months (range 1 month to 7 years). As each of idiopathic inflammatory demyelinating diseases that can present with LETM have specific therapy, the postponement in making the correct diagnosis can lead to a poor recovery. In patients with LETM, a standardized diagnostic approach can result in a correct diagnosis and appropriate treatment.

PMID 22427288  Acta Neurol Belg. 2012 Mar;112(1):39-43. doi: 10.1007/s・・・
著者: A Merwick, S S O'Sullivan, K N O'Regan, C J Marks, D Q Ryders, B J Sweeney
雑誌名: Ir Med J. 2008 Jan;101(1):21-2.
Abstract/Text Imaging of the spine is a fundamental part of assessment of paraparesis. Since the advent of MRI the indications for myelograms have diminished. However, a myelogram, although an invasive test, should still be considered a useful investigation for localising lesions in the spinal cord and for identifying rare causes of myelopathy. This case illustrates how a CT myelogram identified an arachnoid cyst, which is a potentially treatable cause of paraparesis.

PMID 18369020  Ir Med J. 2008 Jan;101(1):21-2.
著者: Langston T Holly, Paul G Matz, Paul A Anderson, Michael W Groff, Robert F Heary, Michael G Kaiser, Praveen V Mummaneni, Timothy C Ryken, Tanvir F Choudhri, Edward J Vresilovic, Daniel K Resnick, Joint Section on Disorders of the Spine and Peripheral Nerves of the American Association of Neurological Surgeons and Congress of Neurological Surgeons
雑誌名: J Neurosurg Spine. 2009 Aug;11(2):112-8. doi: 10.3171/2009.1.SPINE08718.
Abstract/Text OBJECT: The objective of this systematic review was to use evidence-based medicine to assess whether clinical factors predict surgical outcomes in patients undergoing cervical surgery.
METHODS: The National Library of Medicine and Cochrane Database were queried using MeSH headings and keywords relevant to clinical preoperative factors. Abstracts were reviewed, and studies that met the inclusion criteria were selected. The guidelines group assembled an evidentiary table summarizing the quality of evidence (Classes I-III). Disagreements regarding the level of evidence were resolved through an expert consensus conference. The group formulated recommendations that contained the degree of strength based on the Scottish Intercollegiate Guidelines network. Validation was done through peer review by the Joint Guidelines Committee of the American Association of Neurological Surgeons/Congress of Neurological Surgeons.
RESULTS: Preoperative sensory-evoked potentials may aid in providing prognostic information in selected patients in whom clinical factors do not provide clear guidance (Class II). Age, duration of symptoms, and preoperative neurological function may commonly affect outcome (Class III).
CONCLUSIONS: Age, duration of symptoms, and preoperative neurological function should be discussed with patients when surgical intervention for cervical spondylotic myelopathy is considered. Preoperative sensory-evoked potentials may be considered for patients in whom clinical factors do not provide clear guidance if such information would potentially change therapeutic decisions.

PMID 19769490  J Neurosurg Spine. 2009 Aug;11(2):112-8. doi: 10.3171/2・・・
著者: Paul G Matz, Paul A Anderson, Langston T Holly, Michael W Groff, Robert F Heary, Michael G Kaiser, Praveen V Mummaneni, Timothy C Ryken, Tanvir F Choudhri, Edward J Vresilovic, Daniel K Resnick, Joint Section on Disorders of the Spine and Peripheral Nerves of the American Association of Neurological Surgeons and Congress of Neurological Surgeons
雑誌名: J Neurosurg Spine. 2009 Aug;11(2):104-11. doi: 10.3171/2009.1.SPINE08716.
Abstract/Text OBJECT: The objective of this systematic review was to use evidence-based medicine to delineate the natural history of cervical spondylotic myelopathy (CSM) and identify factors associated with clinical deterioration.
METHODS: The National Library of Medicine and Cochrane Database were queried using MeSH headings and keywords relevant to the natural history of CSM. Abstracts were reviewed and studies meeting the inclusion criteria were selected. The guidelines group assembled an evidentiary table summarizing the quality of evidence (Classes I-III). Disagreements regarding the level of evidence were resolved through an expert consensus conference. The group formulated recommendations that contained the degree of strength based on the Scottish Intercollegiate Guidelines network. Validation was done through peer review by the Joint Guidelines Committee of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons.
RESULTS: The natural history of CSM is mixed: it may manifest as a slow, stepwise decline or there may be a long period of quiescence (Class III). Long periods of severe stenosis are associated with demyelination and may result in necrosis of both gray and white matter. With severe and/or long lasting CSM symptoms, the likelihood of improvement with nonoperative measures is low. Objectively measurable deterioration is rarely seen acutely in patients younger than 75 years of age with mild CSM (modified Japanese Orthopaedic Association scale score > 12; Class I). In patients with cervical stenosis without myelopathy, the presence of abnormal electromyography findings or the presence of clinical radiculopathy is associated with the development of symptomatic CSM in this patient population (Class I).
CONCLUSIONS: The natural history of CSM is variable, which may affect treatment decisions.

PMID 19769489  J Neurosurg Spine. 2009 Aug;11(2):104-11. doi: 10.3171/・・・
著者: Kenzo Uchida, Hideaki Nakajima, Naoto Takeura, Takafumi Yayama, Alexander Rodriguez Guerrero, Ai Yoshida, Takumi Sakamoto, Kazuya Honjoh, Hisatoshi Baba
雑誌名: Spine J. 2014 Aug 1;14(8):1601-10. doi: 10.1016/j.spinee.2013.09.038. Epub 2013 Oct 18.
Abstract/Text BACKGROUND CONTEXT: Signal intensity on preoperative cervical magnetic resonance imaging (MRI) of the spinal cord has been shown to be a potential predictor of outcome of surgery for cervical compressive myelopathy. However, the prognostic value of such signal remains controversial. One reason for the controversy is the lack of proper quantitative methods to assess MRI signal intensity.
PURPOSE: To quantify signal intensity and to correlate intramedullary signal changes on MRI T1- and T2-weighted images (WIs) with clinical outcome and prognosis.
STUDY DESIGN: Retrospective case study.
PATIENT SAMPLE: Patients (n=148; cervical spondylotic myelopathy, n=102 and ossified posterior longitudinal ligament, n=46) who underwent surgery for cervical compressive myelopathy and had high signal intensity change on sagittal T2-WI MRI before surgery between 2006 and 2010.
OUTCOME MEASURE: Neurologic assessment was conducted with the Japanese Orthopedic Association (JOA) scoring system for cervical myelopathy. The rate of neurologic improvement was calculated with the use of preoperative and postoperative JOA scores.
METHODS: Quantitative analysis of MRI signal on both T1- and T2-WIs via use of the signal intensity ratio (SIR; signal intensity of lesion relative to that at C7-T1 disc level) was performed. Correlations between SIR on T1- and T2-WIs and preoperative JOA score, JOA improvement rate, disease duration, and MRI morphologic classification (cystic or diffuse type) were analyzed. Multivariate regression analysis for JOA improvement rate was also analyzed. In a substudy, 25 patients underwent follow-up MRI starting from 6 months after surgery to analyze the relationship between changes in SIR on follow-up MRI and clinical outcome.
RESULTS: SIR on T1-WIs, but not SIR on T2-WIs, correlated with postoperative neurologic improvement. The disease duration correlated negatively with SIR on T1-WIs and JOA improvement rate but not with SIR on T2-WIs. SIR on T2-WIs of "cystic type" was significantly greater than of "diffuse type," but SIR on T1-WI and JOA improvement rate were not different in the two types. Stepwise multivariate regression analysis indicated that SIR on T1-WIs and long disease duration were significant predictors of postoperative neurologic outcome. SIR on follow-up T1-WI and changes in SIR on T1-WI after surgery correlated positively with postoperative improvement rate. SIR on follow-up T2-WI and changes on T2-WI correlated negatively with postoperative neurologic improvement.
CONCLUSIONS: Our results suggest that low intensity signal on preoperative T1-WIs but not T2-WIs correlated with poor postoperative neurologic outcome. Furthermore, decreased signal intensity on postoperative T1-WIs and increased signal intensity on postoperative T2-WIs are predictors of poor neurologic outcome.

Copyright © 2014 Elsevier Inc. All rights reserved.
PMID 24411833  Spine J. 2014 Aug 1;14(8):1601-10. doi: 10.1016/j.spine・・・
著者: Peter S Amenta, George M Ghobrial, Kelly Krespan, Phi Nguyen, Muhammed Ali, James S Harrop
雑誌名: Clin Neurol Neurosurg. 2014 May;120:68-72. doi: 10.1016/j.clineuro.2014.02.019. Epub 2014 Mar 2.
Abstract/Text BACKGROUND: Cervical spondylotic myelopathy (CSM) is typically encountered in the elderly population. Significant inconsistencies currently exist regarding the definition of the disorder, the true incidence of CSM in younger populations, and the established diagnostic criteria.
OBJECTIVE: To highlight the lack of standardization in the definition and diagnosis of CSM.
METHODS: A PubMed literature search was conducted spanning the years 2001-2011. The search was limited by the following terms: (1) English language, (2) adults (19-44 years old), and (3) "cervical spondylotic myelopathy." Each article was reviewed to determine if the presence of the definition of CSM existed in the article. The clinical characteristics used to make the diagnosis of CSM were recorded for each article. Cochran's Q statistic was used to determine whether some clinical characteristics were more frequently used than others.
RESULTS: Ninety-three papers were reviewed in detail and 16 case reports, reviews, and articles concerning less than 3 patients were excluded, resulting in 77 articles in the final analysis. The most common clinical definitions were gait disturbance (22/77 articles (28.6%)), upper limb paresthesias or sensory disturbance (21/77 (27.3%)), and clumsy hands (15/77 (19.5%)). Hyperreflexia, spasticity, and pathologically increased reflexes were identified as diagnostic criteria in a minority of patients.
CONCLUSION: The literature employs a wide range of neurologic signs and symptoms to make the diagnosis of CSM, with a majority of studies failing to rely on strict diagnostic criteria. The clinician should not discount CSM as an explanation for the aforementioned findings, as it is well-reported in the literature among the ages 18-44.

Copyright © 2014 Elsevier B.V. All rights reserved.
PMID 24731579  Clin Neurol Neurosurg. 2014 May;120:68-72. doi: 10.1016・・・
著者: I David Kaye, Bryan J Marascalchi, Angel E Macagno, Virginie A Lafage, John A Bendo, Peter G Passias
雑誌名: Eur Spine J. 2015 Dec;24(12):2910-7. doi: 10.1007/s00586-015-4010-2. Epub 2015 May 23.
Abstract/Text PURPOSE: The aim of this study is to report and quantify the associated factors for morbidity and mortality following surgical management of cervical spondylotic myelopathy (CSM).
METHODS: The Nationwide Inpatient Sample (NIS) database was use to retrospectively review all patients over 25 years of age with a diagnosis of CSM who underwent anterior and/or posterior cervical fusion or laminoplasty between 2001 and 2010. The main outcome measures were total procedure-related complications and mortality. Multivariate regression analysis was used to identify demographic, comorbidity, and surgical parameters associated with increased morbidity and mortality risk [reported as: OR (95% CI)].
RESULTS: A total of 54,348 patients underwent surgical intervention for CSM with an overall morbidity rate of 9.83% and mortality rate of 0.43%. Comorbidities found to be associated with an increased complication rate included: pulmonary circulation disorders [6.92 (5.91-8.12)], pathologic weight loss [3.42 (3.00-3.90)], and electrolyte imbalance [2.82 (2.65-3.01)]. Comorbidities found to be associated with an increased mortality rate included: congestive heart failure [4.59 (3.62-5.82)], pulmonary circulation disorders [11.29 (8.24-15.47)], and pathologic weight loss [5.43 (4.07-7.26)]. Alternatively, hypertension [0.56 (0.46-0.67)] and obesity [0.36 (0.22-0.61)] were found to confer a decreased risk of mortality. Increased morbidity and mortality rates were also identified for fusions of 4-8 levels [morbidity: 1.55 (1.48-1.62), mortality: 1.80 (1.48-2.18)] and for age >65 years [morbidity: 1.65 (1.57-1.72), mortality: 2.74 (2.25-3.34)]. An increased morbidity rate was found for posterior-only [1.55 (1.47-1.63)] and combined anterior and posterior fusions [3.20 (2.98-3.43)], and an increased mortality rate was identified for posterior-only fusions [1.87 (1.40-2.49)]. Although revision fusions were associated with an increased morbidity rate [1.81 (1.64-2.00)], they were associated with a decreased rate of mortality [0.24 (0.10-0.59)].
CONCLUSION: The NIS database was used to provide national estimates of morbidity and mortality following surgical management of CSM in the United States. Several comorbidities, as well as demographic and surgical parameters, were identified as associated factors.

PMID 26002352  Eur Spine J. 2015 Dec;24(12):2910-7. doi: 10.1007/s0058・・・
著者: Dean M Wingerchuk, Brenda Banwell, Jeffrey L Bennett, Philippe Cabre, William Carroll, Tanuja Chitnis, Jérôme de Seze, Kazuo Fujihara, Benjamin Greenberg, Anu Jacob, Sven Jarius, Marco Lana-Peixoto, Michael Levy, Jack H Simon, Silvia Tenembaum, Anthony L Traboulsee, Patrick Waters, Kay E Wellik, Brian G Weinshenker, International Panel for NMO Diagnosis
雑誌名: Neurology. 2015 Jul 14;85(2):177-89. doi: 10.1212/WNL.0000000000001729. Epub 2015 Jun 19.
Abstract/Text Neuromyelitis optica (NMO) is an inflammatory CNS syndrome distinct from multiple sclerosis (MS) that is associated with serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Prior NMO diagnostic criteria required optic nerve and spinal cord involvement but more restricted or more extensive CNS involvement may occur. The International Panel for NMO Diagnosis (IPND) was convened to develop revised diagnostic criteria using systematic literature reviews and electronic surveys to facilitate consensus. The new nomenclature defines the unifying term NMO spectrum disorders (NMOSD), which is stratified further by serologic testing (NMOSD with or without AQP4-IgG). The core clinical characteristics required for patients with NMOSD with AQP4-IgG include clinical syndromes or MRI findings related to optic nerve, spinal cord, area postrema, other brainstem, diencephalic, or cerebral presentations. More stringent clinical criteria, with additional neuroimaging findings, are required for diagnosis of NMOSD without AQP4-IgG or when serologic testing is unavailable. The IPND also proposed validation strategies and achieved consensus on pediatric NMOSD diagnosis and the concepts of monophasic NMOSD and opticospinal MS.

© 2015 American Academy of Neurology.
PMID 26092914  Neurology. 2015 Jul 14;85(2):177-89. doi: 10.1212/WNL.0・・・
著者: T F Scott, E M Frohman, J De Seze, G S Gronseth, B G Weinshenker, Therapeutics and Technology Assessment Subcommittee of American Academy of Neurology
雑誌名: Neurology. 2011 Dec 13;77(24):2128-34. doi: 10.1212/WNL.0b013e31823dc535. Epub 2011 Dec 7.
Abstract/Text OBJECTIVE: To assess the evidence for diagnostic tests and therapies for transverse myelitis (TM) and make evidence-based recommendations.
METHODS: A review of the published literature from 1966 to March 2009 was performed, with evidence-based classification of relevant articles. Recommendations: Level B recommendations: neuromyelitis optica (NMO)-immunoglobulin G (IgG) antibodies should be considered useful to determine TM cause in patients presenting with clinical acute complete transverse myelitis (ACTM) features. The presence of NMO-IgG antibodies (aquaporin-4-specific antibodies) should be considered useful in determining increased TM recurrence risk. Level C recommendations: in suspected TM, distinction between ACTM or acute partial transverse myelitis may be considered useful to determine TM etiology and risk for relapse (more common with APTM). Age and gender may be considered useful to determine etiology in patients presenting with TM syndrome, with spinal infarcts seen more often in older patients and more female than male patients having TM due to multiple sclerosis (MS). Brain MRI characteristics consistent with those of MS may be considered useful to predict conversion to MS after a first partial TM episode. Longer spinal lesions extending over >3 vertebral segments may be considered useful in determining NMO vs MS. CSF examination for cells and oligoclonal bands may be considered useful to determine the cause of the TM syndrome. Plasma exchange may be considered in patients with TM who fail to improve after corticosteroid treatment. Rituximab may be considered in patients with TM due to NMO to decrease the number of relapses. Level U recommendations: there is insufficient evidence to support or refute the efficacy of other TM therapies or the usefulness of ethnicity to determine the cause of a subacute myelopathy.

PMID 22156988  Neurology. 2011 Dec 13;77(24):2128-34. doi: 10.1212/WNL・・・
著者: Maurizio Domenicucci, Alessandro Ramieri, Maurizio Salvati, Christian Brogna, Antonino Raco
雑誌名: J Neurosurg Spine. 2007 Nov;7(5):571-4. doi: 10.3171/SPI-07/11/571.
Abstract/Text A spinal epidural hematoma is an extremely rare complication of cervical spine manipulation therapy (CSMT). The authors present the case of an adult woman, otherwise in good health, who developed Brown-Séquard syndrome after CSMT. Decompressive surgery performed within 8 hours after the onset of symptoms allowed for complete recovery of the patient's preoperative neurological deficit. The unique feature of this case was the magnetic resonance image showing increased signal intensity in the paraspinal musculature consistent with a contusion, which probably formed after SMT. The pertinent literature is also reviewed.

PMID 17977203  J Neurosurg Spine. 2007 Nov;7(5):571-4. doi: 10.3171/SP・・・
著者: C V Gopalkrishnan, Amit Dhakoji, Suresh Nair
雑誌名: J Spinal Cord Med. 2012 Mar;35(2):113-7. doi: 10.1179/2045772312Y.0000000001. Epub 2012 Feb 4.
Abstract/Text CONTEXT: Spontaneous spinal epidural hematoma (SSEH) is a rare idiopathic condition that leads to acute onset of neurological deficits, which if not recognized early can have catastrophic consequences. The definition and pathophysiology of this condition remain controversial. High index of suspicion followed by T2-weighted gradient echo sequences are particularly useful in early diagnosis. Management consists of prompt surgical decompression of the hematoma though a recent trend is toward non-surgical treatment.
FINDINGS: A 70-year-old man presented with acute onset neck pain with a radicular component and rapidly progressive quadriparesis. Magnetic resonance imaging revealed a posteriorly located cervical extradural hematoma with cord compression that was promptly evacuated. Functional recovery to near normal function occurred within 24 hours of surgery.
CONCLUSION: SSEH in its true idiopathic form is a rare pathologic entity. Because of the high risk of poor outcome without treatment, SSEH should be a diagnostic possibility when presentation is even slightly suggestive. Prompt surgical evacuation of the hematoma leads to a favorable neurological outcome, whereas delay in treatment can be disastrous. The role of conservative management needs to be proven and should be tailored on an individual basis.

PMID 22333537  J Spinal Cord Med. 2012 Mar;35(2):113-7. doi: 10.1179/2・・・
著者: Tetsu Akimoto, Takeshi Yamada, Soji Shinoda, Yasushi Asano, Daisuke Nagata
雑誌名: J Cent Nerv Syst Dis. 2014;6:15-20. doi: 10.4137/JCNSD.S13252. Epub 2014 Feb 6.
Abstract/Text Hemiparesis develops in response to a wide range of neurological disorders, such as stroke, neoplasms and several inflammatory processes. Occasionally, it may also occur due to a lesion located in the high cervical spinal cord. In this concise review, we describe the features of spontaneous spinal epidural hematoma, which should be included in the large list of stroke mimics. Various concerns regarding the diagnostic and therapeutic conundrums relating to the condition are also discussed.

PMID 24526842  J Cent Nerv Syst Dis. 2014;6:15-20. doi: 10.4137/JCNSD.・・・
著者: Maciej Juryńczyk, Matthew Craner, Jacqueline Palace
雑誌名: J Neurol Neurosurg Psychiatry. 2014 Sep 23;. doi: 10.1136/jnnp-2014-308984. Epub 2014 Sep 23.
Abstract/Text Neuromyelitis optica (NMO) has long been considered as a variant of multiple sclerosis (MS) rather than a distinct disease. This concept changed with the discovery of serum antibodies (Ab) against aquaporin-4 (AQP4), which unequivocally differentiate NMO from MS. Patients who test positive for AQP4-Abs and present with optic neuritis (ON) and transverse myelitis (TM) are diagnosed with NMO and those who show an incomplete phenotype with isolated ON or longitudinally extensive TM (LETM) or less commonly brain/brainstem disease are referred to as NMO spectrum disorders (NMOSD). However, many patients, who have overlapping features of both NMO and MS, test negative for AQP4-Abs and may be difficult to definitively diagnose. This raises important practical issues, since NMO and MS respond differently to immunomodulatory treatment and have different prognoses. Here we review distinct features of AQP4-positive NMO and MS, which might then be useful in the diagnosis of antibody-negative overlap syndromes. We identify discriminators, which are related to demographic data (non-white origin, very late onset), clinical features (limited recovery from ON, bilateral ON, intractable nausea, progressive course of disability), laboratory results (cerebrospinal fluid (CSF) pleocytosis with eosinophils and/or neutrophils, oligoclonal bands, glial fibrillary acidic protein in the CSF) and imaging (LETM, LETM with T1 hypointensity, periependymal brainstem lesions, perivenous white matter lesions, Dawson's fingers, curved or S-shaped U-fibre juxtacortical lesions). We review the value of these discriminators and discuss the compelling need for new diagnostic markers in these two autoimmune demyelinating diseases of the central nervous system.

Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
PMID 25248365  J Neurol Neurosurg Psychiatry. 2014 Sep 23;. doi: 10.11・・・
著者: Ingo Kleiter, Anna Gahlen, Nadja Borisow, Katrin Fischer, Klaus-Dieter Wernecke, Brigitte Wegner, Kerstin Hellwig, Florence Pache, Klemens Ruprecht, Joachim Havla, Markus Krumbholz, Tania Kümpfel, Orhan Aktas, Hans-Peter Hartung, Marius Ringelstein, Christian Geis, Christoph Kleinschnitz, Achim Berthele, Bernhard Hemmer, Klemens Angstwurm, Jan-Patrick Stellmann, Simon Schuster, Martin Stangel, Florian Lauda, Hayrettin Tumani, Christoph Mayer, Lena Zeltner, Ulf Ziemann, Ralf Linker, Matthias Schwab, Martin Marziniak, Florian Then Bergh, Ulrich Hofstadt-van Oy, Oliver Neuhaus, Alexander Winkelmann, Wael Marouf, Jürgen Faiss, Brigitte Wildemann, Friedemann Paul, Sven Jarius, Corinna Trebst, Neuromyelitis Optica Study Group
雑誌名: Ann Neurol. 2016 Feb;79(2):206-16. doi: 10.1002/ana.24554. Epub 2015 Nov 26.
Abstract/Text OBJECTIVE: Neuromyelitis optica (NMO) attacks often are severe, are difficult to treat, and leave residual deficits. Here, we analyzed the frequency, sequence, and efficacy of therapies used for NMO attacks.
METHODS: A retrospective review was made of patient records to assess demographic/diagnostic data, attack characteristics, therapies, and the short-term remission status (complete remission [CR], partial remission [PR], no remission [NR]). Inclusion criteria were NMO according to Wingerchuk's 2006 criteria or aquaporin-4 antibody-positive NMO spectrum disorder (NMOSD). Remission status was analyzed with generalized estimating equations (GEEs), a patient-based statistical approach.
RESULTS: A total of 871 attacks in 185 patients (142 NMO/43 NMOSD, 82% female) were analyzed. The 1,153 treatment courses comprised high-dose intravenous steroids (HD-S; n = 810), plasma exchange (PE; n = 192), immunoadsorption (IA; n = 38), other (n = 80), and unknown (n = 33) therapies. The first treatment course led to CR in 19.1%, PR in 64.5%, and NR in 16.4% of attacks. Second, third, fourth, and fifth treatment courses were given in 28.2%, 7.1%, 1.4%, and 0.5% of attacks, respectively. This escalation of attack therapy significantly improved outcome (p < 0.001, Bowker test). Remission rates were higher for isolated optic neuritis versus isolated myelitis (p < 0.001), and for unilateral versus bilateral optic neuritis (p = 0.020). Isolated myelitis responded better to PE/IA than to HD-S as first treatment course (p = 0.037). Predictors of CR in multivariate GEE analysis were age (odds ratio [OR] = 0.97, p = 0.011), presence of myelitis (OR = 0.38, p = 0.002), CR from previous attack (OR = 6.85, p < 0.001), and first-line PE/IA versus HD-S (OR = 4.38, p = 0.006).
INTERPRETATION: Particularly myelitis and bilateral optic neuritis have poor remission rates. Escalation of attack therapy improves outcome. PE/IA may increase recovery in isolated myelitis.

© 2015 American Neurological Association.
PMID 26537743  Ann Neurol. 2016 Feb;79(2):206-16. doi: 10.1002/ana.245・・・
著者: Sung-Min Kim, Mark R Woodhall, Ji-Sun Kim, Seong-Joon Kim, Kyung Seok Park, Angela Vincent, Kwang-Woo Lee, Patrick Waters
雑誌名: Neurol Neuroimmunol Neuroinflamm. 2015 Dec;2(6):e163. doi: 10.1212/NXI.0000000000000163. Epub 2015 Oct 15.
Abstract/Text OBJECTIVE: To evaluate the clinical relevance of myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in a cohort of adults with inflammatory demyelinating disease (IDD) of the CNS.
METHODS: Live cell-based assays for MOG-Ab (IgG1 subset) and antibody to aquaporin-4 (AQP4-Ab) were performed in a cohort of 270 adult patients with IDD and 72 controls. Patients were first grouped by positive antibody result as MOG-Ab or AQP4-Ab, and the remainder were grouped by published diagnostic criteria.
RESULTS: Seventeen patients with IDD (6.3%) had MOG-Abs and 49 patients (18.1%) had AQP4-Abs; none had both antibodies. The MOG-Ab patients predominantly manifested with isolated symptoms of optic neuritis (83%). One-third of these patients experienced relapses, which involved only the optic nerve, and all relapsed within 1 year of disease onset. At onset, MRI in the MOG-Ab group uniquely demonstrated perineural enhancement, extending to the soft tissues around the optic nerves (33%). Although about 30% of MOG-Ab patients had brain MRI lesions, they had fewer periventricular lesions than the 26 patients with relapsing-remitting multiple sclerosis (MS); none of these lesions were ovoid or perpendicular to the ventricle. Moreover, MOG-Ab patients did not meet the diagnostic criteria for definite neuromyelitis optica (NMO) and had less spinal cord involvement than the AQP4-Ab group. Four patients (23.5%) had poor visual outcomes (<0.2) or paraplegia.
CONCLUSIONS: MOG-Abs may be a disease-specific biomarker in adult patients with IDD who have a disease distinct from NMO or MS. The radiologic as well as clinical manifestations of MOG-Ab patients can be useful in their differential diagnosis.

PMID 26516628  Neurol Neuroimmunol Neuroinflamm. 2015 Dec;2(6):e163. d・・・
著者: Wannapha Petchkrua, Stephen P Burns, Steven A Stiens, Jennifer J James, James W Little
雑誌名: Arch Phys Med Rehabil. 2003 Nov;84(11):1675-9.
Abstract/Text OBJECTIVE: To assess the prevalence of vitamin B(12) deficiency in persons with spinal cord injury (SCI) or disease (SCD).
DESIGN: Cross-sectional study with prospective blood collection and retrospective medical record review.
SETTING: Regional Veterans Affairs SCI service.
PARTICIPANTS: One hundred six adult men with chronic SCI or SCD and without other acute medical or surgical complications; most had SCI or SCD due to trauma or cervical spinal stenosis.
INTERVENTIONS: Not applicable.
MAIN OUTCOME MEASURES: Fasting blood samples were obtained at annual evaluation for serum B(12), folic acid, methylmalonic acid (MMA), and homocysteine. Serum levels were analyzed graphically and associated with patient variables by using statistical tests.
RESULTS: The vitamin B(12) level was subnormal in 5.7% of subjects; of those, all had supranormal MMA levels, all were age 40 to 59, most had complete SCI, and 67% had symptoms suggestive of B(12) deficiency. Low-normal B(12) levels were associated with a high prevalence of supranormal MMA. Subjects with either subnormal B(12) or low normal B(12) with supranormal MMA, both suggestive of vitamin B(12) deficiency, comprised 13.3% of the total group of subjects.
CONCLUSION: Vitamin B(12) deficiency is most common in middle-aged SCI or SCD persons with complete or near-complete spinal cord involvement (ie, American Spinal Injury Association class A-C injuries). Treatment with either parenteral or oral B(12) replacement may optimize health and prevent irreversible neuropsychiatric complications in those with subnormal and low-normal B(12) levels.

PMID 14639569  Arch Phys Med Rehabil. 2003 Nov;84(11):1675-9.
著者: Raquel Aparicio-Ugarriza, Gonzalo Palacios, Monika Alder, Marcela González-Gross
雑誌名: Clin Chem Lab Med. 2015 Jul;53(8):1149-59. doi: 10.1515/cclm-2014-0784.
Abstract/Text Vitamin B12 deficit is one of the most common vitamin deficiencies. However, there is no consensus on the cut-off points for vitamin B12 and its co-markers, such as folate, holotranscobalamin, methylmalonic acid and homocysteine. In order to establish the state of the art about cut-off points used to determine vitamin B12 deficiency in the last decades, the database MEDLINE was used for searching studies published in adults between December 1992 and May 2014 (69 articles), using search terms like 'vitamin B12', 'cobalamin', 'cut-off', 'deficiency' alone or in combinations. Broad ranges of cut-off points for vitamin B12 and its biomarkers were identified: vitamin B12 ranged between 100 pmol/L and 350 pmol/L, holotranscobalamin 20-50 pmol/L, methylmalonic acid 0.210-0.470 μmol/L, homocysteine 10-21.6 μmol/L, serum folate 3.7-15.9 nmol/L and red blood cell 124-397 nmol/L. For the majority of studies, the potential influence of age, analytical methods, gender and fortified food consumption was not taken in account when choosing cut-off values. This could explain the discrepancies between studies on vitamin B12 and folate deficiency prevalences. We conclude that there is inconsistency in the literature regarding vitamin B12 cut-offs. It would be necessary to establish different reference cut-offs according to age, considering the analytical methods used.

PMID 25470607  Clin Chem Lab Med. 2015 Jul;53(8):1149-59. doi: 10.1515・・・
著者: Wannapha Petchkrua, James W Little, Stephen P Burns, Steven A Stiens, Jennifer J James
雑誌名: J Spinal Cord Med. 2003 Summer;26(2):116-21.
Abstract/Text BACKGROUND/OBJECTIVE: Vitamin B12 (or cobalamin) deficiency is well known in geriatric patients, but not in those with spinal cord injury (SCI). This retrospective study describes vitamin B12 deficiency in SCI.
METHODS: This study utilized a retrospective chart review of patients with SCI who had received serum vitamin B12 testing over the last 10 years.
RESULTS: Probable vitamin B12 deficiency was noted in 16 patients with SCI. Twelve patients had subnormal serum vitamin B12 levels (< 220 pg/mL), whereas 4 patients had low-normal vitamin B12 levels (< 300 pg/mL) with neurologic and/or psychiatric symptoms that improved following vitamin B12 replacement. Classic findings of paresthesias and numbness often were not evident; such findings likely were masked by the pre-existing sensory impairment caused by SCI. Of the 16 SCI patients, 7 were ambulatory; 4 of the 7 presented with deterioration of gait. In addition, 3 of the 16 SCI patients presented with depression and fatigue, 2 had worsening pain, 2 had worsening upper limb weakness, and 2 had memory decline. Of the 12 patients with subnormal serum vitamin B12 levels, 6 were asymptomatic. Classic laboratory findings of low serum vitamin B12, macrocytic red blood cell indices, and megaloblastic anemia were not always present. Anemia was identified in 7 of the 16 patients and macrocytic red blood cells were found in 3 of the 16 patients. Only 1 of the 16 SCI patients had a clear pathophysiologic mechanism to explain the vitamin B12 deficiency (ie, partial gastrectomy); none of the patients were vegetarian. Twelve of the SCI patients appeared to experience clinical benefits from cyanocobalamin replacement (some patients experienced more than 1 benefit), including reversal of anemia (5 patients), improved gait (4 patients), improved mood (3 patients), improved memory (2 patients), reduced pain (2 patients), strength gain (1 patient), and reduced numbness (1 patient).
CONCLUSION: It is recommended that physicians consider vitamin B12 deficiency in their patients with SCI, particularly in those with neurologic and/or psychiatric symptoms. These symptoms often are reversible if treatment is initiated early.

PMID 12828286  J Spinal Cord Med. 2003 Summer;26(2):116-21.
著者: S B Jung, V Nagaraja, A Kapur, G D Eslick
雑誌名: Intern Med J. 2015 Apr;45(4):409-16. doi: 10.1111/imj.12697.
Abstract/Text BACKGROUND: Vitamin B12 (cobalamin) deficiency can result in irreversible structural brain changes if not treated appropriately. Long-term use of acid-lowering agents (ALA) has been linked to vitamin B12 deficiency, but results are inconsistent.
AIM: To evaluate the association between prolonged ALA use and vitamin B12 deficiency by performing a meta-analysis.
METHODS: A systematic search was conducted using MEDLINE, PubMed, EMBASE, Current Contents, Cochrane Library, Google Scholar, Science Direct and Web of Science. Original data were abstracted from each study and used to calculate a pooled odds ratio and 95% confidence interval (95% CI).
RESULTS: Of the articles reviewed, four case-control studies (4254 cases and 19,228 controls) and one observational study met full criteria for analysis. The long-term ALA use was significantly associated with development of vitamin B12 deficiency (hazard ratio 1.83, 95% CI: 1.36-2.46, P-value 0.00).
CONCLUSION: Chronic use of ALA is a risk factor for developing vitamin B12 deficiency. Judicious prescribing of ALA and regular monitoring of vitamin B12 in patients who are inevitably on long-term ALA therapy are recommended.

© 2015 Royal Australasian College of Physicians.
PMID 25583062  Intern Med J. 2015 Apr;45(4):409-16. doi: 10.1111/imj.1・・・
著者: Stephan R Jaiser, Gavin P Winston
雑誌名: J Neurol. 2010 Jun;257(6):869-81. doi: 10.1007/s00415-010-5511-x. Epub 2010 Mar 16.
Abstract/Text Acquired copper deficiency has been recognised as a rare cause of anaemia and neutropenia for over half a century. Copper deficiency myelopathy (CDM) was only described within the last decade, and represents a treatable cause of non-compressive myelopathy which closely mimics subacute combined degeneration due to vitamin B12 deficiency. Here, 55 case reports from the literature are reviewed regarding their demographics, aetiology, haematological and biochemical parameters, spinal imaging, treatment and outcome. The pathophysiology of disorders of copper metabolism is discussed. CDM most frequently presented in the fifth and sixth decades and was more common in women (F:M = 3.6:1). Risk factors included previous upper gastrointestinal surgery, zinc overload and malabsorption syndromes, all of which impair copper absorption in the upper gastrointestinal tract. No aetiology was established in 20% of cases. High zinc levels were detected in some cases not considered to have primary zinc overload, and in this situation the contribution of zinc to the copper deficiency state remained unclear. Cytopenias were found in 78%, particularly anaemia, and a myelodysplastic syndrome may have been falsely diagnosed in the past. Spinal MRI was abnormal in 47% and usually showed high T2 signal in the posterior cervical and thoracic cord. In a clinically compatible case, CDM may be suggested by the presence of one or more risk factors and/or cytopenias. Low serum copper and caeruloplasmin levels confirmed the diagnosis and, in contrast to Wilson's disease, urinary copper levels were typically low. Treatment comprised copper supplementation and modification of any risk factors, and led to haematological normalisation and neurological improvement or stabilisation. Since any neurological recovery was partial and case numbers of CDM will continue to rise with the growing use of bariatric gastrointestinal surgery, clinical vigilance will remain the key to minimising neurological sequelae. Recommendations for treatment and prevention are made.

PMID 20232210  J Neurol. 2010 Jun;257(6):869-81. doi: 10.1007/s00415-0・・・
著者: Renata Hebel, Mirosława Dubaniewicz-Wybieralska, Anna Dubaniewicz
雑誌名: J Neurol. 2015 Feb;262(2):258-67. doi: 10.1007/s00415-014-7482-9. Epub 2014 Sep 7.
Abstract/Text Sarcoidosis (SA) is a granulomatous, multisystem disease of unknown etiology. Most often the disease affects lungs and mediastinal lymph nodes, but it may occur in other organs. Neurosarcoidosis (NS) more commonly occurs with other sarcoidosis forms, in 1 % of cases it involves only nervous system. Symptomatic NS occurs but on autopsy study up to 25 % of cases are confirmed. NS can affect central nervous system: the brain, spinal cord and peripheral nerves, and muscles. The diagnosis of neurosarcoidosis facilitates diagnostic criteria: histopathological, imaging and cerebrospinal fluid examination, and clinical symptoms. At present, there are no set standards for treatment of patients suffering from NS. Early therapy of symptomatic patients is recommended. Corticosteroids still are the first line of treatment for NS patients. In cases of steroids resistance, lack of their effectiveness or existence of contraindication to their use, immunosuppressant treatment is recommended. The latest NS algorithm with immunosuppressive treatment is discussed.

PMID 25194844  J Neurol. 2015 Feb;262(2):258-67. doi: 10.1007/s00415-0・・・
著者: P Sendi, T Bregenzer, W Zimmerli
雑誌名: QJM. 2008 Jan;101(1):1-12. doi: 10.1093/qjmed/hcm100. Epub 2007 Nov 3.
Abstract/Text Spinal epidural abscess (SEA) is a rare but severe infection requiring prompt recognition. The major prognostic factor for a favourable outcome is early diagnosis, leading to appropriate treatment. In clinical practice, a diagnosis of SEA is often not considered, particularly in the early stages of the disease when neurological symptoms are not apparent. Knowledge of persons at risk, clinical features and the required diagnostic procedures may decrease the number of initially misdiagnosed cases. Clinical signs, duration of symptoms and the rate of neurological deterioration show a high inter-individual variability, and the classic triad (spinal pain, fever and neurological deficit) is often not found, especially not at first presentation to a physician. However, most patients complain of severe localized back pain. Inflammatory parameters in the blood are generally elevated, but not specific. Gadolinium-enhanced magnetic resonance imaging is the most sensitive, specific and accurate imaging method. Although neurosurgical decompression is still the treatment of choice in the majority of cases, less invasive procedures (e.g. computed tomography-guided needle aspiration) or antimicrobial treatment alone can be applied in selected cases. The choice of the most appropriate therapy should be discussed immediately after a confirmed diagnosis in consultation with infectious disease, radiology and spinal surgery specialists. The outcome of SEA is largely influenced by the severity and duration of neurological deficits prior to surgery, stressing the importance of early recognition.

PMID 17982180  QJM. 2008 Jan;101(1):1-12. doi: 10.1093/qjmed/hcm100. E・・・
著者: Abelardo Q C Araújo, Ana Claudia C Leite, Marco Antonio S D Lima, Marcus Tulius T Silva
雑誌名: Arq Neuropsiquiatr. 2009 Mar;67(1):132-8.
Abstract/Text HTLV-1 is a retrovirus associated with a myriad of clinical conditions, especially hematological and neurological ones. Regarding nervous system diseases, it is of utmost importance to select those cases in which HTLV-1 infection could really be associated. This is particularly true for patients from endemic areas and for HIV-infected patients and drug users, since that these groups are at a higher risk for HTLV infection. This caution in selecting neurological patients for HTLV diagnostic tests is justified by the fact that in some circumstances the seropositivity may merely represent an epiphenomenon. In this paper we enroll some neurological conditions that have been associated with HTLV-1/2 infection in the literature and discuss the real need for HTLV-1/2 diagnostic tests in each one. Because HIV/HTLV-co-infected patients seem to be at an increased risk for neurological diseases development, a special consideration about this matter is also made.

PMID 19330234  Arq Neuropsiquiatr. 2009 Mar;67(1):132-8.
著者: Hirohisa Nose, Mineki Saito, Koichiro Usuku, Amir H Sabouri, Toshio Matsuzaki, Ryuji Kubota, Nobutaka Eiraku, Yoshitaka Furukawa, Shuji Izumo, Kimiyoshi Arimura, Mitsuhiro Osame
雑誌名: J Neurovirol. 2006 Jun;12(3):171-7. doi: 10.1080/13550280600827336.
Abstract/Text The authors have previously developed a logistic regression equation to predict the odds that a human T-cell lymphotropic virus type 1 (HTLV-1)-infected individual of specified genotype, age, and provirus load has HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in southern Japan. This study evaluated whether this equation is useful predictor for monitoring asymptomatic HTLV-1-seropositive carriers (HCs) in the same population. The authors genotyped 181 HCs for each HAM/TSP-associated gene (tumor necrosis factor [TNF]-alpha-863A/C, stromal cell-derived factor 1 (SDF-1) +801G/A, human leukocyte antigen [HLA]-A*02, HLA-Cw*08, HTLV-1 tax subgroup) and measured HTLV-1 provirus load in peripheral blood mononuclear cells using real-time polymerase chain reaction (PCR). Finally, the odds of HAM/TSP for each subject were calculated by using the equation and compared the results with clinical symptoms and laboratory findings. Although no clear difference was seen between the odds of HAM/TSP and either sex, family history of HAM/TSP or adult T-cell lenkemia (ATL), history of blood transfusion, it was found that brisk patellar deep tendon reflexes, which suggest latent central nervous system compromise, and flower cell-like abnormal lymphocytes, which is the morphological characteristic of ATL cells, were associated with a higher odds of HAM/TSP. The best-fit logistic regression equation may be useful for detecting subclinical abnormalities in HCs in southern Japan.

PMID 16877298  J Neurovirol. 2006 Jun;12(3):171-7. doi: 10.1080/135502・・・
著者: Osvaldo J M Nascimento, Wilson Marques
雑誌名: Handb Clin Neurol. 2013;115:531-41. doi: 10.1016/B978-0-444-52902-2.00030-8.
Abstract/Text Symptomatic peripheral neuropathy occurs in a small proportion of HTLV-1 infected patients. Peripheral manifestations are often masked by symptoms and signs of the tropical spastic myelopathy characteristic of the disease. Peripheral neuropathy is often characterized by alteration of small-fiber functions, with inflammatory lesions of peripheral nerves, sometimes associated with symptomatic polymyositis, which may occur in isolation in this setting.

Copyright © 2013 Elsevier B.V. All rights reserved.
PMID 23931800  Handb Clin Neurol. 2013;115:531-41. doi: 10.1016/B978-0・・・
著者: B M van Geel, L Bezman, D J Loes, H W Moser, G V Raymond
雑誌名: Ann Neurol. 2001 Feb;49(2):186-94.
Abstract/Text Our objective was to study the phenotype evolution of X-linked adrenoleukodystrophy (X-ALD) and the relation between axonal degeneration and cerebral demyelination. Although different X-ALD phenotypes are recognized, little is known about their evolution. Neuropathological and electrophysiological studies have shown that X-ALD is a disease with mixed features of axonal degeneration, leading to myeloneuropathy, and a severe inflammatory reaction in the cerebral white matter, resulting in demyelination. Retrospectively, 129 men with X-ALD were studied who were 1) at least 20 years presently or at the time of death, and 2) regularly monitored. Phenotype assignments were made at diagnosis and at present, or at death, using medical history and findings of neurological examination. Handicap was studied with the modified Rankin scale, and cerebral abnormalities with the X-ALD MRI severity (Loes) score. The mean follow-up interval was 10.1 +/- 5.0 years. Among 32 patients neurologically asymptomatic at diagnosis, 16 (50%) developed neurological deficits. Among 68 adrenomyeloneuropathy (AMN) patients initially without clinical brain involvement, 13 (19%) additionally developed cerebral demyelination. In a subset of 60 AMN patients, a moderate handicap evolved over a period of 16.2 +/- 8.9 years. Among 13 AMN patients with additional definite or probable cerebral involvement at diagnosis, eight died and one remained in a vegetative state. Most of the 16 patients with the cerebral phenotypes deteriorated. There is a high risk for adult neurologically asymptomatic patients to develop neurological deficits and for AMN patients to develop cerebral demyelination. Axonal degeneration and cerebral demyelination emerge in X-ALD independently of each other. This may have implications for the phenotype classification, the search for modifying factors, and the development and evaluation of new therapies.

PMID 11220738  Ann Neurol. 2001 Feb;49(2):186-94.
著者: Jonathan Lieske, Brian Cameron, Benjamin Drinkwine, Sean Goretzke, Lily Alemi, Kenneth Needham, John Ventura, Van Thong Ho, Donald V La Barge
雑誌名: J Comput Assist Tomogr. 2011 Jul-Aug;35(4):492-4. doi: 10.1097/RCT.0b013e31821e277b.
Abstract/Text The authors present a case of "surfer's myelopathy," a rarely described syndrome characterized by nontraumatic paraparesis/paraplegia in novice surfers and theorized to result from spinal cord ischemia secondary to surfing-related positional hyperextension. Imaging and clinical course of the youngest known affected individual are discussed, including evidence of acute spinal cord infarction on diffusion-weighted magnetic resonance imaging, a finding not previously described in the literature.

PMID 21765307  J Comput Assist Tomogr. 2011 Jul-Aug;35(4):492-4. doi: ・・・
著者: Christopher Power, Lysa Boissé, Sean Rourke, M John Gill
雑誌名: Can J Neurol Sci. 2009 May;36(3):285-95.
Abstract/Text Over 60,000 Canadians are infected with human immunodeficiency virus (HIV). Greater than 50% of these individuals will develop a neurological disorder despite the availability of highly active antiretroviral therapy. HIV causes nervous system disease at all stages of infection with adverse effects on quality of life, adherence to medications, employment and survival. These disorders include opportunistic infections in addition to distinct HIV-associated neurological syndromes and undesirable treatment-related effects. The latter two groups of disorders are often undiagnosed and untreated in both adolescents and adults. Direct HIV infection of central nervous system causes HIV-associated dementia, which is a progressive subcortical dementia. HIV infection of the peripheral nervous system produces a painful sensory neuropathy termed distal sensory polyneuropathy, which may be exacerbated by several antiretroviral drugs. Other important HIV-induced neurological disorders include vacuolar myelopathy and an increased risk of seizures. Future issues that will confound the presentation and treatment of HIV-induced nervous system disorders include the increasing prevalence of drug-resistant HIV strains, increasing age of HIV-infected patients, hepatitis C virus co-infection and the Immune Reconstitution Inflammatory Syndrome. Herein, we review the clinical presentations, underlying pathogenesis and treatments of this burgeoning group of neurological disorders.

PMID 19534327  Can J Neurol Sci. 2009 May;36(3):285-95.
著者: Aria Nouri, Lindsay Tetreault, Anoushka Singh, Spyridon K Karadimas, Michael G Fehlings
雑誌名: Spine (Phila Pa 1976). 2015 Jun 15;40(12):E675-93. doi: 10.1097/BRS.0000000000000913.
Abstract/Text STUDY DESIGN: Review.
OBJECTIVE: To formally introduce "degenerative cervical myelopathy" (DCM) as the overarching term to describe the various degenerative conditions of the cervical spine that cause myelopathy. Herein, the epidemiology, pathogenesis, and genetics of conditions falling under this hypernym are carefully described.
SUMMARY OF BACKGROUND DATA: Nontraumatic, degenerative forms of cervical myelopathy represent the commonest cause of spinal cord impairment in adults and include cervical spondylotic myelopathy, ossification of the posterior longitudinal ligament, ossification of the ligamentum flavum, and degenerative disc disease. Unfortunately, there is neither a specific term nor a specific diagnostic International Classification of Diseases, Tenth Revision code to describe this collection of clinical entities. This has resulted in the inconsistent use of diagnostic terms when referring to patients with myelopathy due to degenerative disease of the cervical spine.
METHODS: Narrative review.
RESULTS: The incidence and prevalence of myelopathy due to degeneration of the spine are estimated at a minimum of 41 and 605 per million in North America, respectively. Incidence of cervical spondylotic myelopathy-related hospitalizations has been estimated at 4.04/100,000 person-years, and surgical rates seem to be rising. Pathophysiologically, myelopathy results from static compression, spinal malalignment leading to altered cord tension and vascular supply, and dynamic injury mechanisms. Occupational hazards, including transportation of goods by weight bearing on top of the head, and other risk factors may accelerate DCM development. Potential genetic factors include those related to MMP-2 and collagen IX for degenerative disc disease, and collagen VI and XI for ossification of the posterior longitudinal ligament. In addition, congenital anomalies including spinal stenosis, Down syndrome, and Klippel-Feil syndrome may predispose to the development of DCM.
CONCLUSION: Although DCMs can present as separate diagnostic entities, they are highly interrelated, frequently manifest concomitantly, present similarly from a clinical standpoint, and seem to be in part a response to compensate and improve stability due to progressive age and wear of the cervical spine. The use of the term "degenerative cervical myelopathy" is advocated.
LEVEL OF EVIDENCE: 5.

PMID 25839387  Spine (Phila Pa 1976). 2015 Jun 15;40(12):E675-93. doi:・・・
著者: Lindsay Tetreault, Ahmed Ibrahim, Pierre Côté, Anoushka Singh, Michael G Fehlings
雑誌名: J Neurosurg Spine. 2016 Jan;24(1):77-99. doi: 10.3171/2015.3.SPINE14971. Epub 2015 Sep 25.
Abstract/Text OBJECTIVE: Although generally safe and effective, surgery for the treatment of cervical spondylotic myelopathy (CSM) is associated with complications in 11%-38% of patients. Several predictors of postoperative complications have been proposed but few are used to detect high-risk patients. A standard approach to identifying "at-risk" patients would improve surgeons' ability to prevent and manage these complications. The authors aimed to compare the complication rates between various surgical procedures used to treat CSM and to identify patient-specific, clinical, imaging, and surgical predictors of complications.
METHODS: The authors conducted a systematic review of the literature and searched MEDLINE, MEDLINE in Process, EMBASE, and Cochrane Central Register of Controlled Trials from 1948 to September 2013. Cohort studies designed to evaluate predictors of complications and intervention studies conducted to compare different surgical approaches were included. Each article was critically appraised independently by 2 reviewers, and the evidence was synthesized according to the principles outlined by the Grading of Recommendation Assessment, Development and Evaluation (GRADE) Working Group.
RESULTS: A total of 5472 citations were retrieved. Of those, 60 studies met the inclusion criteria and were included in the review. These studies included 36 prognostic cohort studies and 28 comparative intervention studies. High evidence suggests that older patients are at a greater risk of perioperative complications. Based on low evidence, other clinical factors such as body mass index, smoking status, duration of symptoms, and baseline severity score, are not predictive of complications. With respect to surgical factors, low to moderate evidence suggests that estimated blood loss, surgical approach, and number of levels do not affect rates of complications. A longer operative duration (moderate evidence), however, is predictive of perioperative complications and a 2-stage surgery is related to an increased risk of major complications (high evidence). In terms of surgical techniques, higher rates of neck pain were found in patients undergoing laminoplasty compared with anterior spinal fusion (moderate evidence). In addition, with respect to laminoplasty techniques, there was a lower incidence of C-5 palsy in laminoplasty with concurrent foraminotomy compared with nonforaminotomy (low evidence).
CONCLUSIONS: The current review suggests that older patients are at a higher risk of perioperative complications. A longer operative duration and a 2-stage surgery both reflect increased case complexity and can indirectly predict perioperative complications.

PMID 26407090  J Neurosurg Spine. 2016 Jan;24(1):77-99. doi: 10.3171/2・・・

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