본문 바로가기
KERD
논문KCI 등재

한국의 근위축측삭경화증: 임상 특징 및 예후 예측 인자

Amyotrophic Lateral Sclerosis in Korea: Clinical Characteristics and Prognostic Factors

2

조회

0

다운로드

11

인용

0

참고문헌

키워드

주제분류신경과학

초록

Background: No prospective analyses of clinical characteristics and prognostic factors on amyotrophic lateral sclerosis (ALS) have been performed in Korea. Methods: The prognosis of 227 sporadic ALS patient with El-Escorial-compatible criteria were evaluated using the serial Korean Amyotrophic Lateral Sclerosis Functional Rating Scale-revised (K-ALSFRS-R), and the progression rate of K-ALSFRS-R (ΔFS=48-ALSFRS-R at the time of diagnosis). Their clinical characteristics and survival times were also analyzed. The endpoint was defined as either time to death or the tracheostomy state. The effects of individual prognostic factors on disease progression were assessed with the Kaplan-Meier life-table method and multivariate Cox proportional regression analysis. The declining ALSFRS-R curve, epidemiological data, and neuropsychological tests were evaluated. Results: Among 331 motor neuron disease (MND) patients, 227 were finally diagnosed as sporadic ALS. The mean age at onset in 227 sporadic ALS patients was 50.7 years (range 19-76 years). Analysis of sequential ALSFRS-R data revealed that a mean ΔFS of 0.73/month and a high ΔFS score were related with early death or a tracheostomy state. Neuropsychological testing revealed nonmotor symptoms, and especially cognitive impairment, had developed in half of our patients. Conclusions: Our data suggest that ΔFS is valuable parameter for evaluating prognosis and clinical course of ALS patients, and clinical characteristics obtained from this study would be informative data for understanding epidemiology of ALS in Korea.

Abstract

Background: No prospective analyses of clinical characteristics and prognostic factors on amyotrophic lateral sclerosis (ALS) have been performed in Korea. Methods: The prognosis of 227 sporadic ALS patient with El-Escorial-compatible criteria were evaluated using the serial Korean Amyotrophic Lateral Sclerosis Functional Rating Scale-revised (K-ALSFRS-R), and the progression rate of K-ALSFRS-R (ΔFS=48-ALSFRS-R at the time of diagnosis). Their clinical characteristics and survival times were also analyzed. The endpoint was defined as either time to death or the tracheostomy state. The effects of individual prognostic factors on disease progression were assessed with the Kaplan-Meier life-table method and multivariate Cox proportional regression analysis. The declining ALSFRS-R curve, epidemiological data, and neuropsychological tests were evaluated. Results: Among 331 motor neuron disease (MND) patients, 227 were finally diagnosed as sporadic ALS. The mean age at onset in 227 sporadic ALS patients was 50.7 years (range 19-76 years). Analysis of sequential ALSFRS-R data revealed that a mean ΔFS of 0.73/month and a high ΔFS score were related with early death or a tracheostomy state. Neuropsychological testing revealed nonmotor symptoms, and especially cognitive impairment, had developed in half of our patients. Conclusions: Our data suggest that ΔFS is valuable parameter for evaluating prognosis and clinical course of ALS patients, and clinical characteristics obtained from this study would be informative data for understanding epidemiology of ALS in Korea.

참고문헌 (48)

  1. Worms PM, "The epidemiology of motor neuron diseases: a review of recent studies", J Neurol Sci, 191, pp.3~9 (2001)
  2. Czaplinski A, "Slower disease progression and prolonged survival in contemporary patients with amyotrophic lateral sclerosis: is the natural history of amyotrophic lateral sclerosis changing", Arch Neurol, 63, pp.1139~1143 (2006)
  3. Rosen DR, "Mutations in Cu", Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis, 1993, pp.364, 362~
  4. Lomen-Hoerth C, "Are amyotrophic lateral sclerosis patients cognitively normal", Neurology, 60, pp.1094~1097 (2003)
  5. Barson FP, "A neuropsychological investigation of dementia in motor neurone disease (MND)", J Neurol Sci, 180, pp.107~113 (2000)
  6. Arai T, "TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis", Biochem Biophys Res Commun, 351, pp.602~611 (2006)
  7. Neumann M, "Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis", Science, 314, pp.130~133 (2006)
  8. Lagier-Tourenne C, "Rethinking ALS: the FUS about TDP-43", Cell, 136, pp.1001~1004 (2009)
  9. Brooks BR, "El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis", Amyotroph Lateral Scler Other Motor Neuron Disord, 1, pp.293~299 (2000)
  10. Kimura F, "Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS", Neurology, 66, pp.265~267 (2006)
  11. 김현영, "한글판 Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised : 신뢰도와 타당도에 관한 예비연구", 대한신경과학회지, 25(2), pp.149~154 (2007), 대한신경과학회
  12. McKhann G, "Clinical diagnosis of Alzheimer's disease: report of the NINCDSADRDA Work Group under the auspices of Department of Health and Human Services Task Force on Alzheimer’s Disease", Neurology, 34, pp.939~944 (1984)
  13. Neary D, "Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria", Neurology, 51, pp.1546~1554 (1998)
  14. Cummings JL, "The neuropsychiatric inventory: comprehensive assessment of psychopathology in dementia", Neurology, 44, pp.2308~2314 (1994)
  15. Gordon PH, "Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS", Neurology, 67, pp.1314~1315 (2006)
  16. Ravits J, "Focality of upper and lower motor neuron degeneration at the clinical onset of ALS", Neurology, 68, pp.1571~1575 (2007)
  17. Harwood CA, "Physical activity as an exogenous risk factor in motor neuron disease (MND): a review of the evidence", Amyotroph Lateral Scler, 10, pp.191~204 (2009)
  18. Oosthuyse B, "Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration", Nat Genet, 28, pp.131~138 (2001)
  19. Veldink JH, "Physical activity and the association with sporadic ALS", Neurology, 64, pp.241~245 (2005)
  20. Ellis CM, "Volumetric analysis reveals corticospinal tract degeneration and extramotor involvement in ALS", Neurology, 57, pp.1571~1578 (2001)
  21. Grosskreutz J, "Whole brain-based computerized neuroimaging in ALS and other motor neuron disorders", Amyotroph Lateral Scler, 9, pp.238~248 (2008)
  22. Ellis CM, "Diffusion tensor MRI assesses corticospinal tract damage in ALS", Neurology, 53, pp.1051~1058 (1999)
  23. Hong YH, "Diffusion tensor MRI as a diagnostic tool of upper motor neuron involvement in amyotrophic lateral sclerosis", J Neurol Sci, 227, pp.73~78 (2004)
  24. Mezzapesa DM, "Whole-brain and regional brain atrophy in amyotrophic lateral sclerosis", Am J Neuroradiol, 28, pp.255~259 (2007)
  25. Cedarbaum JM, "The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III)", J Neurol Sci, 169, pp.13~21 (1999)
  26. Appel V, "A rating scale for amyotrophic lateral sclerosis: description and preliminary experience", Ann Neurol, 22, pp.328~333 (1987)
  27. Kaufmann P, "The ALSFRSr predicts survival time in an ALS clinic population", Neurology, 64, pp.38~43 (2005)
  28. Kollewe K, "ALSFRS-R score and its ratio: a useful predictor for ALS-progression", J Neurol Sci, 275, pp.69~73 (2008)
  29. Desport JC, "Nutritional status is a prognostic factor for survival in ALS patients", Neurology, 53, pp.1059~1063 (1999)
  30. Czaplinski A, "Predictability of disease progression in amyotrophic lateral sclerosis", Muscle Nerve, 34, pp.702~708 (2006)
  31. Voustianiouk A, "ALSFRS and appel ALS scores: discordance with disease progression", Muscle Nerve, 37, pp.668~672 (2008)
  32. Lechtzin N, "Accurate ALSFRS-R scores can be generated from retrospective review of clinic notes", Amyotroph Lateral Scler, 10, pp.244~247 (2009)
  33. Kaufmann P, "Excellent inter-rater, intra-rater, and telephone-administered reliability of the ALSFRS-R in a multicenter clinical trial", Amyotroph Lateral Scler, 8, pp.42~46 (2007)
  34. Czaplinski A, "Amyotrophic lateral sclerosis: early predictors of prolonged survival", J Neurol, 253, pp.1428~1436 (2006)
  35. Strong MJ, "A prospective study of cognitive impairment in ALS", Neurology, 53, pp.1665~1670 (1999)
  36. Neary D, "Cognitive change in motor neurone disease", amyotrophic lateral sclerosis (MND, J Neurol Sci, pp.2000, 180[DOI]
  37. Ringholz GM, "Prevalence and patterns of cognitive impairment in sporadic ALS", Neurology, 65, pp.586~590 (2005)
  38. Abrahams S, "Relation between cognitive dysfunction and pseudobulbar palsy in amyotrophic lateral sclerosis", J Neurol Neurosurg Psychiatry, 62, pp.464~472 (1997)
  39. Kabashi E, "TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis", Nat Genet, 40, pp.572~574 (2008)
  40. Sreedharan J, "TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis", Science, 319, pp.1668~1672 (2008)
  41. Gitcho MA, "TDP-43 A315T mutation in familial motor neuron disease", Ann Neurol, 63, pp.535~538 (2008)
  42. Massman PJ, "Prevalence and correlates of neuropsychological deficits in amyotrophic lateral sclerosis", J Neurol Neurosurg Psychiatry, 61, pp.450~455 (1996)
  43. Baker M, "Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17", Nature, 442, pp.916~919 (2006)
  44. Mackenzie IR, "The neuropathology and clinical phenotype of FTD with progranulin mutations", Acta Neuropathol, 114, pp.49~54 (2007)
  45. Pickering-Brown SM, "Progranulin and frontotemporal lobar degeneration", Acta Neuropathol, 114, pp.39~47 (2007)
  46. Snowden JS, "Progranulin gene mutations associated with frontotemporal dementia and progressive non-fluent aphasia", Brain, 129, pp.3091~3102 (2006)
  47. Strong MJ, "Cognition, language, and speech in amyotrophic lateral sclerosis: a review", J Clin Exp Neuropsychol, 18, pp.291~303 (1996)
  48. Thommessen B, "The psychosocial burden on spouses of the elderly with stroke, dementia and Parkinson’s disease", Int J Geriatr Psychiatry, 17, pp.78~84 (2002)

관련 논문AI추천

Current Status of the Diagnosis and Management of Amyotrophic Lateral Sclerosis in Korea: A Multi-Center Cross-Sectional Study김병조, 김승현, 배종석, 백원기, 손은희, 조중양, 홍윤호대한신경과학회 · Journal of Clinical Neurology2012 · 제8권 제4호Long-Term Outcome of Amyotrophic Lateral Sclerosis in Korean Subjects강성웅, 박지현, 서미리, 이장우, 최영철, 최원아, 홍정화대한재활의학회 · Annals of Rehabilitation Medicine2017 · 제41권 제6호한국 근위축성측삭경화증 등록체계의 구축과 향후 전망김병조, 김종국, 배종석, 백원기, 서범천, 성정준, 손은희, 안석원, 조중양, 홍윤호대한임상신경생리학회 · Annals of Clinical Neurophysiology2011 · 제13권 제2호국내 근위축성측삭경화증 환자의 임상 특성 및 간병부담에 관한 예비 연구; 한국ALS협회를 통한 설문조사 연구김승현, 김주한, 김현영, 남유현, 박기형, 성정준, 이광우, 주인수대한신경과학회 · 대한신경과학회지2006 · 제24권 제3호근위축측삭경화증 환자에 동반되는 행동심리증상의 임상적 특징과 예후에 대한 영향곽현승, 김승현, 김희진, 박아람, 오기욱, 오성일대한치매학회 · Dementia and Neurocognitive Disorders(대한치매학회지)2014 · 제13권 제1호Homozygous SMN2 Deletion is a Major Risk Factor among Twenty-Five Korean Sporadic Amyotrophic Lateral Sclerosis Patients서경임, 이경아, 이준범, 최영철, 홍지만연세대학교의과대학 · Yonsei Medical Journal2012 · 제53권 제1호

이 간행물의 다른 논문 (17건)