BRAIN and NERVE－神経研究の進歩67巻4号

2015年04月発行

総説

大脳皮質基底核変性症の臨床診断基準と治療

著者： 下畑享良¹ 饗場郁子² 西澤正豊¹

所属機関： ¹新潟大学脳研究所神経内科 ²国立病院機構東名古屋病院神経内科

ページ範囲：P.513 - P.523

文献概要

4リピート・タウオパチーである大脳皮質基底核変性症は，さまざまな臨床表現型を呈するため，臨床診断は難しい。しかし，タウを標的とした病態抑止療法が現実のものとなりつつあることから，生前の正確な診断が求められている。Armstrongらは，2013年に新しい診断基準を提唱したが，2つの検証研究の結果から，診断基準の感度，特異度は必ずしも高くないことが示された。今後，診断バイオマーカーの探索が不可欠である。

参考文献

1) Rebeiz JJ, Kolodny EH, Richardson EP Jr: Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 18: 20-33, 1968

2) Tolosa E, Litvan I, Hoglinger GU, Burn D, Lees A, et al: A phase 2 trial of the GSK-3 inhibitor tideglusib in progressive supranuclear palsy. Mov Disord 29: 470-478, 2014

3) Hoglinger GU, Huppertz HJ, Wagenpfeil S, Andrés MV, Belloch V, et al: Tideglusib reduces progression of brain atrophy in progressive supranuclear palsy in a randomized trial. Mov Disord 29: 479-487, 2014

4) Boxer AL, Lang AE, Grossman M, Knopman DS, Miller BL, et al; AL-108-231 Investigators: Davunetide in patients with progressive supranuclear palsy: a randomised, double-blind, placebo-controlled phase 2/3 trial. Lancet Neurol 13: 676-685, 2014

5) ClinicalTrials. gov Identifier: NCT00532571

6) Gibb WR, Luthert PJ, Marsden CD: Corticobasal degeneration. Brain 112: 1171-1192, 1989

7) Steele JC, Richardson JC, Olszewski J: Progressive supranuclear palsy: a heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 10: 333-359, 1964

8) Ling H, O'Sullivan SS, Holton JL, Revesz T, Massey LA, et al: Does corticobasal degeneration exist?: a clinicopathological re-evaluation. Brain 133: 2045-2057, 2010

9) Grimes DA, Lang AE, Bergeron CB: Dementia as the most common presentation of cortical-basal ganglionic degeneration. Neurology 53: 1969-1974, 1999

10) Hughes AJ, Daniel SE, Ben-Shlomo Y, Lees AJ: The accuracy of diagnosis of parkinsonian syndromes in a specialist movement disorder service. Brain 125: 861-870, 2002

11) Murray R, Neumann M, Forman MS, Farmer J, Massimo L, et al: Cognitive and motor assessment in autopsy-proven corticobasal degeneration. Neurology 68: 1274-1283, 2007

12) Lee SE, Rabinovici GD, Mayo MC, Wilson SM, Seeley WW, et al: Clinicopathological correlations in corticobasal degeneration. Ann Neurol 70: 327-340, 2011

13) Kertesz A, Martinez-Lage P, Davidson W, Munoz DG: The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 55: 1368-1375, 2000

14) Alexander SK, Rittman T, Xuereb JH, Bak TH, Hodges JR, et al: Validation of the new consensus criteria for the diagnosis of corticobasal degeneration. J Neurol Neurosurg Psychiatry 85: 925-929, 2014

15) Jankovic J TEKR, Bergeron C, Pollanen M, Lang AE: Cortical-basal ganglionic degeneration. Jankovic J, Tolosa E (eds): Parkinson's Disease and Movement Disorders. Williams ＆ Wilkins, Baltimore, 1998, pp297-316

16) Lang AE RD, Bergeron C: Cortical-basal ganglionic degeneration. Calne DB (ed): Neurodegenerative Diseases. WB Saunders, Philadelphia, 1994, pp877-894

17) Watts RL MS, Richardson EP: Cortical-basal ganglionic degeneration. Marsden CD, Fahn S (eds): Movement Disorders, vol 3．Butterworth Heinemann, Oxford, 1994, pp282-299

18) Riley DE, Lang AE, Lewis A, Resch L, Ashby P, et al: Cortical-basal ganglionic degeneration. Neurology 40: 1203-1212, 1990

19) Litvan I, Cummings JL, Mega M: Neuropsychiatric features of corticobasal degeneration. J Neurol Neurosurg Psychiatry 65: 717-721, 1998

20) Halpern C, McMillan C, Moore P, Dennis K, Grossman M: Calculation impairment in neurodegenerative diseases. J Neurol Sci 208: 31-38, 2003

21) Bak TH, Hodges JR: Corticobasal degeneration: clinical aspects. Handb Clin Neurol 89: 509-521, 2008

22) Boeve BF, Lang AE, Litvan I: Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 54(Suppl 5): S15-19, 2003

23) Mathew R, Bak TH, Hodges JR: Diagnostic criteria for corticobasal syndrome: a comparative study. J Neurol Neurosurg Psychiatry 83: 405-410, 2012

24) 饗場郁子: Corticobasal syndrome——最近の進歩と今後の課題. Brain Nerve 64: 462-473, 2012

25) 下畑享良, 西澤正豊: CBSの臨床. Brain Nerve 65: 31-40, 2013

26) Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, et al: Criteria for the diagnosis of corticobasal degeneration. Neurology 80: 496-503, 2013

27) Riley DE, Lang AE: Clinical diagnostic criteria. Adv Neurol 82: 29-34, 2000

28) Lee SE, Rabinovici GD, Mayo MC, Wilson SM, Seeley WW, et al: Clinicopathological correlations in corticobasal degeneration. Ann Neurol 70: 327-340, 2011

29) Josephs KA, Petersen RC, Knopman DS, Boeve BF, Whitwell JL, et al: Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology 66: 41-48, 2006

30) Wenning GK, Litvan I, Jankovic J, Granata R, Mangone CA, et al: Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. J Neurol Neurosurg Psychiatry 64: 184-189, 1998

31) Ouchi H, Toyoshima Y, Tada M, Oyake M, Aida I, et al: Pathology and sensitivity of current clinical criteria in corticobasal syndrome. Mov Disord 29: 238-244, 2014

32) Piao YS, Tan CF, Iwanaga K, Kakita A, Takano H, et al: Sporadic four-repeat tauopathy with frontotemporal degeneration, parkinsonism and motor neuron disease. Acta Neuropathol 110: 600-609, 2005

33) Fu YJ, Nishihira Y, Kuroda S, Toyoshima Y, Ishihara T, et al: Sporadic four-repeat tauopathy with frontotemporal lobar degeneration, Parkinsonism, and motor neuron disease: a distinct clinicopathological and biochemical disease entity. Acta Neuropathol 120: 21-32, 2010

34) Ling H, O'Sullivan SS, Holton JL, Revesz T, Massey LA, et al: Does corticobasal degeneration exist?: a clinicopathological re-evaluation. Brain 133: 2045-2057, 2010

35) Cordivari C, Misra VP, Catania S, Lees AJ: Treatment of dystonic clenched fist with botulinum toxin. Mov Disord 16: 907-913, 2001

36) Muller J, Wenning GK, Wissel J, Seppi K, Poewe W: Botulinum toxin treatment in atypical parkinsonian disorders associated with disabling focal dystonia. J Neurology 249: 300-304, 2002

37) Boeve BF, Josephs KA, Drubach DA: Current and future management of the corticobasal syndrome and corticobasal degeneration. Handb Clin Neurol 89: 533-548, 2008

38) Kovacs T, Farsang M, Vitaszil E, Barsi P, Györke T, et al: Levetiracetam reduces myoclonus in corticobasal degeneration: report of two cases. J Neural Transm 116: 1631-1634, 2009

39) http://www.brain-bank.org/brain-bank/index.html

40) Kumar R, Bergeron C, Pollanen MS: Cortical-basal ganglionic degeneration. Jankovic J, Tolosa E (eds): Parkinson's Disease and Mmovement Disorders. Lippincott Williams ＆ Wilkins, Baltimore, 1998, pp297-316