Now processing ... 
 Now searching ... 
 Now loading ... 

認知症診断のフローチャート

認知症診断の手順。
VaD:血管性認知症(vascular dementia) 
FTLD:前頭側頭葉変性症(frontotemporal lobar degeneration)
DLB:Lewy小体型認知症(dementia with Lewy bodies)
CJD:Creutzfeldt-Jakob病(Creutfeldt-Jakob disease)
PSD:periodic synchronous discharge
DWI:diffusion weighted image、
PSP:進行性核上性麻痺(progressive supranuclear palsy)
CBD:大脳基底核変性症(corticobasal degeneration)
*現在は、CBDは病理学的診断として用い、CBS:corticobasal syndrome とされる場合が多い(Cordato NJ, Halliday GM, McCann H, Davies L, Williamson P, Fulham M, Morris JG. Corticobasal syndrome with tau pathology. Mov Disord. 2001 Jul;16(4):656-67. PubMed PMID: 11481689)。
HD:Huntington病(Huntington’s disease)
出典
img
1: 日本神経学会監修、「認知症疾患診療ガイドライン」作成委員会編集:認知症疾患診療ガイドライン 2017, p37, 図1. 医学書院, 2017

ICD-10による認知症診断基準の要約

認知症診断 1
出典
img
1: 日本神経学会監修、「認知症疾患診療ガイドライン」作成委員会編集:認知症疾患診療ガイドライン 2017, p2, 表2. 医学書院, 2017

DSM-5による認知症の診断基準

米国精神医学会(American Psychiatric Association)出版の精神疾患の診断・統計マニュアル 第5版:Diagnostic and Statistical Manual of Mental Disorders;DSM-5(2013/05/18)では、『Dementia』という用語は『Neurocognitive Disorders(神経認知障害群)』に置きかえられ、認知症の診断基準として記憶障害が必須項目から外れている。
Neurocognitive disordersは、
 1.complex attention(複雑性注意)
 2.executive function(実行機能)
 3.learning and memory(学習および記憶)
 4.Language(言語)
 5.perceptual-motor(知覚-運動)
 6.social cognition(社会的認知)
の6つの主要神経認知領域で障害水準・重症度が判定され、日常生活自立度により、『Major Neurocognitive Disorders』と『Mild Neurocognitive Disorders』に分類される。『Mild Neurocognitive Disorders』は日常生活の自立性はほぼ障害されていない状態で、軽度認知障害 Mild Cognitive Impairment:MCIに相当する。
出典
img
1: 日本精神神経学会 日本語版用語監修、高橋三郎、大野 裕 監訳:DSM-5 精神疾患の診断・統計マニュアル, p594.医学書院,2014

NIA-AAによる認知症診断基準の要約

認知症診断 3
出典
img
1: 日本神経学会監修、「認知症疾患診療ガイドライン」作成委員会編集:認知症疾患診療ガイドライン 2017, p3, 表2. 医学書院, 2017

レビー小体

a:黒質;HE染色。
b:黒質;抗α-synuclein抗体を用いた免疫染色でのレビー小体とα-synuclein陽性neurites (Lewy neuritisと表現されることもある)。
c:大脳皮質;HE染色。
d:大脳皮質;抗α-synuclein抗体を用いた免疫染色でのレビー小体。

MRI冠状断 

臨床上、認知機能が同程度のアルツハイマー型認知症(a)とレビー小体型認知症 。内側頭葉(特に海馬)の萎縮は、レビー小体型認知症 (b)では、剖検所見と同様、明らかに軽度。
a:ATD、b:DLB
出典
imgimg
1: Dementia with Lewy bodies.
著者: McKeith I, Mintzer J, Aarsland D, Burn D, Chiu H, Cohen-Mansfield J, Dickson D, Dubois B, Duda JE, Feldman H, Gauthier S, Halliday G, Lawlor B, Lippa C, Lopez OL, Carlos Machado J, O'Brien J, Playfer J, Reid W; International Psychogeriatric Association Expert Meeting on DLB.
雑誌名: Lancet Neurol. 2004 Jan;3(1):19-28. doi: 10.1016/s1474-4422(03)00619-7.
Abstract/Text: Dementia with Lewy bodies (DLB) is the second commonest cause of neurodegenerative dementia in older people. It is part of the range of clinical presentations that share a neuritic pathology based on abnormal aggregation of the synaptic protein alpha-synuclein. DLB has many of the clinical and pathological characteristics of the dementia that occurs during the course of Parkinson's disease. Here we review the current state of scientific knowledge on DLB. Accurate identification of patients is important because they have specific symptoms, impairments, and functional disabilities that differ from those of other common types of dementia. Severe neuroleptic sensitivity reactions are associated with significantly increased morbidity and mortality. Treatment with cholinesterase inhibitors is well tolerated by most patients and substantially improves cognitive and neuropsychiatric symptoms. Clear guidance on the management of DLB is urgently needed. Virtually unrecognised 20 years ago, DLB could within this decade be one of the most treatable neurodegenerative disorders of late life.
Lancet Neurol. 2004 Jan;3(1):19-28. doi: 10.1016/s1474-4422(03)00619-7...

脳血流シンチ IMP 3DSSP

80歳 男性
68歳頃よりREM睡眠行動障害出現。70歳頃より原因不明の意識消失・転倒。
その後、姿勢異常・歩行障害 パーキンソン歩行出現
79歳 幻視出現。
両側後頭葉の血流低下が確認される。
出典
img
1: 著者提供

単一光子放射断層撮影像(SPECT)

フルオロプロピルを用いた線条体レベルでのドパミントランスポーター 単一光子放射断層撮影像(SPECT)はレビー小体型認知症 (DLB)では正常コントロール、アルツハイマー型認知症(ATD)と比して線条体(被殻:putamen)での著しい低下が確認される。
出典
imgimg
1: Dementia with Lewy bodies.
著者: McKeith I, Mintzer J, Aarsland D, Burn D, Chiu H, Cohen-Mansfield J, Dickson D, Dubois B, Duda JE, Feldman H, Gauthier S, Halliday G, Lawlor B, Lippa C, Lopez OL, Carlos Machado J, O'Brien J, Playfer J, Reid W; International Psychogeriatric Association Expert Meeting on DLB.
雑誌名: Lancet Neurol. 2004 Jan;3(1):19-28. doi: 10.1016/s1474-4422(03)00619-7.
Abstract/Text: Dementia with Lewy bodies (DLB) is the second commonest cause of neurodegenerative dementia in older people. It is part of the range of clinical presentations that share a neuritic pathology based on abnormal aggregation of the synaptic protein alpha-synuclein. DLB has many of the clinical and pathological characteristics of the dementia that occurs during the course of Parkinson's disease. Here we review the current state of scientific knowledge on DLB. Accurate identification of patients is important because they have specific symptoms, impairments, and functional disabilities that differ from those of other common types of dementia. Severe neuroleptic sensitivity reactions are associated with significantly increased morbidity and mortality. Treatment with cholinesterase inhibitors is well tolerated by most patients and substantially improves cognitive and neuropsychiatric symptoms. Clear guidance on the management of DLB is urgently needed. Virtually unrecognised 20 years ago, DLB could within this decade be one of the most treatable neurodegenerative disorders of late life.
Lancet Neurol. 2004 Jan;3(1):19-28. doi: 10.1016/s1474-4422(03)00619-7...

123I MIBG心筋シンチグラム

a:DLB
b:ATD
ATDでは正常な心筋への取り組みだが、DLBでは広範囲・全体的に心筋への取り組みが低下している。

[11C]-dihydrotetrabenazine PET

A:正常な被験者
B:非対称的な損失が顕著である認知症のない初期のパーキンソン病の患者
C:認知症のパーキンソン病
D:レビー小体型認知症
出典
imgimg
1: An update on brain imaging in parkinsonian dementia.
著者: Petrou M, Kotagal V, Bohnen NI.
雑誌名: Imaging Med. 2012 Apr;4(2):201-213. doi: 10.2217/iim.12.10.
Abstract/Text: Disturbances of cognition are frequent in Parkinson's disease (PD). Unlike severe loss of dopamine early in PD, extensive cholinergic losses have been consistently reported in PD with dementia. Cholinergic imaging suggests that basal forebrain cholinergic system degeneration appears early in PD and worsens with dementia development. Cortical cholinergic denervation is similar in PD with dementia and dementia with Lewy bodies, supporting a common disease spectrum, at least with respect to cholinergic pathology. Presence of cerebral amyloidopathy in the setting of parkinsonism may accelerate cognitive decline. Novel MRI techniques illustrate the widespread presence of neurodegeneration in PD with dementia, affecting white matter tracts and connectivity functions. This review will outline current concepts regarding dementia development in PD and discuss their correlation with functional and structural neuroimaging including PET and MRI.
Imaging Med. 2012 Apr;4(2):201-213. doi: 10.2217/iim.12.10.

Research criteria for the clinical diagnosis of probable and possible MCI-LB

出典
imgimg
1: Research criteria for the diagnosis of prodromal dementia with Lewy bodies.
著者: McKeith IG, Ferman TJ, Thomas AJ, Blanc F, Boeve BF, Fujishiro H, Kantarci K, Muscio C, O'Brien JT, Postuma RB, Aarsland D, Ballard C, Bonanni L, Donaghy P, Emre M, Galvin JE, Galasko D, Goldman JG, Gomperts SN, Honig LS, Ikeda M, Leverenz JB, Lewis SJG, Marder KS, Masellis M, Salmon DP, Taylor JP, Tsuang DW, Walker Z, Tiraboschi P; prodromal DLB Diagnostic Study Group.
雑誌名: Neurology. 2020 Apr 28;94(17):743-755. doi: 10.1212/WNL.0000000000009323. Epub 2020 Apr 2.
Abstract/Text: The prodromal phase of dementia with Lewy bodies (DLB) includes (1) mild cognitive impairment (MCI), (2) delirium-onset, and (3) psychiatric-onset presentations. The purpose of our review is to determine whether there is sufficient information yet available to justify development of diagnostic criteria for each of these. Our goal is to achieve evidence-based recommendations for the recognition of DLB at a predementia, symptomatic stage. We propose operationalized diagnostic criteria for probable and possible mild cognitive impairment with Lewy bodies, which are intended for use in research settings pending validation for use in clinical practice. They are compatible with current criteria for other prodromal neurodegenerative disorders including Alzheimer and Parkinson disease. Although there is still insufficient evidence to propose formal criteria for delirium-onset and psychiatric-onset presentations of DLB, we feel that it is important to characterize them, raising the index of diagnostic suspicion and prioritizing them for further investigation.

Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
Neurology. 2020 Apr 28;94(17):743-755. doi: 10.1212/WNL.00000000000093...

出典
imgimg
1: Research criteria for the diagnosis of prodromal dementia with Lewy bodies.
著者: McKeith IG, Ferman TJ, Thomas AJ, Blanc F, Boeve BF, Fujishiro H, Kantarci K, Muscio C, O'Brien JT, Postuma RB, Aarsland D, Ballard C, Bonanni L, Donaghy P, Emre M, Galvin JE, Galasko D, Goldman JG, Gomperts SN, Honig LS, Ikeda M, Leverenz JB, Lewis SJG, Marder KS, Masellis M, Salmon DP, Taylor JP, Tsuang DW, Walker Z, Tiraboschi P; prodromal DLB Diagnostic Study Group.
雑誌名: Neurology. 2020 Apr 28;94(17):743-755. doi: 10.1212/WNL.0000000000009323. Epub 2020 Apr 2.
Abstract/Text: The prodromal phase of dementia with Lewy bodies (DLB) includes (1) mild cognitive impairment (MCI), (2) delirium-onset, and (3) psychiatric-onset presentations. The purpose of our review is to determine whether there is sufficient information yet available to justify development of diagnostic criteria for each of these. Our goal is to achieve evidence-based recommendations for the recognition of DLB at a predementia, symptomatic stage. We propose operationalized diagnostic criteria for probable and possible mild cognitive impairment with Lewy bodies, which are intended for use in research settings pending validation for use in clinical practice. They are compatible with current criteria for other prodromal neurodegenerative disorders including Alzheimer and Parkinson disease. Although there is still insufficient evidence to propose formal criteria for delirium-onset and psychiatric-onset presentations of DLB, we feel that it is important to characterize them, raising the index of diagnostic suspicion and prioritizing them for further investigation.

Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
Neurology. 2020 Apr 28;94(17):743-755. doi: 10.1212/WNL.00000000000093...

Summary of key features of psychiatric-onset DLB

出典
imgimg
1: Research criteria for the diagnosis of prodromal dementia with Lewy bodies.
著者: McKeith IG, Ferman TJ, Thomas AJ, Blanc F, Boeve BF, Fujishiro H, Kantarci K, Muscio C, O'Brien JT, Postuma RB, Aarsland D, Ballard C, Bonanni L, Donaghy P, Emre M, Galvin JE, Galasko D, Goldman JG, Gomperts SN, Honig LS, Ikeda M, Leverenz JB, Lewis SJG, Marder KS, Masellis M, Salmon DP, Taylor JP, Tsuang DW, Walker Z, Tiraboschi P; prodromal DLB Diagnostic Study Group.
雑誌名: Neurology. 2020 Apr 28;94(17):743-755. doi: 10.1212/WNL.0000000000009323. Epub 2020 Apr 2.
Abstract/Text: The prodromal phase of dementia with Lewy bodies (DLB) includes (1) mild cognitive impairment (MCI), (2) delirium-onset, and (3) psychiatric-onset presentations. The purpose of our review is to determine whether there is sufficient information yet available to justify development of diagnostic criteria for each of these. Our goal is to achieve evidence-based recommendations for the recognition of DLB at a predementia, symptomatic stage. We propose operationalized diagnostic criteria for probable and possible mild cognitive impairment with Lewy bodies, which are intended for use in research settings pending validation for use in clinical practice. They are compatible with current criteria for other prodromal neurodegenerative disorders including Alzheimer and Parkinson disease. Although there is still insufficient evidence to propose formal criteria for delirium-onset and psychiatric-onset presentations of DLB, we feel that it is important to characterize them, raising the index of diagnostic suspicion and prioritizing them for further investigation.

Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
Neurology. 2020 Apr 28;94(17):743-755. doi: 10.1212/WNL.00000000000093...

Multimodal biomarkers for prodromal DLB

出典
imgimg
1: Research criteria for the diagnosis of prodromal dementia with Lewy bodies.
著者: McKeith IG, Ferman TJ, Thomas AJ, Blanc F, Boeve BF, Fujishiro H, Kantarci K, Muscio C, O'Brien JT, Postuma RB, Aarsland D, Ballard C, Bonanni L, Donaghy P, Emre M, Galvin JE, Galasko D, Goldman JG, Gomperts SN, Honig LS, Ikeda M, Leverenz JB, Lewis SJG, Marder KS, Masellis M, Salmon DP, Taylor JP, Tsuang DW, Walker Z, Tiraboschi P; prodromal DLB Diagnostic Study Group.
雑誌名: Neurology. 2020 Apr 28;94(17):743-755. doi: 10.1212/WNL.0000000000009323. Epub 2020 Apr 2.
Abstract/Text: The prodromal phase of dementia with Lewy bodies (DLB) includes (1) mild cognitive impairment (MCI), (2) delirium-onset, and (3) psychiatric-onset presentations. The purpose of our review is to determine whether there is sufficient information yet available to justify development of diagnostic criteria for each of these. Our goal is to achieve evidence-based recommendations for the recognition of DLB at a predementia, symptomatic stage. We propose operationalized diagnostic criteria for probable and possible mild cognitive impairment with Lewy bodies, which are intended for use in research settings pending validation for use in clinical practice. They are compatible with current criteria for other prodromal neurodegenerative disorders including Alzheimer and Parkinson disease. Although there is still insufficient evidence to propose formal criteria for delirium-onset and psychiatric-onset presentations of DLB, we feel that it is important to characterize them, raising the index of diagnostic suspicion and prioritizing them for further investigation.

Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
Neurology. 2020 Apr 28;94(17):743-755. doi: 10.1212/WNL.00000000000093...

認知症診断のフローチャート

認知症診断の手順。
VaD:血管性認知症(vascular dementia) 
FTLD:前頭側頭葉変性症(frontotemporal lobar degeneration)
DLB:Lewy小体型認知症(dementia with Lewy bodies)
CJD:Creutzfeldt-Jakob病(Creutfeldt-Jakob disease)
PSD:periodic synchronous discharge
DWI:diffusion weighted image、
PSP:進行性核上性麻痺(progressive supranuclear palsy)
CBD:大脳基底核変性症(corticobasal degeneration)
*現在は、CBDは病理学的診断として用い、CBS:corticobasal syndrome とされる場合が多い(Cordato NJ, Halliday GM, McCann H, Davies L, Williamson P, Fulham M, Morris JG. Corticobasal syndrome with tau pathology. Mov Disord. 2001 Jul;16(4):656-67. PubMed PMID: 11481689)。
HD:Huntington病(Huntington’s disease)
出典
img
1: 日本神経学会監修、「認知症疾患診療ガイドライン」作成委員会編集:認知症疾患診療ガイドライン 2017, p37, 図1. 医学書院, 2017

ICD-10による認知症診断基準の要約

認知症診断 1
出典
img
1: 日本神経学会監修、「認知症疾患診療ガイドライン」作成委員会編集:認知症疾患診療ガイドライン 2017, p2, 表2. 医学書院, 2017