DSM-5中有關失智症的新觀念 New concept of major neurocognitive disorder in DSM-5 歐陽文貞 , M.D., M.P.H., Ph.D. 衛生署嘉南療養院高年精神科主任 台灣老年精神醫學會常務理事, 台灣精神醫學會監事 台灣臨床失智症學會理事 台灣失智症協會理事 1 Classification of mental disorders & diagnostic criteria • • • • • • • • • • 1952 DSM-I 1968 DSM-II & 1977(79) 1980 DSM-III, 1987 DSM-IIIR 1992 1994 DSM-IV 2000 DSM-IV-TR 2013 DSM-5 2014 (ISO 成立1947於日內瓦) ICD-8 ICD-9(ICD-9-CM) NINCDS-ADRDA (1984) ICD-10 ICD-11(revision from 2007) 2 from ICD-9 to ICD-10 1. ICD-9 (1977) • 290-299 Psychosis – Dementia and organic psychotic condition(290-294) – Other psychosis • 295 schizophrenia, 296 MDP, 297 paranoid state • 298 other psychosis • 300-314 Neurosis 2. ICD-10(1992) 10 categories F0 Organic, including symptomatic, mental disorder F2 schizophrenia, schizotypal, delusional disorder 3 ICD-9 290-294: organic psychotic condition之一 dementia • 290 老年期及初老期器質性精神病狀態(senile & pre-senile organic psychotic condition) – 290.0 senile dementia, simple type – 290.1 pre-senile dementia – 290.2 senile dementia, depressed(290.21) or paranoid type(290.20) – 290.3 senile dementia with acute confusional state – 290.4 artheriosclerotic dementia – (290.5 mixed dementia) 4 ICD-10 organic, including symptomatic, mental disorder • • • • • • • • • F00 dementia in Alzheimer’s disease F01 vascular dementia F02 dementia in other disease classified elsewhere F03 unspecified dementia F04 organic amnesic syndrome, not induced by alcohol and other psychoactive substance F05 delirium, not induced by alcohol and other psychoactive substance F06 other mental disorder due to brain damage and dysfunction and to physical disease F07 personality and behavior disorder due to brain damage and dysfunction and to physical disease F09 unspecified organic or symptomatic mental disorder 5 ICD-10 dementia • • • • • • • F00.0 dementia in AD with early onset , F00.1 dementia in AD with late onset F01.0 vascular dementia of acute onset F01.1 multi-infarct dementia F01.2 subcortical vascular dementia F01.3 mixed cortical and subcortical dementia F02.x Pick’s disease, CJD, Huntington’s disease, Parkinson’s disease, HIV, other specified disease 6 ICD-10-DCR general criteria for dementia • G1 evidence of each of the following: – (1) decline in memory(mild, moderate, severe) – (2)decline in other cognitive abilities • G2 awareness of the environment is preserved • G3 decline in emotional control or motivation, or a change in social behavior – (1)emotional lability, (2)irritability – (3)apathy (4)corasening of social behavior • G4 present for at least 6 months 7 From DSM-III to DSM-5 • DSM-III (1980), DSM-IV (1994) • DSM-IV-TR (2000): delirium, dementia, and amnesic and other cognitive disorders – – – – – – – Delirium Substance-induced delirium Dementia Amnesic disorder Amnesic disorder NOS Other cognitive disorder NOS Mental disorder due to a general medical condition 8 DSM-IV-TR: dementia syndrome • 294.1x Dementia of the Alzheimer’s type (原DSM-IV coding 及ICD-9 及健保局 290.0, 290.1x, 290.2x) • 290.4x Vascular dementia (formerly multi-infarct dementia) • 294.1x Dementia due to other general medical condition, head injury, Pick’s disease, ,,,, • 292.8x, 291.2x Substance-induced persisting dementia • Dementia due to multiple etiologies • 294.8 Dementia NOS 9 DSM-IV-TR dementia of Alzheimer’s type • A. both – – • • • • • (1)memory impairment, (2)aphasia, agnosia, apraxia, disturbing in executive function B. significant impairment in ….. C . Gradual onset D. not due to other causes E. not occur exclusively during the course of a delirium F. not better accounted for by anther Axis I diagnosis, MDD or schizophrenia 10 DSM-5: Neurocognitive disorder • S 00 - 02 Delirium – S 00 Delirium – S 01 Substance-Induced Delirium – S 02 Delirium Not Elsewhere Classified • S 03 Mild Neurocognitive Disorder • S 04 Major Neurocognitive Disorder • Subtypes of Major and Mild Neurocognitive Disorders 11 DSM-5: neurocognitive disorder • • • • • • • • • • • S XX.01 Neurocognitive Disorder due to Alzheimer's Disease S XX.02 Vascular Neurocognitive Disorder S XX.03 Frontotemporal Neurocognitive Disorder S XX.04 Neurocogntive Disorder due to Traumatic Brain Injury S XX.05 Neurocognitive Disorder due to Lewy Body Dementia S XX.06 Neurocognitive Disorder due to Parkinson's Disease S XX.07 Neurocognitive Disorder due to HIV Infection S XX.08 Substance-Induced Neurocognitive Disorder S XX.09 Neurocognitive Disorder due to Huntington's Disease S XX.10 Neurocognitive Disorder due to Prion Disease S XX.11 Neurocognitive Disorder due to Another Medical Condition • S XX.12 Neurocognitive Disorder Not Elsewhere Classified 12 DSM-5: major neurocognitive disorder • A. Evidence of substantial cognitive decline from a previous level of performance in one or more of the domains outlined above based on: • 1. Concerns of the individual, a knowledgeable informant, or the clinician that there has been a substantial decline in cognitive function; and • 2. A decline in neurocognitive performance, typically involving test performance in the range of 2 or more standard deviations below appropriate norms (i.e., below the 3rd percentile) on formal testing or equivalent clinical evaluation. (太寬了??) • B. The cognitive deficits are sufficient to interfere with independence (i.e., requiring assistance at a minimum with instrumental activities of daily living [more complex tasks such as paying bills or managing medications]). • C. The cognitive deficits do not occur exclusively in the context of a Delirium. • D. The cognitive deficits are not primarily attributable to another mental disorder (e.g., Major Depressive Disorder, Schizophrenia). 13 修改的理由1 • 1.Major Neurocognitive Disorder (including what was formerly known as Dementia) is a disorder with greater cognitive deficits in at least one (typically two or more) of the following domains: • Complex attention (sustained attention, divided attention, selective attention, processing speed), • Executive ability (planning, decision-making, working memory, responding to feedback/error correction, overriding habits, mental flexibility), • Learning and memory(immediate memory, recent memory [including free recall, cued recall, and recognition memory]) • Language(expressive language [including naming, fluency, grammar and syntax] and receptive language), • Visuoconstructional-perceptual ability (construction and visual perception),and • Social cognition (recognition of emotions, theory of mind, behavioral regulation). • 2.The cognitive deficits must be sufficient to interfere with functional independence. Important changes from the DSM-IV criteria include: change in nomenclature (MNCD or Dementia), not necessarily requiring memory to be one of the impaired domains, allowing cognitive deficit limited to one domain. 14 NINCDS-ADRDA 的 AD • The NINCDS-ADRDA Alzheimer's Criteria specify 8 cognitive domains that may be impaired in AD: – – – – – – – memory, language, perceptual skills, constructive abilities, orientation, (DSM-5沒有此名詞) attention, problem solving and functional abilities(DSM-5沒有此 名詞, 但是 DSM -5多了social cognition ). 15 NINCDS-ADRDA-1984, 最常用的診斷標準之一 • Definite Alzheimer's disease: The patient meets the criteria for probable Alzheimer's disease and has histopathologic evidence of AD via autopsy or biopsy. • Probable Alzheimer's disease: Dementia has been established by clinical and neuropsychological examination. Cognitive impairments also have to be progressive and be present in two or more areas of cognition. The onset of the deficits has been between the ages of 40 and 90 years and finally there must be an absence of other diseases capable of producing a dementia syndrome. • Possible Alzheimer's disease: There is a dementia syndrome with an atypical onset, presentation or progression; and without a known etiology; but no co-morbid diseases capable of producing dementia are believed to be in the origin of it. • Unlikely Alzheimer's disease: The patient presents a dementia syndrome with a sudden onset, focal neurologic signs, or seizures or gait disturbance early in the course of the illness. 16 修改的理由2 • 3.The term “dementia” is replaced by Major Neurocognitive Disorder, which is conceptualized as including what was formerly known as dementia as well as entities like amnestic disorder. “Dementia” is an accepted term for older adults (e.g., with Alzheimer’s disease)—although even in this setting it has acquired a pejorative or stigmatizing connotation, it is less well accepted among younger adults with deficits related to e.g., HIV or head injury. (去汙名化) • 4.This rewording focuses on decline (rather than deficit—consistent with the requirement in the basic definition of an acquired disorder) from a previous level of performance. (和ICD-10 一致, 變寬) • 5.The previous criteria for dementia used Alzheimer’s disease as their prototype and thus required memory impairment as a criterion for all dementias. There is growing recognition that, in other neurocognitive disorders (e.g., HIV-related cognitive decline, cerebrovascular disease, frontotemporal degeneration, traumatic brain injury, etc.), other domains such as language or executive functions may be impaired first, or exclusively, depending on the part of the brain affected and the natural history of the disease. • 6. The terminology for the cognitive domains has been updated to reflect current usage in neuropsychology and neurology. 17 修改的理由3 • 7.The new definition, consistent with DSM-wide changes, focuses first on performance rather than disability. In the introductory table, we provide for each domain examples of specific symptoms or observations consistent with the Major level of decline and objective assessments. This encourages the use of objective measures, including formal neuropsychological testing where feasible with lesser exclusive reliance on individual judgment. • 8.The presence of both symptoms/observations and objective assessment is included to ensure specificity. This is a larger issue for Minor Neurocognitive Disorder but included here for parallel structure of the criteria. • NOTE: …. refining criteria A1 and A2 to achieve a balance between preferred formal neuropsychological test 及臨床判斷….. • 9. The new language preserves the traditional function-based threshold for dementia but tries to operationalize it more clearly as a loss of independence. • NOTE: The committee is still refining criterion D and discussing to what extent Major Neurocognitive Disorder should be diagnosed in the setting of disorders like schizophrenia and depression (although this concern applies primarily to Minor Neurocognitive Disorder). (不排斥精神分裂病 可以有”重大認知障礙症”) 18 DSM-5 Major neurocognitive disorder • 增加 DLB, FTD診斷, 維持MNCD due to Traumatic brain injury • AD仍保有以記憶力早期受影響的症狀, 緩慢 發病 • VaD不強調focal neurological sign, sudden onset, stepwise course 19 DSM-5 vascular MNCD • B. The clinical features are consistent with a vascular etiology as suggested by one of the following: • 1. The onset of the cognitive deficits is temporally related to one or more vascular events • 2. Evidence for decline is prominent in speed of information processing, complex attention and/or frontal-executive functioning 20 DSM-5 vascular MNCD, specify if • 1. Probable: Vascular Neurocognitive Disorder is considered probable if: – a) Clinical criteria are supported by neuroimaging evidence of significant parenchymal injury due to cerebrovascular disease (neuroimaging-supported) – b) The neurocognitive syndrome is temporally related to one or more cerebrovascular events and there is documented evidence of these events – c) If both clinical and genetic (e.g. cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, CADASIL) evidence of cerebrovascular disease are present. 21 MNCD due to Lewy Body dementia • Core Diagnostic Features of Lewy Body Disease include the following: – a) Fluctuating cognition with pronounced variations in attention and alertness. – b) Recurrent visual hallucinations that are typically well-formed and detailed. – c) Spontaneous features of parkinsonism with onset at least 1 year later than the cognitive impairment. • Suggestive Diagnostic Features of Lewy Body Disease include the following: • a) REM sleep behavior disorder • b) Severe neuroleptic sensitivity • c) Low dopamine transporter uptake in basal ganglia demonstrated by SPECT or PET imaging. 22 Summary and take home message • Dementia was renamed as MNCD • Major vs minor NCD • Criteria of MNCD are different from those of dementia in DSM-IV-TR or ICD-10, approaching those of NINCDS-ADRDA. – Amnesia is not the only core symptom of MNCD. • Add Dx of FTD and DLB in DSM-5. • Some of concept of vascular NCD was changed 23 Thank you for your attention! 24
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