额颞痴呆
Frontotemporal dementia
- 上级条目
- 痴呆
- 层级
- 4 级
定义
额颞痴呆（FTD）是一组主要影响额叶和颞叶的原发性神经退行性疾病。该病通常起病隐匿，病程逐渐加重。目前已描述了几种综合征变异（其中一些已确定有遗传基础或家族性），包括主要表现为明显的人格和行为改变（如执行功能障碍、冷漠、社会认知退化、重复行为和饮食改变）、主要表现为语言缺陷（包括语义、 语法失能/非流畅和 Logopenic型），主要与运动有关的缺陷（进行性核上性麻痹、皮质基底节变性、多系统萎缩或肌肉萎缩性侧索硬化症），或这些缺陷的组合。记忆功能通常保持相对完整，特别在疾病的早期阶段。
Frontotemporal dementia (FTD) is a group of primary neurodegenerative disorders primarily affecting the frontal and temporal lobes. Onset is typically insidious with a gradual and worsening course. Several syndromic variants (some with an identified genetic basis or familiality) are described that include presentations with predominantly marked personality and behavioral changes (such as executive dysfunction, apathy, deterioration of social cognition, repetitive behaviours, and dietary changes), predominantly language deficits (that include semantic, agrammatic/nonfluent, and logopenic forms), predominantly movement-related deficits (progressive supranuclear palsy, corticobasal degeneration, multiple systems atrophy, or amyotrophic lateral sclerosis), or a combination of these deficits. Memory function often remains relatively intact, particularly during the early stages of the disorder.
索引词 可用于检索该条目的同义词与别名
- 额颞痴呆
- 颞叶痴呆
- 额颞叶痴呆
- 额颞叶变性痴呆
- FTD[额颞叶痴呆]
- 额颞叶痴呆
- 额叶痴呆 [possible translation]
- 额叶痴呆
- 额颞叶痴呆，行为变异型
- Pick病引起的痴呆
- Pick病
- 脑皮克病
- 皮克病引起的痴呆
- 匹克病引起的痴呆
- 皮克病
- 匹克病
- 脑匹克病
- 脑Pick病 [possible translation]
- 脑Pick病
- 额颞叶痴呆，语言变异型
- 原发性进行性失语
- 进行性失语
- 额颞叶痴呆，非流利型或语法变异型失语
- PPA[原发性进行性失语]语法缺失型
- 进行性非流利型失语
- 进行性非流利性失语
- 额颞叶痴呆，语义变异型
- PPA[原发性进行性失语]语义型
- 语义性痴呆
- 额颞叶痴呆，少词变异型
- 少词型进行性失语
- 少词型原发性进行性失语
- logopenic型进行性失语
- logopenic型原发性进行性失语
- 额颞叶痴呆，logopenic型
- 额颞叶痴呆伴运动神经元病
- 额颞叶痴呆伴家族性包涵体肌病，伴Paget骨病
- 额颞叶痴呆伴家族性包涵体肌病，伴畸形性骨炎
- 基因突变所致额颞叶痴呆
- C9orf72突变所致额颞叶痴呆
- MAPT突变所致额颞叶痴呆
- VCP突变所致额颞叶痴呆
- GRN突变所致额颞叶痴呆
- CHMP2B突变所致额颞叶痴呆
- UBS突变所致额颞叶痴呆
- FUS突变所致额颞叶痴呆
- TARDBP突变所致额颞叶痴呆
- 其他或新突变所致额颞叶痴呆
英文词条（34）
- Frontotemporal dementia
- FTD - [frontotemporal dementia]
- dementia in fronto-temporal lobar degeneration
- frontal lobe dementia
- frontotemporal lobe dementia
- temple dementia
- Frontotemporal dementia, behavioural variant
- Dementia in Pick disease
- Pick disease
- Pick disease of brain
- Frontotemporal dementia, language variant
- Primary progressive aphasia
- Progressive aphasia
- Frontotemporal dementia, non-fluent or agrammatic variant
- PPA - [primary progressive aphasia] agrammatic
- progressive non-fluent aphasia
- Frontotemporal dementia, semantic variant
- PPA - [primary progressive aphasia] semantic
- Semantic dementia
- Frontotemporal dementia, logopenic variant
- Logopenic primary progressive aphasia
- Logopenic progressive aphasia
- Frontotemporal dementia with motor neuron disease
- Frontotemporal dementia with familial inclusion body myopathy with Paget’s disease of bone
- Frontotemporal dementia due to genetic mutation
- Frontotemporal dementia due to C9orf72 mutation
- Frontotemporal dementia due to MAPT mutation
- Frontotemporal dementia due to VCP mutation
- Frontotemporal dementia due to GRN mutation
- Frontotemporal dementia due to CHMP2B mutation
- Frontotemporal dementia due to UBS mutation
- Frontotemporal dementia due to FUS mutation
- Frontotemporal dementia due to TARDBP mutation
- Frontotemporal dementia due to other or new mutations
编码备注 Coding note
这些分类不可用作主要编码。当需要标识分类于他处的疾病引起的痴呆时，可将这些编码作为补充或附加编码。 对于多种病因引起的痴呆，对所有适用的病因进行编码。
This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere. When dementia is due to multiple aetiologies, code all that apply.
后组配轴 Postcoordination
后组配是在主干编码之后追加的可选扩展轴（如侧别、具体解剖部位、严重程度），用于把「部位 + 侧别 + 病程」等细节一次表达完整。只有标注「必填」的轴在组合编码时必须给出，其余可留空。
- 相关疾患associatedWith可选
- 严重程度hasSeverity可选
- 病因hasCausingCondition必填