Achiria
Achiria, also written acheiria, is the inability to recognise or to localise which side of the body has received a tactile stimulus, even though the touch itself is felt with normal intensity.失手症(achiria,亦写作 acheiria)指患者无法辨认或定位触觉刺激施加于身体的哪一侧,尽管触觉本身强度正常。
It belongs to the dyschirias, a group of spatial errors described at the end of the nineteenth century, and it is now understood as a bedside marker of right-hemisphere damage and unilateral neglect rather than as an independent disease.该症属于十九世纪末描述的一类空间定位错误即感觉侧别障碍,现今被视为右半球损伤与单侧忽略的床旁标志,而非独立疾病。
Terminology and classification
- The word derives from the Greek prefix a-, meaning without, and cheir, meaning hand, although the deficit concerns the whole half of the body and not only the hand.词源为希腊语前缀(a-,无)与(cheir,手),但缺损涉及整个半侧身体而非仅手部。
- Ernest Jones, a Welsh psychiatrist, introduced achiria in a 1907 paper in Brain and placed it at the primary stage of a three-part scheme of dyschiria.威尔士精神病学家 Ernest Jones 于 1907 年在《Brain》提出失手症,并将其置于感觉侧别障碍三分法的初级位置。[1]
- Jones distinguished achiria, in which the side is simply not identified, from allochiria, in which a stimulus is referred to the opposite side, and from synchiria, in which one stimulus is felt on both sides.Jones 区分了三种情况:失手症为侧别完全未被识别,异侧感觉为刺激被误指至对侧,同侧感觉为一个刺激被感知于两侧。
- Older British texts also call the sensory form simple allochiria, a usage that has caused lasting confusion because allochiria proper is a displacement rather than a failure of identification.较早的英国文献又称感觉型为单纯性异侧感觉,这一用法造成长期混淆,因为真正的异侧感觉是位置移置而非识别失败。
- The term fell out of favour after Marcel Kinsbourne replaced dyschiria with unilateral neglect in 1970, and modern clinics speak of tactile extinction, personal neglect or alloesthesia instead.1970 年 Marcel Kinsbourne 以单侧忽略取代感觉侧别障碍后,该词逐渐淡出,现代临床多称触觉消退、个人忽略或异侧感觉。
- Achiria has no dedicated code in WHO ICD-10 and no entry in MONDO or Orphanet, because it is a sign rather than a disease entity.失手症在 WHO ICD-10 中无专属编码,在 MONDO 与 Orphanet 中亦无条目,因为它属于体征而非疾病实体。
- The nearest formal categories are ICD-11 release 2025-01 code MB40.9 for neurological neglect syndrome and the United States clinical modification code R41.4 for attention and concentration deficit with neglect.最接近的正式分类是 ICD-11 2025-01 版的神经系统忽略综合征编码 MB40.9,以及美国临床修订版中伴忽略的注意障碍编码 R41.4。
- For interoperability the concept is indexed as Wikidata Q104844018, and the broader literature on spatial neglect is indexed under MeSH D010336 for perceptual disorders.互操作层面该概念登记为 Wikidata Q104844018,而空间忽略的相关文献归入 MeSH D010336 知觉障碍类别。
History
- Pierre Janet reported the first clinical case in 1899, describing a woman who could feel a touch but could not say on which side of her body it had been applied.皮埃尔·让内于 1899 年报告首例,描述一名女性能感到触碰却无法说出刺激施加于身体哪一侧。[2]
- Jones consolidated these observations in 1907 and 1909, naming sensory, motor and introspective forms and attributing them to a disorder of the body schema.Jones 于 1907 与 1909 年整合这些观察,命名感觉型、运动型与内省型,并将其归因于身体图式障碍。
- Experimental support came earlier, from Albert Paul Weiss in 1891, who sectioned the posterior columns of the spinal cord, and from Bosc in 1892, who used a hemispheric lesion to interrupt crossed sensory traffic.实验支持更早出现:Albert Paul Weiss 于 1891 年切断脊髓后柱,Bosc 于 1892 年以半球病变阻断交叉的感觉传导。
- Jones argued that hysterical patients lost the side of a stimulus because attention was withdrawn from one half of the body, a psychogenic explanation that dominated for three decades.Jones 认为癔症患者之所以丧失刺激的侧别,是注意从半侧身体撤出所致,这一心因性解释主导了三十年。
- The psychogenic model was overturned when careful testing showed intact primary sensation and intact peripheral pathways in most patients, and when the deficit proved to follow focal brain lesions.当细致检查显示多数患者初级感觉与外周通路完好,且缺损被证实随局灶性脑病变出现时,心因性模型被推翻。
- In 1970 Kinsbourne published a model of unilateral neglect of space, and the older vocabulary of dyschiria was progressively absorbed into the literature on spatial attention.1970 年 Kinsbourne 发表空间单侧忽略模型,旧有的感觉侧别障碍术语逐步被空间注意研究吸收。
- From the 1980s the bedside phenomenon was reconceptualised as extinction, a measurable competition between simultaneous stimuli, and standardised neglect batteries replaced informal side-of-touch questions.自 1980 年代起,该床旁现象被重新概念化为消退,即同时刺激之间的可测量竞争,标准化忽略量表取代了非正式的侧别询问。
Epidemiology
- Achiria itself has never been counted in population surveys, and all frequency estimates refer to the neglect syndrome of which it is one component.失手症本身从未进入人群调查,所有频率估计均针对其所属的忽略综合征。
- Systematic reviews of post-stroke neglect report a prevalence between 13 and 82 per cent acutely, with most well-designed studies clustering at 30 to 50 per cent.卒中后忽略的系统综述报告急性期患病率介于 13% 至 82%,其中多数设计良好的研究集中在 30% 至 50%。
- Neglect is far more common and more persistent after right-hemisphere stroke, whereas left-hemisphere lesions produce mild and transient forms that are easily missed.忽略在右半球卒中后更常见且更持久,而左半球病变所致者轻微短暂,极易漏诊。
- About one third of patients still show neglect three months after stroke, and persistence beyond that point predicts poor functional recovery and institutional care.约三分之一患者在卒中后三个月仍有忽略,超过该时点仍持续者预示功能恢复差与需机构照护。
- Age, hypertension, atrial fibrillation, diabetes and previous stroke raise the risk of the causative lesion, and advanced age is associated with more severe and more lasting neglect.年龄、高血压、心房颤动、糖尿病与既往卒中等提高致病病变的风险,高龄与更严重、更持久的忽略相关。
- Achiria is under-recognised because routine neurological examination rarely asks the patient to name the stimulated side, and chart review detects only a minority of cases.失手症被普遍低估,因为常规神经系统检查很少要求患者说出受刺激侧别,病历回顾仅能识别少数病例。
- The global burden is therefore carried by stroke statistics rather than by any achiria-specific dataset, and WHO lists stroke among the leading causes of death and disability worldwide.因此全球负担体现在卒中统计而非任何失手症专门数据集,WHO 将卒中列为全球主要死亡与致残原因之一。
Pathophysiology
- The responsible lesions lie most often in the right inferior parietal lobule, the temporoparietal junction and the posterior superior temporal gyrus, regions that integrate personal and extrapersonal space.责任病变最常位于右侧顶下小叶、颞顶交界与颞上回后部,这些区域整合身体空间与体外空间。
- Subcortical lesions of the right pulvinar, the posterior thalamus and the basal ganglia can reproduce the same error, and thalamic damage is particularly associated with allochiria.右侧丘脑枕、丘脑后部与基底节的皮质下病变可再现同样的错误,丘脑损伤尤其与异侧感觉相关。
- Three frameworks compete: a representational account in which the contralesional half of space is no longer encoded, an attentional account in which the ipsilesional field captures arousal, and an intentional account in which motor exploration is biased.三种框架相互竞争:表征说认为对侧半空间不再被编码,注意说认为同侧视野劫持了觉醒,意向说认为运动探索出现偏向。
- Interhemispheric rivalry is central to the attentional model, because the left hemisphere exerts unopposed inhibition on the damaged right hemisphere and drives attention rightward.半球间竞争是注意模型的核心,因为左半球对受损右半球施加失去对抗的抑制,使注意偏向右侧。
- Primary somatosensory pathways remain intact, so achiria is a failure of spatial attribution rather than a sensory loss, which is why the patient can describe the quality of the touch but not its side.初级躯体感觉通路保持完好,因此失手症是空间归属失败而非感觉丧失,这也是患者能描述触觉性质却不能说出侧别的原因。
- Dissociations are common, and a patient may show dense personal neglect while anosognosia for the same deficit is absent, indicating that awareness and spatial encoding depend on separable networks.分离现象常见,患者可有严重个人忽略却不对该缺损表现病感缺失,提示觉察与空间编码依赖可分离的网络。
Clinical features
- Sensory achiria is the inability to name the side of a touch while location, intensity and quality are judged correctly, and it is the form usually meant by the bare term.感觉型失手症指无法说出触觉侧别而位置、强度与性质判断正确,通常单独使用该词即指此型。
- Motor achiria is the inability to move the limb on command when the command specifies left or right, although the same movement can be performed when the side is indicated in another way.运动型失手症指当指令要求左或右时无法移动相应肢体,而改用其他方式指示侧别时同一动作可以完成。
- Introspective achiria is the loss of the feeling of owning or retaining memory for one half of the body, even though the patient knows that the part exists.内省型失手症指对半侧身体的所有感或感觉记忆丧失,尽管患者知道该部位存在。
- Allochiria and synchiria frequently accompany achiria, and the three can alternate within a single examination session as attention fluctuates.异侧感觉与同侧感觉常与失手症并存,且随注意波动可在同一次检查中交替出现。
- Personal neglect appears as shaving, dressing or washing only one side, and it can be present when spatial neglect on paper-and-pencil tasks is mild.个人忽略表现为只刮一侧胡须、只穿一侧衣物或只清洗半侧身体,且可在纸笔空间忽略轻微时独立存在。
- Tactile extinction on double simultaneous stimulation is the commonest bedside sign, with the left hand extinguished in right-hemisphere lesions.双侧同时刺激下的触觉消退是最常见的床旁体征,右半球病变时左手被消退。
- Anosognosia, hemispatial neglect, constructional apraxia and gaze deviation often coexist, and their combination predicts falls, wheelchair dependence and prolonged rehabilitation.病感缺失、半侧空间忽略、结构性失用与凝视偏斜常同时存在,其组合预示跌倒、依赖轮椅与康复期延长。
Diagnosis
- Bedside testing asks the patient to identify the side of a light touch to each hand and cheek, first singly and then with both sides stimulated together.床旁检查要求患者说出轻触左手、右手与面颊的侧别,先单侧后双侧同时刺激。
- The Behavioral Inattention Test, the Catherine Bergego Scale and cancellation or line-bisection tasks quantify the deficit and separate personal from extrapersonal neglect.行为忽略测验、Catherine Bergego 量表以及划消或线段二等分任务可量化缺损并区分个人忽略与体外空间忽略。
- Diagnosis requires exclusion of peripheral neuropathy, spinal cord disease and thalamic sensory stroke, because each can impair side localisation without neglect.诊断需排除周围神经病、脊髓疾病与丘脑感觉性卒中,因为它们均可造成侧别定位障碍而无忽略。
- The differential includes hemianopia, aphasia, conversion disorder, dementia with posterior cortical atrophy and unilateral limb apraxia, and each has a distinguishing bedside feature.鉴别诊断包括偏盲、失语、转换障碍、后皮质萎缩型痴呆与单侧肢体失用,各有可区分的床旁特征。
- MRI is the investigation of choice and should include diffusion-weighted sequences, because small thalamic or parietal infarcts are easily missed on CT.磁共振是首选检查并应包含弥散加权序列,因为小的丘脑或顶叶梗死易在 CT 上漏诊。
- A structured neglect screen should be routine in every stroke unit, since spontaneous reporting identifies fewer than half of affected patients.每个卒中单元都应常规进行结构化忽略筛查,因为自发报告仅能识别不足一半的患者。
Management
- There is no specific treatment for achiria, and management is the management of the underlying stroke with reperfusion where indicated and secondary prevention thereafter.失手症无特异性治疗,处理即针对原发卒中:有指征时再灌注,其后进行二级预防。
- Visual scanning training, cueing to the neglected side and limb activation are the best established behavioural techniques and are delivered by occupational therapists.视觉扫描训练、对忽略侧的提示与肢体激活是最成熟的行为技术,由作业治疗师实施。
- Prism adaptation, introduced experimentally by Rossetti and colleagues in 1998, produces short-term improvement in many patients but its durable benefit remains uncertain.棱镜适应由 Rossetti 及其同事于 1998 年引入实验研究,可使多数患者短期改善,但持久获益仍不确定。
- Non-invasive brain stimulation, including inhibitory repetitive transcranial magnetic stimulation over the intact left hemisphere, has shown inconsistent results in randomised trials.非侵入性脑刺激,包括对完好左半球施加抑制性重复经颅磁刺激,在随机试验中结果不一致。
- Dopaminergic drugs such as apomorphine and levodopa have been tested on the premise that neglect reflects a hypodopaminergic attention deficit, without consistent clinical benefit.多巴胺能药物如阿扑吗啡与左旋多巴曾被试用,前提是忽略反映多巴胺能性注意缺陷,但临床获益不一致。
- Falls prevention, safe mobilisation, family education and environmental modification are essential, and the rehabilitation team should screen for anosognosia before allowing unsupervised transfers.预防跌倒、安全移动、家属教育与环境的改造十分重要,康复团队应在允许无监护转移前筛查病感缺失。
- Persistent neglect predicts longer hospital stay, lower functional independence scores and higher rates of discharge to nursing homes, so it should be recorded as a prognostic flag.持续忽略预示住院时间更长、功能独立评分更低与转护理机构比例更高,因此应作为预后标志记录。
Research and controversies
- The taxonomy remains contested, because achiria, allochiria, extinction and neglect overlap clinically yet are treated as separate constructs in different traditions.分类仍有争议,因为失手症、异侧感觉、消退与忽略在临床上相互重叠,却在不同学术传统中被当作独立构念。
- The mechanism debate between representational and attentional accounts is unresolved, and lesion-symptom mapping has not consistently separated the two predictions.表征说与注意说之间的机制之争尚未解决,病变症状映射也未能稳定区分二者的预测。
- Trials of prism adaptation have produced conflicting results, with some showing benefit at one week and others finding no difference at six months, and heterogeneity of treatment dose is a leading explanation.棱镜适应试验结果相互冲突,部分显示一周时获益,另一些在六个月时未见差异,治疗剂量异质性被视为主要解释。
- Underdiagnosis remains the largest unmet need, because neglect is not coded in many routine datasets and therefore disappears from quality metrics and service planning.诊断不足是最大的未满足需求,因为忽略未进入许多常规数据集,从而在质量指标与服务规划中消失。
- Digital cancellation tasks, virtual reality assessment and wearable gaze tracking are being developed to make detection objective, but none has yet replaced the trained bedside examiner.数字划消任务、虚拟现实评估与可穿戴眼动追踪正在开发,以图使检测客观化,但尚无一项取代受训的床旁检查者。