Pommepuy N, Januel D
Service du Docteur Bouley, EPS de Ville-Evrard, 202 avenue Jean Jaurès, 93330 Neuilly-sur-Marne.
Encephale. 2002 Nov-Dec;28(6 Pt 1):481-92.
Catatonia was first described in 1874 by Kahlbaum as being a cyclic disease mixing motor features and mood variations. Because most cases ended in dementia, Kraepelin recognized catatonia as a form of dementia praecox and Bleuler included it within his wide group of schizophrenias. This view influenced the psychiatric practice for more than 70 years. But catatonia was recently reconsidered and this because of the definition of more precise diagnosis criteria, the discovery of a striking association with mood disorders, and the emphasis on effective therapeutics. Peralta et al empirically developed a performant diagnostic instrument with the 11 most discriminant signs among catatonic features. Diagnostic threshold is three or more signs with sensitivity of 100% and specificity of 99%. These signs are: immobility/stupor (extreme passivity, marked hypokinesia); mutism (includes inaudible whisper); negativism (resistance to instructions, contrary comportment to whose asked); oppositionism, other called gegenhalten (resistance to passive movement which increases with the force exerted); posturing (patient adopts spontaneously odd postures); catalepsy (patient retains limb positions passively imposed during examination; waxy flexibility); automatic obedience (exaggerated co-operation to instructed movements); echo phenomena (movements, mimic and speech of the examiner are copied with modification and amplifications); rigidity (increased muscular tone); verbigeration (continuous and directionless repetition of single words or phrases); withdrawal/refusal to eat or drink (turning away from examiner, no eye contact, refusal to take food or drink when offered). Using this diagnostic tool, prevalence of catatonic syndrome appears to be close to 8% of psychiatric admissions. Other signs are also common but less specific: staring, ambitendance, iterations, stereotypes, mannerism, overactivity/excitement, impulsivity, combativeness. Some authors complete this description by adding an affective dimension which is considered specific. Clinical forms are differentiated according to evolution: acute, chronic and periodic forms exist; and symptomatology: excited catatonias have a best prognostic than retarded catatonias. Malignant catatonia is the most studied form because of its severity and high rate of mortality (25%); catatonic patients develop autonomic disturbances with labile blood pressure, hyperthermia, diaphoresis, etc. Malignant catatonia requires ECT intervention in emergency. While catatonias due to general medical conditions are well admitted (first concerned are neurologic etiologies) and concern 14,1% of catatonias, psychiatric comorbidity remains a problem. The documented decline in the proportion of patients with schizophrenia diagnosed as catatonic is congruent with the fact that most studies highlight the strong association between catatonia and mood disorders. However, customary clinical practice continues to over value diagnostic of schizophrenia because catatonic symptoms are recognized as schizophrenic and schizophrenia corresponds to a pharmacological target. Other authors stress that on average 20 to 40% of catatonias are idiopathic. Conceptual proximity between catatonic symptomatology and extrapyramidal syndrome could give some ways for neurobiological grasp of the trouble; mesolimbic and mesostriatal dopaminergic imbalance in a frontal lobe-basal ganglia-brainstem system is supposed to be involved. Treatment procedure could be standardized as follows: 1) Withhold neuroleptic medication. Those drugs are proven to be lethal when catatonic symptoms are developed; 2) Investigations to exclude treatable physical disorders (including standard blood laboratory tests, urinary drug screening, electroencephalogram and brain computerized tomography); 3) Trial of lorazepam. This therapeutic is safe and 80% effective. We propose to administer an initial oral 2,5 mg challenge; catatonic signs are rated after the first hours. If necessary, the patient could receive 3 mg/day with a 6-day full dose treatment and then, treatment would progressively be reduced; 4) If the patient failed to respond to lorazepam, ECT are needed; 5) Earlier use of ECT is recommended if autonomic instability or hyperthermia appears and malignant catatonia is suspected. In conclusion, catatonia has always had an unstable and blurred place in the psychiatric nosography since its first description. It has been incorporated within the group of schizophrenias and underdiagnosed for a long time, but has been predominantly associated with mood disorders for the last ten years. Psychopathological considerations, particularly on cognitive and affective status of catatonic patients, should clarify the nosologic discussion.
紧张症最早于1874年由卡尔鲍姆描述为一种兼具运动特征和情绪变化的周期性疾病。由于大多数病例最终发展为痴呆,克雷佩林将紧张症视为早发性痴呆的一种形式,而布鲁勒则将其纳入他所定义的广泛的精神分裂症范畴。这种观点影响了精神科临床实践长达70多年。但近年来,由于更精确诊断标准的定义、与情绪障碍显著关联的发现以及对有效治疗方法的重视,紧张症得到了重新审视。佩拉尔塔等人根据经验开发了一种高效的诊断工具,该工具包含11个在紧张症特征中最具鉴别性的体征。诊断阈值为三个或更多体征,其敏感性为100%,特异性为99%。这些体征包括:不动/木僵(极度被动、明显运动减少);缄默(包括听不见的低语);违拗(抗拒指令,行为与要求相反);对立症,也称为gegenhalten(抗拒被动运动,且随着施加的力量增加而加剧);姿势异常(患者自发采取奇特姿势);蜡样屈曲(检查时患者被动保持肢体位置;蜡样柔韧性);自动服从(对指令动作过度配合);模仿现象(模仿检查者的动作、表情和言语,并伴有改变和夸张);强直(肌张力增加);言语重复(持续且无方向地重复单个单词或短语);退缩/拒食或拒饮(背向检查者,无眼神接触,提供食物或饮料时拒绝食用或饮用)。使用该诊断工具,紧张症综合征在精神科住院患者中的患病率似乎接近8%。其他体征也很常见,但特异性较低:凝视、徘徊、重复动作、刻板动作、怪癖、多动/兴奋、冲动、好斗。一些作者通过增加一个被认为具有特异性的情感维度来完善这一描述。临床形式根据病程演变进行区分:存在急性、慢性和周期性形式;根据症状学区分:兴奋型紧张症的预后优于迟缓型紧张症。恶性紧张症因其严重性和高死亡率(25%)而受到最多研究;紧张症患者会出现自主神经功能紊乱,如血压不稳定、体温过高、出汗等。恶性紧张症需要在紧急情况下进行电休克治疗(ECT)干预。虽然因一般躯体疾病导致的紧张症已被广泛认可(首先涉及神经系统病因),且占紧张症患者的14.1%,但精神科共病仍然是一个问题。记录显示,被诊断为紧张型精神分裂症的患者比例下降,这与大多数研究强调紧张症与情绪障碍之间的强关联这一事实相符。然而,传统临床实践仍然过度重视精神分裂症的诊断,因为紧张症症状被视为精神分裂症的症状,且精神分裂症对应着一个药理学靶点。其他作者强调,平均20%至40%的紧张症是特发性的。紧张症症状学与锥体外系综合征在概念上的接近可能为从神经生物学角度理解这种疾病提供一些思路;额叶 - 基底神经节 - 脑干系统中的中脑边缘和中脑纹状体多巴胺能失衡被认为与之有关。治疗程序可标准化如下:1)停用抗精神病药物。当出现紧张症症状时,这些药物已被证明是致命的;2)进行检查以排除可治疗的躯体疾病(包括标准血液实验室检查、尿液药物筛查、脑电图和脑部计算机断层扫描);3)试用劳拉西泮。这种治疗方法安全且有效率达80%。我们建议初始口服给予2.5毫克进行试验;在最初几个小时后对紧张症体征进行评分。如有必要,患者可接受3毫克/天的剂量,进行为期6天的全剂量治疗,然后逐渐减少治疗剂量;4)如果患者对劳拉西泮无反应,则需要进行电休克治疗;5)如果出现自主神经不稳定或体温过高并怀疑为恶性紧张症,建议尽早使用电休克治疗。总之,自首次被描述以来,紧张症在精神疾病分类学中的地位一直不稳定且模糊不清。它曾被纳入精神分裂症范畴且长期未得到充分诊断,但在过去十年中主要与情绪障碍相关。精神病理学方面的考量,特别是关于紧张症患者的认知和情感状态,应有助于澄清疾病分类学的讨论。