Ristić Aleksandar J, Petrović Igor, Vojvodić Nikola, Janković Slavko, Sokić Dragoslav
Institut za neurologiju Klinicki centar Srbije Dr Subotića 6, 11000 Beograd.
Srp Arh Celok Lek. 2004 Jan-Feb;132(1-2):22-7. doi: 10.2298/sarh0402022r.
Psychogenic nonepileptic seizure (PNES) is a sudden change in a person's behavior, perception, thinking, or feeling that is usually time limited and resembles, or is mistaken for, epilepsy but does not have the characteristic electroencephalographic (EEG) changes that accompanies a true epileptic seizure [1]. It is considered that PNES is a somatic manifestation of mental distress, in response to a psychological conflict or other stressors [2]. A wide spectrum of clinical presentation includes syncope, generalized tonic-clonic seizure, simple and complex partial seizure, myoclonic seizure, frontal lobe seizures and status epilepticus [3]. Coexistence of epilepsy and PNES is seen in approximately 9% of cases [5]. Between 25-30% of patients referred to tertiary centers and initially diagnosed as refractory epilepsy were on further examination diagnosed as PNES [6, 7]. In DSM-IV [12] PNES are usually categorized under conversion disorder with seizures or convulsions. However, psychiatric basis of PNES may be anxiousness (panic attack), somatization or factitious disorder, simulation, dissociative disorders and psychosis [1].
The aim of the study was to establish clinical phenomenology and EEG characteristics as well as basic psychiatric disorder in patients with PNES.
In a retrospective study covering the period from January 1st 1999 till April 31st 2003, 24 patients (22 female, 2 male) treated at the institute of Neurology in Belgrade were analyzed. PNES were defined as sudden change in behavior incoherent with epileptiform activity registered on EEG. Possible PNES were determined on the basis of history data and clinical examination during the attack but definitive confirmation was established only by the finding of no ictal EEG changes during typical seizure of each patient. Patients with coexisting epilepsy were included in the study, too. At least two standard EEG (range 2-6, median 4) were performed at the beginning of diagnostic evaluation. Demographic data, clinical presentation (apparent loss of consciousness, type of convulsion and associated clinical signs) and placebo-induced seizures (administration of saline near the cubital vein) with EEG or video-EEG monitoring were analyzed. Basic psychiatric disorder was classified according to DSM IV classification criteria.
Duration of PNES was 4.7 years (range from 2 months to 30 years). The time from onset to the diagnosis of PNES was 4.5 years. Epilepsy comorbidity was diagnosed in 9 patients (37.5%). The average time of use of antiepileptic drugs (AED) in the group of isolated PNES was 2.4 years and 20% of patients were treated with two or more AED. The vast majority of patients presented with bilateral convulsions (54.16%) with apparent loss of consciousness found in 91.6% of cases. Ictal iwury (16.7%), tongue bite (4.2%) and premonition of the seizure (17.4%) were uncommon. Variability in clinical presentation of seizures was found in over half of patients (57%). Psychological trigger could be determined in over 60% of patients. EEG findings in a group with isolated PNES suggesting the existence of epileptiform activity was found in one case. EEG monitoring of placebo-induced seizure was performed in 20 patients, of whom 19 (95%) showed typical habitual attack with no electroclinical correlate. In 70% of cases conversion disorder DSM-IV criteria were fulfilled. Somatization disorder and undifferentiated somatoform disorder were found in 3 patients. The diagnosis of factitious disorder was made in one case and only two patients were undiagnosed according to DSM-IV.
Average delay from onset to diagnosis of PNES in larger studies was estimated to be approximately 7 years [8]. Even though diagnostic delay in our study was shorter, organizational reasons for this could not be found. Longer duration of a typical attack (compared to the epileptic seizure), apparent loss of consciousness, bilateral convulsion behavior and significant clinical variability in absence of typical epileptic elements such as tongue bite and ictal iwury could be the main clinical manifestation of PNES. We found rare interictal abnormalities (6.7%) in the group with isolated PNES and significant percentage (77.7%) in patients with coexisting epilepsy which is coherent with other reports [8]. The latest could lead to prolonged delay in appropriate diagnosis and suitable treatment. Clear psychological trigger wasn't noted in whole group of patients (61%). This, however, is not unusual since PNES represents a chronic disorder with repeated triggering that could lead to less significant role of the same psychological trigger in developed PNES. Even insufficiently resolved in ethical terms, placebo-induced procedure was of huge sensitivity. In clinical practice conversion disorder is hard to differ from malingering or implementation of secondary gain. One could make the conclusion only on the basis of detailed and careful estimation of the symptoms developing context. Conversion disorder is more prevalent among women (from 2:1 to 10:1) [4, 13] but modest percentage of affected men could be explained only by limited sample in this study.
PNES is often replaced with epilepsy and in number of cases clinical differentiation is not easy. One should be acquainted with clinical presentation of PNES as well as its psychiatric origin in order to adequately recognize and treat the disorder.
心理性非癫痫发作(PNES)是指人的行为、感知、思维或感觉突然发生变化,通常持续时间有限,类似于癫痫发作或被误诊为癫痫发作,但不具有真正癫痫发作所伴随的特征性脑电图(EEG)变化[1]。一般认为,PNES是心理困扰的躯体表现,是对心理冲突或其他应激源的反应[2]。其临床表现形式多样,包括晕厥、全身强直阵挛发作、简单和复杂部分性发作、肌阵挛发作、额叶发作和癫痫持续状态[3]。癫痫和PNES共存的情况在约9%的病例中可见[5]。在转诊至三级中心且最初被诊断为难治性癫痫的患者中,有25% - 30%经进一步检查后被诊断为PNES[6, 7]。在《精神疾病诊断与统计手册》第四版(DSM-IV)中,PNES通常归类于伴有癫痫发作或惊厥的转换障碍。然而,PNES的精神基础可能是焦虑(惊恐发作)、躯体化或做作性障碍、伪装、分离性障碍和精神病[1]。
本研究的目的是确定PNES患者的临床现象学、脑电图特征以及基本精神障碍。
在一项回顾性研究中,分析了1999年1月1日至2003年4月31日期间在贝尔格莱德神经病学研究所接受治疗的24例患者(22例女性,2例男性)。PNES被定义为行为突然改变,且脑电图上未记录到癫痫样活动。根据发作期间的病史数据和临床检查确定可能的PNES,但只有在每位患者典型发作期间未发现发作期脑电图变化时才能确定最终诊断。同时患有癫痫的患者也纳入了研究。在诊断评估开始时至少进行了两次标准脑电图检查(范围为2 - 6次,中位数为4次)。分析了人口统计学数据、临床表现(明显的意识丧失、惊厥类型及相关临床体征)以及通过脑电图或视频脑电图监测的安慰剂诱发发作(在肘静脉附近注射生理盐水)情况。基本精神障碍根据DSM-IV分类标准进行分类。
PNES的病程为4.7年(范围从2个月至30年)。从发病到诊断为PNES的时间为4.5年。9例患者(37.5%)被诊断为合并癫痫。在单纯PNES组中,抗癫痫药物(AED)的平均使用时间为2.4年,20%的患者接受了两种或更多种AED治疗。绝大多数患者表现为双侧惊厥(54.16%),91.6%的病例出现明显的意识丧失。发作期损伤(16.7%)、舌咬伤(4.2%)和发作预感(17.4%)并不常见。超过一半的患者(57%)发作临床表现存在变异性。超过60%的患者可确定心理触发因素。在单纯PNES组中,有1例脑电图结果提示存在癫痫样活动。对20例患者进行了安慰剂诱发发作的脑电图监测,其中19例(95%)表现出典型的习惯性发作,无电临床相关性。在70%的病例中符合DSM-IV转换障碍标准。3例患者被诊断为躯体化障碍和未分化躯体形式障碍。1例被诊断为做作性障碍,根据DSM-IV仅有2例患者未确诊。
在更大规模的研究中,从发病到诊断为PNES的平均延迟时间估计约为7年[8]。尽管本研究中的诊断延迟较短,但未发现组织方面的原因。典型发作持续时间较长(与癫痫发作相比)、明显的意识丧失、双侧惊厥行为以及在无典型癫痫发作要素(如舌咬伤和发作期损伤)情况下显著的临床变异性可能是PNES的主要临床表现。我们发现单纯PNES组的发作间期异常罕见(6.7%),而合并癫痫的患者中这一比例较高(77.7%),这与其他报道一致[8]。后者可能导致适当诊断和合适治疗的长期延迟。在整个患者组中未发现明确的心理触发因素(61%)。然而,这并不罕见,因为PNES是一种慢性疾病,反复触发可能导致相同心理触发因素在已发展的PNES中作用减弱。尽管在伦理方面存在不足,但安慰剂诱发程序具有很高的敏感性。在临床实践中,转换障碍很难与诈病或继发获益相区分。只有在详细、仔细评估症状发展背景的基础上才能得出结论。转换障碍在女性中更为普遍(比例从2:1至10:1)[4, 13],但本研究中受影响男性比例较低可能仅由样本有限所致。
PNES常被误诊为癫痫,在许多情况下临床鉴别并不容易。为了充分认识和治疗该疾病,应熟悉PNES的临床表现及其精神根源。