Huff J. Stephen, Lui Forshing, Murr Najib I.
University of Virginia
CA Northstate Uni, College of Med
Psychogenic nonepileptic seizures (PNES) are relatively common but poorly understood and often misdiagnosed as epilepsy, which can lead to unnecessary procedures and treatments along with the possibility of failure to engage patients in necessary behavioral health care. Despite a superficial resemblance to epilepsy, in PNES, the underlying cause has long been considered to be psychological. However, increasingly integrated theories of causation invoking genetics, environmental factors, temperament, and early childhood experiences are being proposed. Rarely is a nonepileptic event intentional, in which case it could be due to factitious disorder or malingering, but by definition, PNES themselves are never intentional. "Pseudoseizure" is a now-outmoded term for paroxysmal events that appear to be epileptic seizures but do not arise from the abnormal excessive synchronous cortical activity that defines an epileptic seizure. Patients and healthcare practitioners alike are prone to misinterpret "pseudoseizure" as indicating that the patient is "faking" or otherwise feigning the events when, in fact, the events are involuntary behavioral responses to underlying psychological triggers or stresses. Other terms used in the past that should now be avoided are hysterical seizures, functional seizures, stress seizures, and others. Distinguishing PNES from epileptic seizures may be difficult at the bedside, even for experienced clinicians. Indeed, some researchers have characterized PNES as occupying a no-man's land at the intersection between Neurology and Psychiatry. Diagnostic delay of years with PNES is common. Video electroencephalography (video-EEG) of a typical event showing the absence of epileptiform activity during the spell in the setting of a compatible history is the gold standard for diagnosis. Between 20% and 40% of patients referred to epilepsy monitoring units for difficult-to-control seizures are ultimately found to have PNES. A recently reported pediatric series examined 15 years of video-EEG monitoring and found that the final diagnosis was PNES in nearly 20% of monitored individuals; eventual discontinuation of antiseizure medication (ASM) on the grounds of initial misdiagnosis was necessary for nearly 25%. Correct diagnosis is imperative for the successful treatment of PNES. Still, misdiagnosis is common, especially among primary care and emergency physicians, nearly two-thirds of whom reported their belief that video-EEG is not needed for diagnostic confirmation in a recent study. A comprehensive history and examination are vital steps toward a correct diagnosis. Consultation with neurology is nearly always beneficial; admission to an epilepsy monitoring unit for video-EEG analysis is almost always required. Referral to a comprehensive epilepsy center may be helpful in challenging cases. The diagnosis of PNES needs to be conveyed to the patient effectively and empathically; doing otherwise carries a non-trivial risk of prompting confusion, anger, or resentment, any or all of which can then exacerbate PNES symptomatology. Diagnostic disclosure is particularly delicate if a given patient was previously diagnosed with epilepsy, and patients with a history of trauma or abuse can easily be re-traumatized by a clumsily rendered diagnosis. Above all, the clinician must acknowledge and underscore that help is available for the patient's symptoms, that these symptoms are real, and that symptoms represent a source of distress to the patient, family, and friends. Treatment of PNES may be complex, but it is clear that ASMs are of no benefit, and they may cause harm.ASMs should be discontinued unless they are in use to manage concomitant epilepsy, chronic pain, or mood disorders; continuation of ASMs after the PNES diagnosis has been made is associated with poor outcomes. Psychotherapy is effective and can improve seizure frequency, overall psychosocial functioning, and health-related quality of life.
心因性非癫痫性发作(PNES)相对常见,但人们对其了解甚少,且常被误诊为癫痫,这可能导致不必要的检查和治疗,同时患者也可能无法接受必要的行为健康护理。尽管PNES在表面上与癫痫相似,但长期以来人们一直认为其根本原因是心理因素。然而,越来越多融合了遗传学、环境因素、气质和童年早期经历的病因理论被提出。非癫痫性发作很少是故意的,在这种情况下可能是人为障碍或诈病,但根据定义,PNES本身绝不是故意的。“假性发作”是一个现已过时的术语,用于描述那些看似癫痫发作但并非由定义癫痫发作的异常过度同步皮层活动引起的阵发性事件。患者和医护人员都容易误解“假性发作”,认为患者是在“假装”或以其他方式伪造这些事件,而实际上这些事件是对潜在心理触发因素或压力的非自愿行为反应。过去使用的其他一些现在应避免使用的术语包括癔症性发作、功能性发作、应激性发作等。即使对于经验丰富的临床医生来说,在床边区分PNES和癫痫发作也可能很困难。事实上,一些研究人员将PNES描述为处于神经病学和精神病学交叉的无人地带。PNES的诊断延迟数年很常见。对于典型发作事件进行录像脑电图(video - EEG)检查,在有相应病史的情况下显示发作期间无癫痫样活动,这是诊断的金标准。转至癫痫监测单元以评估难以控制的癫痫发作的患者中,最终发现有PNES的比例在20%至40%之间。最近报道的一项儿科研究系列检查了15年的录像脑电图监测情况,发现近20%的受监测个体最终诊断为PNES;近25%的患者因最初误诊而最终有必要停用抗癫痫药物(ASM)。正确诊断对于PNES的成功治疗至关重要。然而,误诊仍然很常见,尤其是在初级保健医生和急诊医生中,在最近的一项研究中,近三分之二的医生表示他们认为诊断确认不需要录像脑电图。全面的病史和检查是正确诊断的关键步骤。几乎总是需要咨询神经科医生;几乎总是需要入住癫痫监测单元进行录像脑电图分析。对于疑难病例,转诊至综合癫痫中心可能会有所帮助。需要以有效且富有同理心的方式将PNES的诊断告知患者;否则很可能会引发困惑、愤怒或怨恨,其中任何一种或全部都可能加重PNES的症状。如果某个患者之前被诊断为癫痫,那么诊断披露会特别棘手,而且有创伤或虐待史的患者很容易因诊断表述不当而再次受到创伤。最重要的是,临床医生必须承认并强调患者的症状有相应的治疗方法,这些症状是真实存在的,并且这些症状给患者、家人和朋友都带来了困扰。PNES的治疗可能很复杂,但很明显ASM并无益处,而且可能会造成伤害。除非是用于治疗合并的癫痫、慢性疼痛或情绪障碍,否则应停用ASM;在诊断为PNES后继续使用ASM与不良预后相关。心理治疗是有效的,可以改善发作频率、整体心理社会功能以及与健康相关的生活质量。