Kumar Anil, Ighodaro Eseosa T., Sharma Sandeep
Great Plains Health
Emory University Hospital
The 2017 classification by the International League Against Epilepsy has categorized seizures based on 3 key features: the location of seizure onset, level of awareness during a seizure, and other features of seizures. Focal impaired awareness seizures refer to focal seizures that start in one hemisphere of the brain and are associated with impairment in consciousness. Formerly known as "complex partial seizures," they are now called "focal impaired awareness seizure" or "focal onset impaired awareness seizure" and are typically referred to as a "focal onset seizure." Focal onset seizures refer to epileptiform activity starting in one area on one side of the brain. If awareness is impaired or affected at any time during the seizure, it is called a focal impaired awareness seizure. Focal seizures are further classified into: : Motor onset (automatisms, atonic, clonic, myoclonic, tonic, epileptic spasms, hyperkinetic) . Nonmotor onset (autonomic, emotional, sensory, cognitive, behavior arrest) . A seizure that starts on one side or one part of the brain and then spreads to both sides, previously referred to as secondarily generalized seizures, is now preferably termed a "focal to bilateral seizure." Focal onset impaired awareness seizures can present with or without an aura. Auras can last from a few seconds to 1 to 2 minutes before consciousness is impaired. Consciousness is maximally impaired in the beginning. Most of the seizures with automatisms will last longer than 30 seconds, up to 1 to 2 minutes, and sometimes can be as long as 10 minutes. Absence seizures can occasionally present with the same symptomatology; however, ictal electroencephalogram (EEG) will show generalized 3-Hz spike-wave complexes. The characteristic feature of the focal impaired awareness seizure is impaired awareness, referring to decreased overall arousal and responsiveness. Symptoms of focal onset seizures with impaired awareness depend on the area of the brain it is arising from. Simple responses like visual tracking may be preserved in focal impaired awareness seizures; however, higher-order processing tasks like verbal response decision-making are profoundly impaired. Most focal impaired awareness seizures originate in the temporal lobe, although extratemporal origin has been reported in at least 10% to 30% of patients. Temporal lobe seizures are the most common type of focal impaired awareness seizures. Stereotyped automatisms occur in about 40% to 80% of patients with temporal lobe epilepsies. Seizures with predominantly oral and manual automatism and some other motor manifestations suggest temporal lobe origin. About 60% of temporal lobe seizures spread to involve both cerebral hemispheres. Gradual recovery after several minutes of confusion occurs postictally in most patients; however, in some patients, automatic behavior like running or walking about and non-directed violent behavior may occur. Temporal lobe focal impaired seizures can have features similar to frontal seizures, but temporal lobe focal impaired seizures typically have slower onset and progression and with more pronounced confusion. Certain features can help in localizing the seizure onset to 1 hemisphere. Ictal vomiting, ictal speech, urinary urge, and automatisms with intact consciousness suggest seizure onset in the non-dominant hemisphere, and speech disturbance postictally is suggestive of seizure onset in the dominant hemisphere. Upper limb dystonia lateralizes seizure to the opposite hemisphere. In young children with focal seizures of temporal lobe onset, behavioral arrest and unresponsiveness are common. Oroalimentary automatisms tend to occur in children older than 5. The symmetric motor movement of the limbs and head nodding is typical in younger children. In infants, these seizures may be subtle with few automatisms. In very young infants, central apnea can occur. Temporal focal impaired seizures can be confused with absence seizures as both may have automatisms, but temporal seizures are usually more prolonged and are associated with postictal confusion. Seizures arising from the medial temporal lobe are characterized by auras such as epigastric sensation, deja vu, a feeling of fear, and unpleasant smells. Autonomic features like tachycardia, flushing, and pallor are common. Auras may be followed by impaired awareness and oroalimentary automatisms. Automatisms in the upper limb with or without unilateral pupillary dilatation may lateralize the seizure to the ipsilateral hemisphere. Dystonia in the upper limbs, head, and eye version on the opposite side can occur. Lateral temporal seizures may have vertigo, auditory (buzzing, ringing), or visual symptoms as initial aura symptoms. An auditory aura in only one ear may lateralize a seizure to the contralateral hemisphere. Initial aura is usually not prolonged, and impaired awareness is an early feature. Seizures are of shorter duration, and progression to bilateral convulsions is more common than those arising from the medial temporal lobe. Up to 30% of the patients with focal epilepsy have seizures arising from the frontal lobe; therefore, this is the most common extratemporal type. Seizures are accompanied by loss of consciousness in about half of the patients with frontal lobe epilepsy. Focal impaired awareness seizures can arise from various locations within the frontal lobe, except the rolandic strip. These seizures typically are brief, lasting about 30 seconds, occurring in clusters multiple times a day, are often nocturnally occurring during sleep, and have minimal postictal confusion. Motor symptoms are predominant and range from hypermotor thrashing episodes like pelvic thrusting and bicycling movements to asymmetric tonic posturing. Sexual automatisms, bizarre behavior, and vocalizations are common. These seizures often have a stereotypical pattern for each patient. Nocturnal frontal lobe seizures may be mistaken for parasomnias. The ictal EEG may be difficult to interpret because of movement artifacts. Identification based on semiology alone and differentiating from mesial temporal lobe epilepsy may be difficult; however, earliest signs and symptoms and their order of appearance may help in distinction. Seizures with hypermotor features are more likely to have an ictal focus in the orbitofrontal and frontopolar regions. Temporal lobe seizures have more oroalimentary automatisms, gesturing, and fumbling semiology. Epileptiform activity in frontal convexity can cause clonic seizures, and can cause tonic seizures in the supplementary motor area. Unique semiology of the supplementary sensorimotor cortex includes deviation of head and eye to the side contralateral to seizure onset, the asymmetrical posturing of upper limbs with an extension of arm contralateral to the side of seizure onset, and flexion of the ipsilateral arm. Orbitofrontal region seizures are auto-motor type and manifest prominently with autonomic phenomena like flushing, vocalization, and automatisms. Anterior cingulate gyrus seizures have predominant motor manifestations like hypermotor seizures and complex motor seizures. Posterior cingulate cortex epilepsies predominantly have altered consciousness and automotor seizures as the main clinical manifestations. Antero-lateral dorsal convexity seizures may manifest with auras such as dizziness, epigastric sensation, behavioral arrest, and speech arrest. Seizures from the parietal lobe may be difficult to diagnose because of their subjective nature. Sensorimotor phenomenon and vestibular hallucinations suggest onset in the parietal lobe. Positive and/or negative sensory features are common. Paresthesias, visual hallucinations, visual illusions, somatic illusions, and vertiginous features can occur. Seizures arising from the dominant hemisphere can cause receptive language impairment. Parietal lobe focal impaired awareness seizures can have auras like epigastric sensations, visual hallucinations, panic attacks, and behavioral arrest. Often, there is an involvement of other lobes as the seizure spreads. When focal seizures from the parietal lobe spread and involve the temporal lobe, loss of consciousness and automatisms may occur. A visual aura characterizes seizures with ictal origin in the occipital lobe but is difficult to diagnose, especially in young children. Visual auras, typically of elementary sensations, ictal blindness, versions of the head and eyes to the opposite side, rapid and forced blinking, and oculoclonic activity are some features suggesting the occipital lobe as an origin of focal seizure with impaired consciousness. Seizures from the primary visual cortex can cause bilateral vision loss in the form of white-out or black-out. A shorter duration of the visual aura (less than 2 minutes) can help to differentiate from a migraine aura, which is typically longer (5-15 minutes). Complex-formed visual hallucinations, like pictures of people, animals, etc., are associated with seizure onset in the extra-striate cortex. Other symptoms may result from the spread of the seizure to the temporal or parietal lobes. Seizures arising from the insula can mimic frontal, temporal, and parietal lobe seizures. Origin from the insula is suspected when viscerosensitive symptoms (nausea, vomiting, salivation), motor symptoms (tonic, hypermotor, or generalized tonic-clonic movements), and/or sensory symptoms (numbness, tightness, vibration, pain, vertigo) occur at seizure onset.
国际抗癫痫联盟2017年的分类根据3个关键特征对癫痫发作进行了分类:发作起始部位、发作期间的意识水平以及癫痫发作的其他特征。局灶性意识障碍性发作是指起始于大脑一侧半球并伴有意识障碍的局灶性发作。以前称为“复杂部分性发作”,现在称为“局灶性意识障碍性发作”或“局灶性起始意识障碍性发作”,通常简称为“局灶性起始发作”。局灶性起始发作是指起源于大脑一侧某一区域的癫痫样活动。如果在发作期间的任何时候意识受到损害或影响,则称为局灶性意识障碍性发作。局灶性发作进一步分为:运动性起始(自动症、失张力、阵挛、肌阵挛、强直、癫痫性痉挛、运动过多)。非运动性起始(自主神经、情感、感觉、认知、行为停止)。起始于大脑一侧或一部分然后扩散至双侧的发作,以前称为继发性全身性发作,现在最好称为“局灶性至双侧性发作”。局灶性起始意识障碍性发作可伴有或不伴有先兆。先兆可在意识受损前持续数秒至1至2分钟。意识在开始时受损最严重。大多数伴有自动症的发作持续时间会超过30秒,长达1至2分钟,有时可达10分钟。失神发作偶尔也会有相同的症状表现;然而,发作期脑电图(EEG)会显示全身性3赫兹棘慢复合波。局灶性意识障碍性发作的特征性表现是意识障碍,即整体觉醒和反应性降低。伴有意识障碍的局灶性起始发作的症状取决于发作起源的脑区。在局灶性意识障碍性发作中,像视觉追踪这样的简单反应可能会保留;然而,像言语反应决策这样的高级处理任务会受到严重损害。大多数局灶性意识障碍性发作起源于颞叶,尽管至少10%至30%的患者发作起源于颞叶以外的部位。颞叶发作是最常见的局灶性意识障碍性发作类型。约40%至80%的颞叶癫痫患者会出现刻板的自动症。以口部和手部自动症及其他一些运动表现为主的发作提示颞叶起源。约60%的颞叶发作会扩散至双侧大脑半球。大多数患者发作后会在数分钟的意识模糊后逐渐恢复;然而,在一些患者中,可能会出现如奔跑或四处走动的自动行为以及无目的的暴力行为。颞叶局灶性发作可能具有与额叶发作相似的特征,但颞叶局灶性发作通常起病和进展较慢,意识模糊更明显。某些特征有助于将发作起始定位至一侧半球。发作期呕吐、发作期言语、尿急和意识清醒时的自动症提示发作起始于非优势半球,发作后言语障碍提示发作起始于优势半球。上肢肌张力障碍提示发作定位于对侧半球。在颞叶起始局灶性发作的幼儿中,行为停止和无反应很常见。口消化道自动症多见于5岁以上儿童。幼儿典型表现为肢体对称运动和点头。在婴儿中,这些发作可能很轻微,自动症较少。在非常小的婴儿中,可能会出现中枢性呼吸暂停。颞叶局灶性发作可能会与失神发作混淆,因为两者可能都有自动症,但颞叶发作通常持续时间更长,且伴有发作后意识模糊。起源于颞叶内侧的发作以如胃部感觉、似曾相识感、恐惧感和难闻气味等先兆为特征。心动过速、脸红和苍白等自主神经特征很常见。先兆之后可能会出现意识障碍和口消化道自动症。上肢自动症伴或不伴单侧瞳孔散大可能提示发作定位于同侧半球。对侧上肢、头部和眼球偏斜可能出现肌张力障碍。外侧颞叶发作可能以眩晕、听觉(嗡嗡声、铃声)或视觉症状作为初始先兆症状。仅一侧耳朵出现听觉先兆可能提示发作定位于对侧半球。初始先兆通常持续时间不长,意识障碍是早期特征。发作持续时间较短,进展为双侧抽搐比起源于颞叶内侧的发作更常见。高达30%的局灶性癫痫患者发作起源于额叶;因此,这是最常见的颞叶外类型。约一半的额叶癫痫患者发作时伴有意识丧失。除中央沟周围皮质外,局灶性意识障碍性发作可起源于额叶内的各种部位。这些发作通常很短暂,持续约30秒,一天内成簇发作多次,常在睡眠期间夜间发作,发作后意识模糊很轻微。运动症状为主,范围从如骨盆前冲和骑自行车运动等运动过多的猛烈发作到不对称强直姿势。性自动症、怪异行为和发声很常见。这些发作对每个患者通常都有刻板模式。夜间额叶发作可能被误诊为睡眠障碍。由于运动伪迹,发作期脑电图可能难以解释。仅根据发作症状学进行鉴别并与内侧颞叶癫痫区分可能很困难;然而,最早的症状及其出现顺序可能有助于鉴别。具有运动过多特征的发作更可能在眶额和额极区域有发作期病灶。颞叶发作有更多的口消化道自动症、手势和摸索样发作症状。额叶凸面的癫痫样活动可引起阵挛发作,在辅助运动区可引起强直发作。辅助感觉运动皮质的独特发作症状包括头和眼向发作起始对侧偏斜、上肢不对称姿势,发作起始侧对侧上肢伸展,同侧上肢屈曲。眶额区域发作是自动运动型,突出表现为脸红、发声和自动症等自主神经现象。前扣带回发作主要有运动过多发作和复杂运动发作等运动表现。后扣带回皮质癫痫主要以意识改变和自动运动发作作为主要临床表现。前外侧背凸发作可能以头晕、胃部感觉、行为停止和言语停止等先兆为表现。顶叶发作可能因其主观性而难以诊断。感觉运动现象和前庭幻觉提示发作起源于顶叶。阳性和/或阴性感觉特征很常见。可出现感觉异常、视幻觉、视错觉、躯体错觉和眩晕特征。起源于优势半球的发作可导致感受性语言障碍。顶叶局灶性意识障碍性发作可伴有如胃部感觉、视幻觉、惊恐发作和行为停止等先兆。随着发作扩散,通常会累及其他脑叶。当顶叶局灶性发作扩散并累及颞叶时,可能会出现意识丧失和自动症。枕叶发作以视觉先兆为特征,但难以诊断,尤其是在幼儿中。视觉先兆,通常是基本感觉、发作期失明、头和眼向对侧偏斜、快速和强迫性眨眼以及眼球阵挛活动等一些特征提示枕叶是伴有意识障碍的局灶性发作的起源部位。初级视觉皮质发作可导致双侧视力丧失,表现为白茫茫或黑蒙蒙。视觉先兆持续时间较短(少于2分钟)有助于与偏头痛先兆区分,偏头痛先兆通常持续时间更长(5 - 15分钟)。复杂形式的视幻觉,如人物、动物等图片,与纹状体外皮质发作起始有关。其他症状可能是发作扩散至颞叶或顶叶所致。岛叶发作可类似额叶、颞叶和顶叶发作。当发作起始出现内脏敏感症状(恶心、呕吐、流涎)、运动症状(强直、运动过多或全身性强直阵挛运动)和/或感觉症状(麻木、紧绷、振动、疼痛、眩晕)时,则怀疑发作起源于岛叶。