Andermann F, Zifkin B
Department of Neurology, McGill University, Montreal Neurological Hospital and Institute, Quebec, Canada.
Epilepsia. 1998;39 Suppl 4:S9-23. doi: 10.1111/j.1528-1157.1998.tb05129.x.
Benign occipital epilepsy of childhood is an idiopathic partial epilepsy syndrome with elementary visual symptomatology, frequently associated with other ictal phenomena. Seizures are usually followed by postictal headache and are often associated with interictal occipital rhythmic paroxysmal EEG activity that appears only after eye closure. In some children the ictal visual symptoms or the interictal EEG abnormalities may not be demonstrated. The clinical and/or EEG manifestations of other forms of idiopathic partial or generalized epilepsy may be found in association. Occipital spikes in non-epileptic children with defective vision, occipital slow spike-and-wave found in some patients with the Lennox-Gastaut syndrome, focal epilepsy due to occipital lesions, seizures originating in the temporal lobe secondary to an occipital abnormality, and complicated or basilar migraine must be considered in the differential diagnosis. Early-onset benign occipital epilepsy or seizure susceptibility syndrome deserves to be considered separately. It has been defined by Panayiotopoulos as consisting of brief, infrequent attacks or prolonged status epilepticus and characterized by ictal deviation of the eyes and/or head and vomiting, occurring in children usually between the ages of 3 and 7 years. Advances in molecular genetics will help decide whether these two disorders are indeed distinct. Benign occipital and benign rolandic epilepsy are commonly associated with migraine. The selective involvement of the occipital lobe in migraine has not been fully explained. The association between benign occipital epilepsy and migraine is likely related to this underlying mechanism as well. The "fixation off" phenomenon or blocking of occipital epileptic discharges by eye opening is not specific to benign occipital epilepsy of childhood and may be found in symptomatic epilepsies as well. Migraine and epilepsy are distinct disorders, both as far as their pathophysiologic mechanisms and clinical symptomatology are concerned. There is however an overlap in some patients and a causal relationship may exist in some, leading to clinically distinct migraine epilepsy syndromes. Here too, clarification of the molecular basis of migraine and of epilepsy will throw light on the nature of the relationship between the two conditions.
儿童良性枕叶癫痫是一种特发性局灶性癫痫综合征，具有基本的视觉症状，常伴有其他发作期现象。发作后通常会出现发作后头痛，且常与仅在闭眼后出现的发作间期枕叶节律性阵发性脑电图活动相关。在一些儿童中，发作期视觉症状或发作间期脑电图异常可能无法显示。可能会发现其他形式的特发性局灶性或全身性癫痫的临床和/或脑电图表现与之相关。视力有缺陷的非癫痫儿童出现枕叶棘波、一些 Lennox-Gastaut 综合征患者出现枕叶慢棘慢波、枕叶病变导致的局灶性癫痫、枕叶异常继发颞叶起源的发作以及复杂性或基底型偏头痛都必须在鉴别诊断中予以考虑。早发性良性枕叶癫痫或癫痫易感性综合征值得单独考虑。Panayiotopoulos 将其定义为包括短暂、不频繁的发作或长时间的癫痫持续状态，其特征为发作期眼球和/或头部偏斜以及呕吐，通常发生在 3 至 7 岁的儿童中。分子遗传学的进展将有助于确定这两种疾病是否确实不同。良性枕叶癫痫和良性罗兰多癫痫通常与偏头痛相关。偏头痛中枕叶的选择性受累尚未得到充分解释。良性枕叶癫痫与偏头痛之间的关联可能也与此潜在机制有关。“睁眼阻断”现象或睁眼对枕叶癫痫放电的阻断并非儿童良性枕叶癫痫所特有，在症状性癫痫中也可能出现。偏头痛和癫痫是不同的疾病，就其病理生理机制和临床症状而言都是如此。然而，在一些患者中存在重叠，在一些患者中可能存在因果关系，导致临床上不同的偏头痛癫痫综合征。同样，阐明偏头痛和癫痫的分子基础将有助于揭示这两种疾病之间关系的本质。
