Department of Pediatric Neurology, Hopital Robert Debré, APHP, Paris, France.
Epilepsia. 2010 Jul;51(7):1290-6. doi: 10.1111/j.1528-1167.2010.02534.x. Epub 2010 Mar 18.
Late-onset spasms (LOS) are epileptic spasms starting after the first year of life. Our aim was to assess the electroclinical features and the follow-up of the patients with this particular type of epileptic seizure.
We retrospectively included all patients with LOS confirmed by electroencephalography between 1989 and 2008. Clinical and electroencephalographic findings at diagnosis and during follow-up were collected. The Vineland scale was used to evaluate the neuropsychological outcome.
We report 19 patients with LOS of 240 patients with recorded epileptic spasms. Eighteen patients had an epileptic encephalopathy with late-onset spasms. The ictal electroencephalography (EEG) showed a focal or generalized discharge of triphasic slow-waves, slow-spikes, or slow spikes-waves with fast activities. The interictal EEG usually showed focal or generalized slow-waves or slow spikes-waves without hypsarhythmia. LOS were controlled in only six patients. Three developed typical Lennox-Gastaut syndrome and 10 had a severe epileptic encephalopathy. Neuropsychological outcome was evaluated in 15 patients with the Vineland scale. Cognitive functions were normal in only one patient, whereas severe cognitive delay was observed in 12 of 15.
Epileptic spasms may appear after the age of one. They are more frequently observed in patients with epileptic encephalopathy. In few patients this type of seizure was observed before the patients fulfill Lennox-Gastaut syndrome criteria. In one patient, we diagnosed a focal epilepsy with seizures occurring in cluster. When LOS are related to an epileptic encephalopathy, this epileptic syndrome seems to be linked to refractory epilepsy and severe cognitive outcome unrelated to the etiology.
迟发性痉挛（LOS）是指在生命的第一年之后开始的癫痫痉挛。我们的目的是评估具有这种特殊类型癫痫发作的患者的电临床特征和随访结果。
我们回顾性地纳入了 1989 年至 2008 年间经脑电图证实为 LOS 的所有患者。收集了诊断时和随访期间的临床和脑电图发现。使用 Vineland 量表评估神经心理学结局。
我们报告了 240 例记录到癫痫痉挛的患者中 19 例 LOS 患者。18 例患者存在晚发性痉挛的癫痫性脑病。发作期脑电图显示三相慢波、慢棘波或慢棘慢波伴有快活动的局灶性或全身性放电。发作间期脑电图通常显示局灶性或全身性慢波或慢棘慢波，无高波幅失律。仅 6 例患者 LOS 得到控制。3 例患者发展为典型 Lennox-Gastaut 综合征，10 例患者存在严重癫痫性脑病。我们使用 Vineland 量表评估了 15 例患者的神经心理学结局。仅 1 例患者认知功能正常，而 15 例中有 12 例存在严重认知延迟。
癫痫痉挛可能在 1 岁后出现。它们更常发生在患有癫痫性脑病的患者中。在少数患者中，这种类型的发作在患者符合 Lennox-Gastaut 综合征标准之前已经观察到。在 1 例患者中，我们诊断为局灶性癫痫，发作呈簇状发生。当 LOS 与癫痫性脑病有关时，这种癫痫综合征似乎与难治性癫痫和与病因无关的严重认知结局相关。
