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伴有中央颞区棘波的儿童良性局灶性癫痫(BPERS)中癫痫源性言语和口面部运动功能障碍。与获得性失语-癫痫综合征的关系。

Speech and oromotor deficits of epileptic origin in benign partial epilepsy of childhood with rolandic spikes (BPERS). Relationship to the acquired aphasia-epilepsy syndrome.

作者信息

Deonna T W, Roulet E, Fontan D, Marcoz J P

机构信息

Centre Hospitalier Universitaire Vaudois, Pediatric Department, Lausanne, Switzerland.

出版信息

Neuropediatrics. 1993 Apr;24(2):83-7. doi: 10.1055/s-2008-1071519.

DOI:10.1055/s-2008-1071519
PMID:7687041
Abstract

The authors report three children who suffered temporary oromotor or speech disturbances as focal epileptic manifestations within the frame of benign partial epilepsy of childhood with rolandic spikes and review similar cases described in the literature. The deficit can occur as an initial symptom of the disorder without visible epileptic seizures and interferes in a variable way with simple voluntary oromotor functions or complex movements including speech production, depending on the exact location and spread of the discharging epileptic focus around the perisylvian region. The most severe deficit produces the anterior operculum syndrome. More subtle non-linguistic deficits such as intermittent drooling, oromotor apraxia or dysfluency, as well as linguistic ones involving phonologic production, can occur. The rapidity of onset, progression and recovery of the deficit is very variable as well as its duration and presumably reflects the degree of epileptic activity. In some cases, rapid improvement with antiepileptic medication occurs and coincidence between the paroxysmal EEG activity (which is usually bilateral) and the functional deficit is seen. The clinical and EEG profile of the seizures disorder and the dynamic of the deficit in these cases bear a strong resemblance to what is seen in the acquired epilepsy-aphasia syndrome (Landau and Kleffner). The variations in clinical symptoms appear more related to the main site, local extension and bilaterality of the epileptic foci rather than a basic difference in physiopathology.

摘要

作者报告了三名儿童,他们在伴有中央颞区棘波的儿童良性部分性癫痫框架内,出现了暂时的口面部运动或言语障碍作为局灶性癫痫表现,并回顾了文献中描述的类似病例。这种功能缺陷可作为该疾病的初始症状出现,而无明显癫痫发作,并根据放电性癫痫病灶在外侧裂周围区域的确切位置和扩散情况,以不同方式干扰简单的随意口面部运动功能或包括言语产生在内的复杂运动。最严重的功能缺陷会导致前岛盖综合征。可能会出现更细微的非语言性缺陷,如间歇性流口水、口面部失用症或言语不流畅,以及涉及语音产生的语言性缺陷。功能缺陷的起病、进展和恢复速度以及持续时间差异很大,大概反映了癫痫活动的程度。在某些情况下,抗癫痫药物治疗后会迅速改善,且可观察到阵发性脑电图活动(通常为双侧性)与功能缺陷之间的一致性。这些病例中癫痫发作疾病的临床和脑电图特征以及功能缺陷的动态变化与获得性癫痫性失语综合征(Landau和Kleffner综合征)所见情况极为相似。临床症状的差异似乎更多地与癫痫病灶的主要部位、局部扩展和双侧性有关,而非生理病理学上的根本差异。

相似文献

1
Speech and oromotor deficits of epileptic origin in benign partial epilepsy of childhood with rolandic spikes (BPERS). Relationship to the acquired aphasia-epilepsy syndrome.伴有中央颞区棘波的儿童良性局灶性癫痫(BPERS)中癫痫源性言语和口面部运动功能障碍。与获得性失语-癫痫综合征的关系。
Neuropediatrics. 1993 Apr;24(2):83-7. doi: 10.1055/s-2008-1071519.
2
Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and regressive autistic disorders with epileptic EEG abnormalities: the continuing debate.早发性获得性癫痫性失语( Landau-Kleffner综合征,LKS)以及伴有癫痫性脑电图异常的退行性自闭症谱系障碍:持续的争论
Brain Dev. 2010 Oct;32(9):746-52. doi: 10.1016/j.braindev.2010.06.011. Epub 2010 Jul 15.
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Benign childhood focal epilepsies: assessment of established and newly recognized syndromes.儿童良性局灶性癫痫:已确立和新认识综合征的评估
Brain. 2008 Sep;131(Pt 9):2264-86. doi: 10.1093/brain/awn162. Epub 2008 Aug 21.
4
Acquired aphasia in childhood with seizure disorder: a heterogeneous syndrome.儿童期获得性失语伴癫痫发作障碍:一种异质性综合征。
Neuropadiatrie. 1977 Aug;8(3):263-73. doi: 10.1055/s-0028-1091522.
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[Epileptic syndromes which show continuous spike and wake complexes during slow wave sleep].[在慢波睡眠期间表现为持续性棘慢复合波的癫痫综合征]
Rev Neurol. 1997 Jul;25(143):1045-51.
6
Acquired aphasia of childhood with epilepsy: the Landau-Kleffner syndrome.
Clin Exp Neurol. 1987;24:187-94.
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Autosomal dominant rolandic epilepsy and speech dyspraxia: a new syndrome with anticipation.常染色体显性遗传性罗兰多癫痫伴言语失用症:一种具有遗传早现现象的新综合征。
Ann Neurol. 1995 Oct;38(4):633-42. doi: 10.1002/ana.410380412.
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Atypical benign partial epilepsy/pseudo-Lennox syndrome.非典型良性部分性癫痫/假性 Lennox 综合征
Epileptic Disord. 2000;2 Suppl 1:S11-7.
9
[The Landau-Kleffner syndrome. Infantile "acquired" aphasia, paroxysmal electroencephalographic changes and epileptic seizures].[ Landau-Kleffner综合征。婴儿期“获得性”失语、阵发性脑电图改变和癫痫发作]
Nouv Presse Med. 1982 Dec 18;11(51):3787-91.
10
[Assessment of EEG in children with partial epilepsy].[部分性癫痫患儿的脑电图评估]
Przegl Lek. 2006;63(11):1181-90.

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The role of sleep-related cognitive functions in the spectrum of benign epilepsy with centro-temporal spikes.睡眠相关认知功能在中央颞区棘波良性癫痫谱中的作用。
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New genes for focal epilepsies with speech and language disorders.伴有言语和语言障碍的局灶性癫痫的新基因。
Curr Neurol Neurosci Rep. 2015 Jun;15(6):35. doi: 10.1007/s11910-015-0554-0.
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Antiepileptic drug treatment of rolandic epilepsy and Panayiotopoulos syndrome: clinical practice survey and clinical trial feasibility.罗兰多癫痫和帕纳约托普洛斯综合征的抗癫痫药物治疗:临床实践调查和临床试验可行性。
Arch Dis Child. 2015 Jan;100(1):62-7. doi: 10.1136/archdischild-2013-304211. Epub 2014 Sep 8.
5
GRIN2A mutations cause epilepsy-aphasia spectrum disorders.GRIN2A 突变导致癫痫-失语综合征。
Nat Genet. 2013 Sep;45(9):1073-6. doi: 10.1038/ng.2727. Epub 2013 Aug 11.
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Molecular networks implicated in speech-related disorders: FOXP2 regulates the SRPX2/uPAR complex.与言语障碍相关的分子网络:FOXP2 调控 SRPX2/uPAR 复合物。
Hum Mol Genet. 2010 Dec 15;19(24):4848-60. doi: 10.1093/hmg/ddq415. Epub 2010 Sep 21.
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Staggered spondaic word test in epileptic patients.癫痫患者的交错扬扬格词测试
Sao Paulo Med J. 2002 Nov 1;120(6):185-8. doi: 10.1590/s1516-31802002000600006. Epub 2003 Jan 22.