Daniel Roy Thomas, Meagher-Villemure Kathleen, Roulet Eliane, Villemure Jean-Guy
Department of Neurosurgery, Centre Hospitalier Universitaire Vauduois, Lausanne, Switzerland.
Epilepsia. 2004 Jul;45(7):872-6. doi: 10.1111/j.0013-9580.2004.65603.x.
Extensive multilobar cortical dysplasia in infants commonly is first seen with catastrophic epilepsy and poses a therapeutic challenge with respect to control of epilepsy, brain development, and psychosocial outcome. Experience with surgical treatment of these lesions is limited, often not very encouraging, and holds a higher operative risk when compared with that in older children and adults.
Two infants were evaluated for surgical control of catastrophic epilepsy present since birth, along with a significant psychomotor developmental delay. Magnetic resonance imaging showed multilobar cortical dysplasia (temporoparietooccipital) with a good electroclinical correlation. They were treated with a temporal lobectomy and posterior (parietooccipital) disconnection.
Both infants had excellent postoperative recovery and at follow-up (1.5 and 3.5 years) evaluation had total control of seizures with a definite "catch up" in their development, both motor and cognitive. No long-term complications have been detected to date.
The incorporation of disconnective techniques in the surgery for extensive multilobar cortical dysplasia in infants has made it possible to achieve excellent seizure results by maximizing the extent of surgical treatment to include the entire epileptogenic zone. These techniques decrease perioperative morbidity, and we believe would decrease the potential for the development of long-term complications associated with large brain excisions.
婴儿广泛性多叶皮质发育异常通常最初表现为灾难性癫痫，在癫痫控制、脑发育及社会心理结局方面构成治疗挑战。与大龄儿童和成人相比，对这些病变进行手术治疗的经验有限，通常不太乐观，且手术风险更高。
对两名自出生起就患有灾难性癫痫且伴有明显精神运动发育迟缓的婴儿进行手术控制评估。磁共振成像显示多叶皮质发育异常（颞顶枕叶），具有良好的电临床相关性。对他们实施了颞叶切除术和后部（顶枕叶）离断术。
两名婴儿术后恢复良好，在随访（1.5年和3.5年）评估时癫痫完全得到控制，运动和认知发育均有明显“追赶”。迄今为止未发现长期并发症。
在婴儿广泛性多叶皮质发育异常手术中采用离断技术，通过最大限度扩大手术范围以包括整个致痫区，从而有可能获得优异的癫痫治疗效果。这些技术降低了围手术期发病率，我们认为也会降低与大脑大面积切除相关的长期并发症发生的可能性。
