Fauser Susanne, Schulze-Bonhage Andreas, Honegger Juergen, Carmona Hans, Huppertz Hans-Juergen, Pantazis Georgios, Rona Sabine, Bast Thomas, Strobl Karl, Steinhoff Bernhard J, Korinthenberg Rudolf, Rating Dietz, Volk Benedikt, Zentner Josef
Epilepsy Center, University of Freiburg, Breisacher Strasse 64, 79106 Freiburg, Germany.
Brain. 2004 Nov;127(Pt 11):2406-18. doi: 10.1093/brain/awh277. Epub 2004 Aug 19.
The purpose of this study was to assess whether the histological subtype of focal cortical dysplasia and dual pathology affect surgical outcome in patients with medically intractable epilepsy due to focal cortical dysplasia (FCD). We retrospectively analysed the outcome of 67 patients from 2 to 66 years of age at follow-up periods of 6 to 48 months after epilepsy surgery. Histological subtypes were classified according to Palmini and included a few cases with mild histological abnormalities corresponding to the definition of mild malformations of cortical development. The seizure outcome was classified according to Engel and evaluated at the last follow-up visit as well as at follow-up periods of 12 and 24 months after surgery. The outcome in patients with FCD and additional hippocampal pathology (dual pathology) was analysed separately. Distribution of histological subtypes differed in temporal and extratemporal localization, with a significantly higher extratemporal prevalence of FCD type 2. There was a tendency towards better postsurgical outcome related to the last follow-up visit in patients with more subtle abnormalities classified as mild malformations of cortical development (mMCD) (63% Engel Ia), FCD type 1a (67% Engel Ia) and FCD type 1b (55% Engel Ia) compared with patients with FCD type 2a (43% Engel Ia) and FCD type 2b (Taylor type) (50% Engel Ia). Considering the outcome at follow-up periods over 12 and 24 months, complete seizure-freedom was achieved significantly more often in patients with FCD type 1 and mMCD than with FCD type 2, and seizure reduction by less than 75% (Engel IV) occurred in more patients with FCD type 2a compared with the other subgroups. This tendency was seen in the whole patient group and in the extratemporal subgroup. Patients with dual pathology almost always had temporal lobe epilepsy; the outcome in this patient group was generally favourable (66% complete seizure-freedom at the last follow-up visit). The outcome remained almost constant with longer periods of follow-up. We conclude that patients with FCD type 1 and mMCD had a better outcome compared with those with more severe forms of cortical dysplasia. A higher incidence of FCD type 1 in temporal localization did not allow the effects of histological subtype and localization to be separated. A subanalysis of extratemporal FCDs, however, revealed a similar tendency for a better outcome with FCD type 1, suggesting that the histological subtype itself seems to be at least a relevant cofactor influencing postsurgical outcome.
本研究的目的是评估局灶性皮质发育不良的组织学亚型和双重病理是否会影响因局灶性皮质发育不良（FCD）导致药物难治性癫痫患者的手术效果。我们回顾性分析了67例年龄在2至66岁之间的患者在癫痫手术后6至48个月随访期的结果。组织学亚型根据帕尔米尼进行分类，包括一些组织学轻度异常的病例，这些病例符合皮质发育轻度畸形的定义。癫痫发作结果根据恩格尔进行分类，并在最后一次随访以及术后12个月和24个月的随访期进行评估。对FCD合并额外海马病理（双重病理）患者的结果进行了单独分析。组织学亚型的分布在颞叶和颞外定位有所不同，FCD 2型在颞外的患病率显著更高。与FCD 2a型（恩格尔I级43%）和FCD 2b型（泰勒型，恩格尔I级50%）患者相比，被归类为皮质发育轻度畸形（mMCD）（恩格尔I级63%）、FCD 1a型（恩格尔I级67%）和FCD 1b型（恩格尔I级55%）的异常更轻微的患者在最后一次随访时术后效果有更好的趋势。考虑到术后12个月和24个月以上随访期的结果，FCD 1型和mMCD患者实现完全无发作的频率显著高于FCD 2型患者，与其他亚组相比，FCD 2a型患者中发作减少不到75%（恩格尔IV级）的情况更多。这种趋势在整个患者组和颞外亚组中都有体现。双重病理患者几乎总是患有颞叶癫痫；该患者组的结果总体较好（最后一次随访时66%完全无发作）。随着随访时间延长，结果几乎保持不变。我们得出结论，与皮质发育不良更严重形式的患者相比，FCD 1型和mMCD患者的效果更好。颞叶定位中FCD 1型的较高发病率使得无法区分组织学亚型和定位的影响。然而，对颞外FCD的亚分析显示，FCD 1型也有类似的更好效果趋势，这表明组织学亚型本身似乎至少是影响术后效果的一个相关辅助因素。
