METHODS: Single, colloidal, or vesicular parenchymal NCC cases were treated with albendazole and steroids and followed at a tertiary care north Indian hospital. Serum MMP-9 was estimated in colloidal or vesicular treatment-naive state and in a subset of calcified cases at six-month follow-up. The same subset of calcified cases also underwent perfusion MRI of the brain at six-month follow-up.
RESULTS: Among 70 cases, 70% calcified at six-month follow-up. Over a median follow-up of 30 months, the incidence of breakthrough seizures was 48.6% (61.2% in calcified and 19.2% in resolved, P = 0.001; 32.9% early [within six months] and 15.7% late [beyond six months], P = 0.02). Serum MMP-9 levels were higher in colloidal and vesicular compared with calcified stage (242.5 vs 159.8 ng/mL, P = 0.007); however, there was no significant association with breakthrough seizures and/or calcification in follow-up. In a subgroup of calcified cases (n = 31), the median relative cerebral blood volume on perfusion MRI in and around the lesion was lower in those with seizures (n = 12) than in those without (n = 19) (10.7 vs 25.2 mL/100 g, P = 0.05).
CONCLUSIONS: In post-treatment colloidal or vesicular NCC, incident breakthrough seizures decrease beyond six months. In calcified NCC with remote breakthrough seizures, significant perilesional hypoperfusion is seen compared with those without seizures.
方法:单一,胶体,或囊泡实质性NCC病例接受阿苯达唑和类固醇治疗,并在印度北部的一家三级医院接受随访。在六个月的随访中,在胶体或囊泡治疗初期状态以及一部分钙化病例中估计了血清MMP-9。在6个月的随访中,相同的钙化病例也进行了脑灌注MRI检查。
结果:70例,在6个月的随访中,有70%钙化。经过30个月的中位随访,突破性癫痫的发生率为48.6%(钙化率为61.2%,消退率为19.2%,P=0.001;提前[六个月内]为32.9%,晚[六个月后]为15.7%,P=0.02)。与钙化期相比,胶体和囊泡中的血清MMP-9水平更高(242.5比159.8ng/mL,P=0.007);然而,随访中与突破性惊厥和/或钙化无显著关联.在钙化病例亚组(n=31)中,有癫痫发作的患者(n=12)在病灶内和周围的灌注MRI上的相对脑血容量中位数低于没有癫痫发作的患者(n=19)(10.7vs25.2mL/100g,P=0.05)。
结论:在治疗后的胶体或囊泡NCC中,突发性癫痫发作减少超过六个月。在钙化的NCC伴远程突破性癫痫发作中,与没有癫痫发作的患者相比,可见明显的病灶周围灌注不足。