结节性硬化症患者颅内皮层结节对智力的影响

The effects of the cerebral cortical tubers on the intelligence in patients with tuberous sclerosis complex

目的通过对患者影像学资料的分析,研究结节性硬化症(tuberous sclerosis complex,TSC)患者颅内皮层结节对智力的影响。方法本研究收集了73例临床确诊的TSC患者,包括影像学资料及韦氏智力测试结果,分析颅内皮层结节的数量、部位及类囊状结节对患者智力的影响。结果在入组患者中男43例,女30例,患者出现癫痫的发病年龄最小10 d,最大28岁。智力正常组36例(49%),智力低下组37例(51%)。颅内皮层一侧有结节者4例(5%),双侧均有结节者69例(95%)。左右两侧脑区皮层结节数量之间无统计学差异(P=0.707)。全脑皮层结节数量>15个患者比结节数量≤15个患者更容易出现智力低下(P=0.010)。左颞叶皮层结节数量(P=0.011)、双侧颞叶皮层结节数量(P=0.022)及左侧脑区皮层结节数量(P=0.032)在智力方面差异有统计学意义。27例患者头颅MR上至少存在一个类囊状结节,未发现类囊状结节对患者智力的影响(P=0.524)。结论颅内皮层结节数量>15的患者癫痫发病年龄早,容易出现智力低下。智力低下与左颞叶结节数量、双侧颞叶结节数量及左侧脑区结节数量有关。

Objective To study the effects of the cerebral cortical tubers on the intelligence of patients with tuberous sclerosis complex （TSC） using MRI. Methods We collected the imaging information and intelligence test results of seven- ty-three patients with TSC. We further examined the effects of the number of cerebral cortical tubers, location and cyst-like cortical tubers on intelligence. Results Among total of 73 patients, 43 patients were male and 30 patients were female. The onset age of seizure ranged from 10 days to 28 years. Thirty-six patients （49%） had normal intelligence whereas thirty-seven patients （51%） had intelligence decline. Sixty-nine patients had bilateral cortical tubers whereas four patients had unilateral cortical tubers. There was no statistically significant difference in the number of the cortical tuber between left hemisphere and right hemisphere （P=-0.707）. Patients with cortical tubers over 15 were more likely to have intelligence decline than patients with cortical tubers less than 15. The cortical tuber in the left temporal lobe （P=-0.011）, bilateral temporal lobe （P=-0.022） and left hemisphere （P=-0.032） were significantly related to intelligence decline. Twenty-seven patients had at least one cyst-like cortical tuber on MRI images. There were no statistically significant cor-relation between cyst-like cortical tubers and intelligence decline （P =0.524）. Conclusions The patients with cortical tu-bers over fifteen have the early onset age of seizure and intelligence decline.The intelligence decline is correlated with the cortical tubers in the left temporal lobe, bilateral temporal lobes and left hemisphere.