目的 评估国际抗癫痫联盟（ILAE）耐药癫痫定义专家共识在发展中国家、发展中地区应用的可行性及应用中存在的问题。 方法 2010年12月9日－2011年2月18日，连续登记癫痫专科门诊患者409例。共纳入183例患者，根据ILAE耐药癫痫新定义对每位纳入患者癫痫分类进行评估。 结果 耐药癫痫患者18例（8.7%），临床治愈患者29例（14.1%），不能判断为159例（77.2%）。入组患者共涉及癫痫药物治疗方案321项。根据ILAE定义步骤一分类为不确定的治疗方案共有199项（62.00%），其中数量最多的为服药剂量＜50% WHO限定日剂量有157例（78.89%）。 结论 由于治疗剂量未能够达到国际统一标准，大量患者分类不明确，使得该共识应用面临巨大挑战，但目前为止该共识对于发展中地区耐药癫痫治疗有很强的指导促进意义，对未来耐药癫痫的早期识别有非常大的应用潜力。
ObjectiveTo summarize the clinical features of and prognosis factors for spontaneous intracranial hypotension (SIH). MethodsWe continuously registered hospitalized patients diagnosed with SIH from December 1st, 2010 to February 1st, 2014. Etiology information and clinical features were collected at the first day of admission. Routine blood test and lumbar puncture were done as soon as possible. Every patient got position and fluid infusion therapy. During the one-week follow-up, headache level was evaluated with Visual Analogue Scale (VAS). ResultsThere were 110 patients included, and among them, 39(35.5%) were male and 71(64.55%) were female. The age of onset was between 17 and 91 years old with a mean onset age of (42.0±12.4). Besides postural headache, common signs were nausea (68 cases, 61.8%), vomiting (63 cases, 57.3%), dizziness (40 cases, 36.4%), neck pain (27 cases, 24.5%), and tinnitus (23 cases, 20.9%). VAS at baseline was (7.46±0.86), and at the last follow-up, VAS was (3.45±2.17), with an average improvement of 53.75%. Patients with ANA marker positive had better prognosis. ConclusionSIH can accompany serious brain stem and cerebellum signs, and even meningeal irritation. Cerebrospinal fluid (CSF) changes are similar to virus infection with negative serum virus screening. If CSF leak cannot be found on imaging, patients can improve through fluid infusion therapy and postural treatment.
ObjectiveTo reveal impairments in the perceptual networks in tuberous sclerosis complex (TSC) with epilepsy by functional connectivity MRI (fcMRI). MethodsThe fcMRI-based independent component analysis (ICA) was used to measure the resting state functional connectivity in nine TSC patients with epilepsy recruited from June 2010 to June 2012 and perceptual networks including the sensorimotor network (SMN), visual network (VN), and auditory network (AN) were investigated. The correlation between Z values in regions of interest (ROIs) and age of seizure onset or duration of epilepsy were analyzed. ResultsCompared with the controls, the TSC patients with epilepsy presented decreased functional connectivity in primary visual cortex within the VN networks and there were no increased connectivity. Increased connectivity in left middle temporal gyrus and inferior temporal gyrus was found and decreased connectivity was detected in right inferior frontal gyrus within AN networks. Decreased connectivity was detected at the right inferior frontal gyrus and the increase in connectivity was found in right thalamus within SMN netwoks. No significant correlations were found between Z values in ROIs including the primary visual cortex within the VN, right thalamus and inferior frontal gyrus within SMN, left temporal lobe and right inferior frontal gyrus within AN and the duration of the disease or the age of onset. ConclusionFhere is altered (both increased and decreased) functional connectivity in the perceptual networks of TSC patients with epilepsy. The decreased functional connectivity may reflect the dysfunction of correlative perceptual networks in TSC patients, and the increased functional connectivity may indicate the compensatory mechanism or reorganization of cortical networks. Our fcMRI study may contribute to the understanding of neuropathophysiological mechanisms underlying perceptual impairments in TSC patients with epilepsy.