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目的探讨神经元移行异常(NMD)的临床特征和诊断依据。方法对37例NMD患儿的临床、影像和实验室资料进行分析,并进行随访。结果37例NMD中无脑回巨脑回21例,主要临床表现为智力低下(20例)、癫(14例)和脑性偏瘫(6例),头围小于正常同龄儿是一重要体征,占80%(17/21);无脑回巨脑回合并其他畸形8例;灰质异位5例,主要表现为癫(4例);脑裂畸形3例,脑性偏瘫为其主要临床特征(2例)。16例脑电图中12例异常,全导阵发不规则尖棘慢波、慢波10例。本组随访18例,癫患儿14例,经抗癫药物治疗后均难以控制发作,运动发育有改善但语言发育障碍4例。结论NMD的临床特征以智力低下、癫及脑性偏瘫为主,头颅MRI是诊断本病的最佳检查方法。
Objective To investigate the clinical features and diagnostic criteria of neuronal migration abnormalities (NMD). Methods The clinical, imaging and laboratory data of 37 children with NMD were analyzed and followed up. Results Totally 21 cases of NMDA without brain back were found in 37 cases of NMD. The main clinical manifestations were mental retardation (20 cases), epilepsy (14 cases) and cerebral hemiparesis (6 cases). The head circumference less than normal peers was an important sign , Accounting for 80% (17/21); no brain back to the giant brain round and other deformities in 8 cases; gray matter ectopic in 5 cases, mainly for epilepsy (4 cases); schizencephaly in 3 cases, cerebral hemiplegia as its main Clinical features (2 cases). Of the 16 cases, 12 cases were abnormal in EEG, and all-spike had irregular sharp spike and slow wave in 10 cases. This group were followed up in 18 cases, 14 cases of epilepsy in children, after the treatment of epilepsy drugs are difficult to control the attack, exercise development improved but language development disorders in 4 cases. Conclusion The clinical features of NMD are mainly mental retardation, epilepsy and cerebral hemiparesis, and cranial MRI is the best method to diagnose this disease.