Curatolo P
Section of Pediatric Neurology, University of Rome, Tor Vergata, Rome, Italy.
Childs Nerv Syst. 1996 Sep;12(9):515-21. doi: 10.1007/BF00261603.
CNS lesions of tuberous sclerosis complex (TSC) are due to a developmental disorder of neurogenesis and neuronal migration. MRI studies provide excellent in vivo demonstration of the various pathologic lesions. Symptoms of cortical tubers may include seizures, mental retardation, learning disabilities, and abnormal behavior. Seizures have a focal or multifocal origin, this clinical feature depending on the localization of the cortical tubers. Epilepsy associated with TSC is often intractable, but seizure control has benefited from the introduction of the new antiepileptic drugs. Carefully selected drug-resistant patients can be assessed with intensive monitoring as candidates for surgical removal of epileptogenic lesions. The success of epilepsy surgery is predicated on the clear identification of epileptogenic foci.
结节性硬化症（TSC）的中枢神经系统病变是由于神经发生和神经元迁移的发育障碍所致。MRI研究能在体内很好地显示各种病理病变。皮质结节的症状可能包括癫痫发作、智力迟钝、学习障碍和异常行为。癫痫发作起源于局灶性或多灶性，这一临床特征取决于皮质结节的定位。与TSC相关的癫痫通常难以治疗，但新型抗癫痫药物的引入有助于控制癫痫发作。经过精心挑选的耐药患者可通过强化监测进行评估，作为手术切除致痫病灶的候选对象。癫痫手术的成功取决于明确识别致痫灶。
