Suzuki A, Aso K, Ariyoshi C, Ishimaru M
Department of Pediatrics, Japanese Red Cross, Nagoya First Hospital.
Epilepsia. 1992 Jan-Feb;33(1):108-11. doi: 10.1111/j.1528-1157.1992.tb02291.x.
A young woman with acute intermittent porphyria (AIP) and juvenile myoclonic epilepsy began to have generalized tonic-clonic seizures (GTCs) at age 13. Subsequently, she had myoclonic seizures, which were often precipitated by visual stimulation, tended to occur in the morning, and sometimes evolved into GTCs. Valproate (VPA) resulted in a worsening of latent AIP, and treatment with a combination of phenytoin (PHT), carbamazepine (CBZ), and clonazepam (CZP) led to severe neuropathy of AIP and an electrolyte imbalance. These conditions were improved by water restriction, infusion of high doses of carbohydrates, and discontinuation of all antiepileptic drugs (AEDs) except for CZP. CZP appeared to be effective both in improving GTCs and myoclonic seizures and did not induce any symptoms of AIP. CZP may be porphyrogenic but can be used safely at a low dose for treatment of epilepsy in patients with AIP.
一名患有急性间歇性卟啉病（AIP）和青少年肌阵挛性癫痫的年轻女性在13岁时开始出现全身强直阵挛发作（GTCs）。随后，她出现肌阵挛发作，常由视觉刺激诱发，多在早晨发作，有时会演变为GTCs。丙戊酸盐（VPA）导致潜伏性AIP恶化，苯妥英（PHT）、卡马西平（CBZ）和氯硝西泮（CZP）联合治疗导致严重的AIP神经病变和电解质失衡。通过限制水分摄入、输注高剂量碳水化合物以及停用除CZP外的所有抗癫痫药物（AEDs），这些情况得到改善。CZP似乎对改善GTCs和肌阵挛发作均有效，且未诱发任何AIP症状。CZP可能具有卟啉原性，但可在低剂量下安全用于治疗AIP患者的癫痫。
