Kubota M, Nagata J
Department of Pediatric Neurology, Metropolitan Medical Center for the Severely Handicapped, Tokyo, Japan.
No To Shinkei. 1993 Jul;45(7):669-71.
We reported a 33-year-old man with Lennox syndrome of 26 years' duration associated with unusual symptom complexes such as severe cerebellar ataxia and dysarthria, and peripheral neuropathy. His convulsive disorder was very intractable despite multiple anticonvulsants including phenytoin (PHT), phenobarbital (PB), primidone (PRM), valproate and so on. At the age of 25 he was no longer able to walk without help. PHT blood levels were kept almost within the therapeutic range, while PB blood levels tended to be greater than the therapeutic range. Needle EMG study revealed denervation pattern. Motor conduction velocity of the peroneal nerve was 25.2 m/s and sensory conduction velocity of the sural nerve could not be elicited. Brain CT and MRI showed the marked cerebellar atrophy predominant in the vermis. To our knowledge there were no previously reported cases of Lennox syndrome associated with such cerebellar dysfunctions and peripheral neuropathy. From the clinical course and laboratory findings, metabolic disorders and degenerative diseases were ruled out. We consider his cerebellar symptoms and peripheral neuropathy could be attributable to the long-term use of multiple anticonvulsants, i.e. PHT in combination with PB and PRM. These symptoms seem to be irreversible, because our patient's condition did not change after PHT and PB dose reduction, and discontinuation of PRM.
我们报告了一名33岁男性，患有病程长达26年的Lennox综合征，伴有严重小脑共济失调、构音障碍和周围神经病变等不寻常的症状复合体。尽管使用了多种抗惊厥药物，包括苯妥英（PHT）、苯巴比妥（PB）、扑米酮（PRM）、丙戊酸盐等，他的惊厥性疾病仍然非常难治。25岁时，他在没有帮助的情况下已无法行走。PHT血药浓度几乎保持在治疗范围内，而PB血药浓度往往高于治疗范围。针极肌电图检查显示失神经模式。腓总神经运动传导速度为25.2 m/s，腓肠神经感觉传导速度未引出。脑部CT和MRI显示明显的小脑萎缩，以蚓部为主。据我们所知，此前尚无Lennox综合征合并此类小脑功能障碍和周围神经病变的病例报道。从临床病程和实验室检查结果来看，排除了代谢紊乱和退行性疾病。我们认为他的小脑症状和周围神经病变可能归因于长期使用多种抗惊厥药物，即PHT联合PB和PRM。这些症状似乎是不可逆的，因为在降低PHT和PB剂量以及停用PRM后，我们患者的病情并未改变。
