We examined the incidence of hyperuricemia due to sodium valproate (VPA) administration. We evaluated pediatric epileptic patients who received VPA and reviewed VPA concentration in blood, uric acid value in blood, and urate clearance ratio for creatinine clearance.
VPA concentration in blood and uric acid values in blood were correlated, and blood concentration and urate clearance ratio were negatively correlated. Hyperuricemia with degradation of uratic clearance rate indicates the presence of a kidney excretion disorder. Therefore, uratic excretion disorder involving the kidney suggests an eruption of hyperuricemia due to VPA.

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Efficacy of intravenous midazolam was evaluated in 97 children (1 month to 16 years of age) with status epilepticus (108 seizure episodes) from 1996 to 2004. The etiologies of 108 seizure episodes were as follows: epilepsy in 50, febrile convulsion (FC) in 37, and acute encephalopathy/encephalitis in 10. In epilepsy group, the duration of status epilepticus was significantly short in patients with midazolam treatment compared with those without midazolam administration. And the rate of seizure cessation was significantly higher in patients with midazolam than those with diazepam treatment in epilepsy group. Adverse effects of midazolam were recognized in only one patient showing transient hypoxemia, but mechanical ventilation was not required. Considering these results, it is suggested that intravenous midazolam is efficacious and safe treatment for status epilepticus in children.

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We followed up 68 patients with temporal lobe epilepsy and succeeded to assess four episodes of postictal psychosis with BPRS and SDAS. We extended the concept of postictal psychosis and defined it as psychotic episodes occurring within 1 week after the end of seizures or within 3 days before the end of seizures. The average of initial BPRS scores (3.3 days after the end of the seizure cluster) was 18.8, decreased up to 6.3 after one week, and finally normalized to 1.5 after one month follow-up. There was a statistically significant decrease of BPRS scores between the initial assessment and the assessment after one week (t=4.887, p=0.039). Interestingly, those who showed psychotic episodes without lucid interval tended to have very frequent episodes (more often than monthly) and to experience additional seizure recurrence even during the psychotic episodes. In the discussion, we tried to compare nuclear postictal psychoses with periictal ones and related the latter to limbic status epilepticus proposed by Wieser.

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A girl with myolonic absence epilepsy (MAE), confirmed with repetitive provocations of absences by hyperventilations (HV), was reported. She first exhibited absence seizures (AS) at age 3, and visited to our clinic at age 6. An AS was provoked by HV, but myoclonic jerk of the neck or the shoulders was not observed at that time. As generalized 3 Hz spike-waves were present on EEG, valproic acid (VPA) was initiated with a diagnosis of childhood absence epilepsy. However, AS was not decreased instead of high serum VPA concentration (99 μg/ml). At age 8, a Video-EEG during HV was evaluated. Although, rhythmical myoclonic jerks of the neck and the shoulders did not develop in the first AS, they developed after the second AS, consistent with MAE. AS ceased with high dose VPA (138 μg/ml). Following results are recommended in this study; to observe AS or Video-EEG with repeated AS provocations by HV is necessary in patients with intractable AS to confirm MAE.

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Periodic spasms (PS) is an epileptic seizure type exhibits West syndrome-like series-forming spasms. Ictal EEG consists of generalized high voltage slow waves preceded by fast waves. This type of discharge is often misinterpreted as a non-epileptic discharge, because it lacks spike or sharp wave. Observation on the ictal symptoms or a video-EEG is necessary to confirm PS. PS is usually resistant to antiepileptic drugs. The present patient first experienced PS at the age of 18 months. Complex partial seizures, sporadic tonic spasms and drop attacks emerged and increased in frequency in spite of polypharmacy. At age 15 he was subjected to a total corpus callosotomy because of the increased incidence of traumas due to drop attacks. During two years of follow-up after callosotomy, all types of seizures have never been recurred. The reason why the callosotomy was effective in this case was not clear. However, it suggests that the role of the corpus callosum is not only a pathway of epileptic discharges but affects on the bilateral synchrony of multifocal epileptic discharges.

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We report two male children aged 7 and 8 years suffering from drug-resistant epilepsy with myoclonic absences, for whom bromide was effective in controlling seizures and improving IQ. The diagnosis was made based on characteristic myoclonic absences and ictal EEG (3 Hz spike-wave) in one child, and on ictal video EEG-EMG findings of 3 Hz spike-wave discharge accompanied by deltoid activity at 3 Hz in the other. Use of bromide as an adjunctive drug for the two patients resulted in significant reductions in seizure frequency and improvements in IQ. The diagnosis of epilepsy with myoclonic absences can be difficult because myoclonias are overlooked when there is tonic contraction or patients are being treated with antiepileptic drugs, and because interictal EEG may not exhibit 3 Hz spike-wave discharges. Therefore, it is important to diagnose this condition earlier with ictal video EEG-EMG, and to consider bromide for patients whose seizures are intractable to other antiepileptic drugs.

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A severely handicapped 15-year-old girl had been treated with valproic acid since age 4 months. She was admitted to our hospital because of fever and increased CRP level. Laboratory examinations revealed no abnormal findings except for the increased CRP level (16.8 mg/dl) and leukocyturia (50-99/hp). Pyrexia disappeared soon, however, elevated CRP and leukocyturia persisted despite administration with various antibiotics and antifungal drugs. Although renal tubular function remained normal, gallium scanning showed mild renal uptake. A renal biopsy revealed mild interstitial edema and cell infiltrations. Valproic acid was replaced, and thereafter she showed marked improvement of the laboratory data. Although valproic acid-induced renal tubular involvement is well-known, acute interstitial nephritis without renal tubular involvement is rare. We have to pay attention to adverse renal effects such as interstitial nephritis when we encountered leukocyturia due to unknown origin.

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We report two Japanese boys with Klinefelter syndrome (KS) complicating focal epilepsy with its onset in infancy. Case 1, aged 4 years, showed multiple minor anomalies, borderline mentality and epilepsy. Case 2, aged 9 years, had moderate psychomotor delay and epilepsy. Chromosomal analysis revealed 47,XXY, establishing the diagnosis of KS in early infancy. The previous literatures have not described seizure types or EEG abnormalities specific to KS but many cases were described to have focal epilepsies. The presences of phobia with visual hallucination in case 1 and oro-alimentary automatism in case 2 suggest the involvement of amygdalohippocampal region in the temporal lobe. As KS is well known to complicate schizophrenia, it is suggested that functional impairment of the temporal lobes may be caused by chromosomal inbalance in KS or by epileptic discharges themselves spreading easily to the temporal lobes.

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The patient was a 12-month-old male infant who experienced the first seizure at 27 days of age. He was born at full term and he had no abnormality at birth. Seizures developed from twitches of the left or right eyelid to tonic jerks of one or both upper limbs. Ictal EEG showed spikes started in the right or left occipital region and spread out to the entire cerebrum, and spikes moved to the frontal region. Ictal ECD-SPECT showed hyperperfusion in the occipital region. Conventional antiepileptic drugs failed to control the seizures. His development arrested and he showed spastic quadriplegia and severe mental retardation. His brain MRI was normal and there was no cortical dysplasia. He was diagnosed as having occipital lobe epilepsy; however, his seizure types, other symptoms and ictal EEG resemble to the malignant migrating partial seizures in infancy. Intractable partial seizures and migrating epileptic focus may depend on the brain immaturity in infancy.

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We report a 14-year-old boy with hypothalamic hamartoma whose symptoms improved after second gamma knife radiosurgery. The patient developed epilepsy at three months of age. The patient had gelastic seizures, complex partial seizures, and secondarily generalized tonic-clonic seizures. His seizures were intractable to conventional antiepileptic drugs, and sometimes occurred in clusters or as status epilepticus. At the age of 5 years and 10 months, he underwent the first gamma knife radiosurgery with a central dose of 28 Gy and marginal dose of 14 Gy. However, the frequency of seizures was unchanged and behavior problems became more evident. Six years after the first radiosurgery, the second gamma knife radiosurgery (central dosage 36 Gy and marginal dosage 18 Gy) was performed. Four months later, however, he developed dyshidrosis and headache which subsided immediately after administration of steroid. MRI demonstrated transient expansion of hamartoma with enhanced lesion and brain edema. Complex partial seizure and secondarily generalized tonic-clonic seizure ceased five months after the second radiosurgery. Two years after the second radiosurgery, he has rare gelastic seizures and there are no behavior problems or obvious complications. The second gamma knife surgery might be effective, especially in patients with hypothalamic hamartoma whose first gamma knife surgery has failed to improve symptoms due to low marginal dosage.

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