This study examined psychosocial changes after epilepsy surgery and the effects of psychosocial changes to hopelessness and satisfaction with surgery. Post-operative testing was done on 40 patients following epilepsy surgery (21: seizure-free, 19: non-seizure-free). The analysis revealed the seizure-free group showed more positive psychosocial changes than the non-seizure-free group. On the other hand, some of the patients in both groups showed negative psychosocial changes. Psychosocial changes influenced hopelessness and satisfaction with surgery. The results indicate psychosocial support is needed to decrease hopelessness and increase satisfaction with surgery.

View full abstract

To evaluate the efficacy and pharmacokinetics of intravenous phenobarbital (PB) maintenance therapy for status epilepticus and cluster seizures in childhood, we performed a retrospective study on seven children (median age 2.9 years, range 0.4-15.3 years) . The mean initial and maintenance doses of PB were 18.5mg/kg and 4.8mg/kg/day, respectively. The mean duration of maintenance therapy was 2.6 days. Intravenous PB maintenance therapy was effective in five children, who had focal epilepsies. This therapy allowed us to discontinue midazolam continuous infusion therapy in one child. An electroencephalogram was performed on five children, and electroencephalographic abnormalities were found in two. Drowsiness was found to be an adverse effect in two. The drowsiness disappeared without any other treatment in one. In another child, we could not evaluate the outcome of the drowsiness because of subsequent barbiturate treatment. We suggest that we can perform intravenous PB maintenance therapy without severe adverse effects such as respiratory depression or hypotension, and that this therapy is effective for status epilepticus and cluster seizures in childhood. To keep the serum levels of PB from 20 to 30μg/ml after initial intravenous PB administration, we suggest that the appropriate maintenance dose of PB should be 3.5-5mg/kg/day.

View full abstract

To investigate immune pathophysiology of West syndrome (WS), we measured plasma and cerebrospinal fluid levels of cytokines (IL-1β, 4, 5, 6, 8, 10, 12, 15, 18, IFN-γ, TNF-α) and plasma ACTH level in 51 patients with WS. We compared the cytokine levels in the following conditions: 1) symptomatic or cryptogenic form of the disease, 2) efficacy of treatments, 3) frequency of spasms before treatment, and 4) long-term seizure prognosis. As a result, plasma IL-18 level elevated in all patients. Plasma INF-γ level in the frequent seizure group and CSF IL-15 level in the infrequent seizure group were higher than the counterpart, respectively. However, there was no significant difference in the rest of the comparison studies. We also found an elevation of plasma ACTH level. IL-18 is reported to be found in the endocrine tissue including the adrenal and pituitary glands and to be elevated by activation of hypothalamic-pituitary-adrenal axis. As pathophysiology of WS, an excess of corticotropin-releasing hormone induced by stress during a perinatal period is hypothesized to be a cause of this disorder. IL-18 may play a role in neuro-immuno-modulatory action in WS.

View full abstract

Degenerate diseases such as cerebrovascular disorder, tumor, and dementia are the common causes of elderly onset epilepsy. However, reports suggesting an association with amygdalar enlargement have been published recently. We report a case of elderly onset partial epilepsy accompanied by amygdalar enlargement.
A 67 year-old man was admitted for investigation of nocturnal abnormal motions. From around 64 years of age, the patient started having "attacks of sudden violent motions" during sleep at night. Meanwhile, he also started to show symptoms including amnesia and impaired sense of smell. Dementia or sleep apnea syndrome was diagnosed in another hospital but treatment did not resolve the symptoms. After admission to our center, detailed investigations including video-EEG led to a diagnosis of elderly onset partial epilepsy. Treatment with carbamazepine resulted in resolution of seizures and improvement of amnesia and smell disorder. Right amygdalar enlargement is observed in this patient. From previous case reports and results of kindling rat experiments, we speculate that the amygdala might be subjected to some kind of kindling stimulus and the excitation spread to the anterior cingulate gyrus causing the violent seizures.

View full abstract

A patient with elderly onset temporal lobe epilepsy also manifested amnesia, mood disorder, sleep disorder, urination disorder, and salivary hypersecretion. Anti-voltage-gated potassium channel (VGKC)-complex (leucine-rich glioma inactivated 1 protein:LGI-1) antibody was positive, leading to a diagnosis of anti-VGKC complex (LGI-1) antibody-associated limbic encephalitis (VGKC-LE). This patient had seizures of choking and grimacing face lasting several seconds more than 100 times a day, sometimes with left dystonic upper limb. Irani et al. reported that among VGKC-LE patients, those with anti LGI-1 antibody manifest faciobrachial seizures (FBDS) with grimacing face and dystonic limbs of 3-5 second durations. The present case is the first reported in Japan indicating a relationship between anti-LGI-1 antibody and FBDS.

View full abstract

We report the cases of 2 severely mentally and physically handicapped children with epileptic spasms (ES). The first patient was a quadriplegic 1-year-old boy with carnitine palmitoyltransferase type 2 (CPT2) deficiency with severe mental retardation due to acute encephalopathy. The second patient was a quadriplegic 2-year-old boy with Leigh encephalopathy and severe mental retardation. Topiramate was effectively administered for treating these cases, for which interictal EEGs did not show hypsarrhythmia, but showed high voltage slow waves corresponding to spasms. An accurate characterization of attacks as epileptic spasms, myoclonic seizures, or tonic seizures on the basis of the parents' observations was difficult. However, an accurate evaluation of seizures is essential for determining the appropriate treatment. Therefore, evaluation of the ictal EEG is important for treating severely mentally and physically handicapped children with multiple seizure types, including epileptic spasms.

View full abstract