Kazuichi Yagi

Long‐Term Course of Childhood Epilepsy with Intractable Grand Mal Seizures

Abstract: Twenty‐nine children with childhood epilepsy characterized by frequent grand mal (generalized tonic‐clonic) seizures in spite of maximal doses of antiepileptic drugs and by an early onset of seizures (before 1:year of age) were followed up for more than 5:years. The children were divided into 3:groups: severe myoclonic epilepsy in infancy (SME), no SME, and intractable childhood epilepsy with generalized tonic‐clonic seizures (GTC). In all the 3:groups, the grand mal seizures persisted, whereas the other types of seizures tended to disappear as the patients aged, and the prognosis for mental development was poor. In the majority of cases in all the 3:groups, the waking grand mal seizures altered to sleep grand mal seizures with aging. Two pairs of monozygotic twins with SME suggested that genetic factors play a role in this epileptic syndrome. Intractable childhood epilepsy with GTC is distinguished by the absence of other types of generalized seizures. It cannot be regarded as an epileptic syndrome, but its pathogenesis and treatment require further studies.

Emotional Facial Expressions at the Onset of Temporal Lobe Seizures: Observations on Scalp and Intracranial EEG Recordings

Abstract: The initial facial expressions of 195 complex partial seizures of 98 patients with temporal lobe epilepsy were reviewed in relation to the laterality and focality of electroencephalographic (EEG) seizure origin. A neutral expression was observed most often (71) followed in frequency by expressions of disgust (13), happiness (7) and sadness (7). There was no expression of anger, surprise and fear.

Neuropathology of Hippocampus of Intractable Temporal Lobe Epilepsy

A neuropathological investigation of specimens of the temporal lobes surgically resected for the amelioration of intractable epileptic seizures provides us with useful information on the postoperative seizure prognosis as well as etiologies of epilepsy. In 18 cases whose epileptogenic focus in space was accurately identified by means of a chronic depth EEG recording and who subsequently underwent an anterior temporal lobectomy including the hippocampus, a correlative study was conducted between the site of focus exemplified by depth EEG exploration and neuropathological findings of the hippocampus in which the volumetric cell density was measured as a quantitative evaluation.

Inositol Trisphosphate (IP,) Receptors and Epileptic Seizure

The effects of anticonvulsants and Ca2+ channel antagonists on the inositol trisphosphate (IP3) binding and IP3-induced Ca2+ release were examined in brain membrane fractions. Anticonvulsant (PHT and valproate) and Ca2+ channel antagonists (verapamil, diltiazem, flunarizine, nicardipine and cinnarizine), examined did not significantly inhibit the IP3-receptor binding. The Kd and Bmax values of the IP3 binding did not change significantly in the various brain regions of the amygdala-kindled rats, killed 10 days after the last seizure, compared to those of controls. On the other hand, PHT, PB and carbamazepine inhibited the Ca2+ releasing activity of IP3 in the cerebellar membrane fractions by approximately 20% at therapeutic concentrations.

A peculiar state observed in 4 patients with severe myoclonic epilepsy in infancy.

A peculiar state observed during status epilepticus was documented by CCTV/EEG in 4 patients with severe myoclonic epilepsy in infancy (SME).l The state was characterized by slight clonic movements of the extremities and/or face accompanying bilateral, diffuse high voltage slow waves and those occasionally notched with spikes. All of the 4 cases were male ranging from 5 to 7 years of age (Table 1). Cases 1 and 2 were identical twins. Their initial attack began with clonic seizures during the first year of life and later combined with myoclonic seizures in all the cases and complex partial seizures in 2 cases. Their mentality seemed to be normal before the onset, and severe mental retardation became manifest following the onset of seizures. Despite a heavy medication, the convulsive seizures were extremely therapy resistant. They seemed to fulfill the diagnostic criteria of SME.l The peculiar state occurred after a short interval (40 sec. in case 1, 330 sec. in case 2, undetermined in case 3, 690 sec. in case 4) following generalized tonic-clonic seizures (GTCS) that had been repeating in all the 4 cases, in other words, never without antecedent GTCS. Namely, this state was observed as part of GTCS status epilepticus. Fig. 1 shows the mode of occurrence of the peculiar state in the 4 cases. The state began with eye opening and slight clonic movements of the extremities and/or face, paleness and change in the respiration rate were observed. The state lasted for 7 min. in case 1, 10 min. in case 2, about 16 min. in case 3, and 1-15 min. in case 4. The concurrent EEGs during this state showed bursts of bilateral, diffuse high voltage slow waves and those occasionally notched with small spikes (Fig. 2). This state was considered as an ictaI phenomenon based on the fact that it accompanied epileptiform discharges and occurred after an intermission following the cessation of GTCS. Namely, it apparently differed from the post-grand ma1 confusional state or posthypoxic myoclonus. The clinicoelectrographic features of this state might resemble those of “atypical absence” status which in children occurs as a repetition or prolongation of short-lived atypical absences.2 However, all the 4 cases had not shown any atypical absence as an identical seizure type. Further, it is absurd to recognize two different status epilepticus at a time. A “peculiar state” was used to denote this unusual nonconvulsive longstanding condition. Dravet et al. observed a state of disturbance of consciousness with myoclonia in cases with SME and coined the name “obtundation status.”l Although the definition

Neuropsychological Evaluation before and after Surgical Treatment of Temporal Lobe Epilepsy

Abstract: We present the results of pre‐ and postoperative neuropsychological evaluations of 58:patients with temporal lobe epilepsy who underwent a chronic intracranial EEG monitoring and a subsequent standard anterior temporal lobectomy. Wadas test provided valuable information on the speech dominant side and on the focus lateralization. Some warning signs as well as verbal automatisms indicated the effect for focus localization and lateralization. The results of interictal neuropsychological tests suggested that each subgroup of TLE performed differently. A postoperative neuropsychological performance has improved in many tests that may be explained by the diminished epileptic bombardment resulting from the resection.

A Correlative Study between Hippocampal Atrophy Quantified by Tomo‐Pneumoencephalography and Epileptogenic Focus in Temporal Lobe Epilepsy

It appeared certain that we can quantify the rate of hippocampal atrophy by utilizing the sagittal cuts in tomo‐pneumoencephalog‐raphy. This is a reliable method to infer the side of the epileptogenic focus in temporal lobe epilepsy since a close correlation was disclosed between the side with more atrophic features of the hippocampus and that of the epileptogenic focus explored by depth EEG, in particular, in the mesial temporal focus group. On the other hand, it seems to be plausible that the hippocampal atrophy could be secondarily induced by epileptic discharges in the lateral temporal group. Namely, controversies dealing with the casual relationship of hippocampal atrophy should be discussed based on the epileptogenic focus localization in temporal lobe epilepsy.

Nonconvulsive Status Epilepticus

Status epilepticus of nonconvulsive seizures has been divided into that of absences and of complex partial seizures. Although absence status has heretofore been the subject of numerous articles, complex partial status was regarded as a rare condition. In the meantime, attention of many investigators has been concentrated on the nature of nonconvulsivc status epilepticus since the advent of new strategies of CCTV-EEG as well as SEEG. In this symposium, studies of absence status and complex partial status are presented and discussed in terms of clinicoelectroenczphalographic manifestations. In addition to these two types of nonconvulsive status, a study of epilepsy with continuous diffuse spike-waves during sleep (CSWS) is presented in an implication of subclinical electrical status. Absence status epilepticus is, as in other types of status, divided into two categories; a recurrent form and continuous form according to the mode of occurrence. The recurrent form is a repetition of an absence seizure which occurs commonly in children with Lennox-Gastaut syndrome or other symptomatic generalized epilepsies. The continuous form is a prolongation of an absence seizure which occurs generally in female patients over the age of 18 without habitual

A clinical study on frontal lobe epilepsy.

Of 30 patients whose epileptogenic focus was presumed to be located in the frontal lobe on the basis of a clinico-electrographic correlation, the principal seizure manifestation consisted of peculiar automatisms in about 40% of the cases. However, the nature of the automatisms was of a short duration lasting about one minute but in most cases it was accompanied by extremely violent movements, but this was not true in all cases. Of the 30 cases, only 5 cases showed marked improvement one year after being discharged from the hospital so that this type of seizure event is extremely intractable.

Intractable epilepsy and disturbed visuomotor performance.

Abstract: The factors relevant to intractability, types of epilepsy and impairment of dexterity in patients with intractable epilepsy were studied independently in different groups of patients. The factors relevant to intractable epilepsy that were disclosed in 202 patients, who required hospitalization more than twice, were as follows: strong seizure propensity, neuropsychiatric disorders including mental deterioration of various degrees, ataxia, personality changes and psychotic episodes, intolerance to antiepileptic drugs due to acute or chronic side effects, idiosyncrasy and internal disorders, self‐induced seizure, misdiagnosis and mistreatment, and breakdown of family care of patients. The types of epilepsy in 224 patients with intractable epilepsy whose seizures were not adequately controlled and recurred on a monthly basis in spite of hospitalization were classified as follows: 101 patients with localization‐related epilepsies or syndromes, 106 with generalized epilepsies or syndromes, 16 with undetermined epilepsies or syndromes and one with specific syndrome. In regard to partial epilepsies, frontal lobe epilepsy and partial epilepsy with multiple foci were at least partially intractable as temporal lobe epilepsy. With respect to intractable generalized epilepsies, miscellaneous symptomatic generalized epilepsies like intractable grand mal epilepsy with progressive mental retardation in childhood were as important as Lennox‐Gastaut or West syndrome though it defies classification into any established syndromes. The proposed International Classification of Epileptic Syndromes and Epilepsies1 was found adequate for analysis of intractable epilepsy. The disturbance of fine motor performance found in 84 patients who participated in occupational therapy was investigated by test programs comprising nine subbatteries. Disturbance of manual dexterity was found in about 80% of the patients and that of the visuomotor performance in about 90%. These impairments seemed to be responsible for the patients failing to be properly employed. It is necessary that in addition to persisting seizures, neuropsychological impairment must be identified for better social care for patients with intractable epilepsy.