Etsuko Takaesu

Benign complex partial epilepsies in infancy

Nine infants with benign complex partial seizures, diagnosed by simultaneous electroencephalogram and video recordings, are described. At mostly 3-10 months of age, these infants demonstrated clusters of seizures which consisted of motion arrest, decreased responsiveness, staring or blank eyes mostly with simple automatisms, and mild convulsive movements associated with focal paroxysmal discharges. The seizures were controlled easily with carbamazepine or phenobarbital and all patients remained seizure-free for more than 3 years. Four patients had family histories of benign types of infantile convulsions. Interictal electroencephalogram and psychomotor development were normal in all patients.

Long-Term Prognosis of Convulsive Disorders in the First Year of Life: Mental and Physical Development and Seizure Persistence

Summary: A follow‐up study was made on 304 children (164 boys, 140 girls) with convulsive disorders, excluding occasional convulsions, in the first year of life. All patients except 45 who died were followed until 6 years of age or older. At the final follow‐up, the subjects were divided into six groups according to the degree of mental and physical development (groups I‐VI). Seizures were regarded as absent if the patient had been seizure free for more than 3 years. At the final follow‐up, seizures had ceased in 57.7%, and 43.4% had normal mental and physical development (group I). As to the initial diagnosis, the percentage of group I at the final follow‐up was 81.8% with febrile convulsions and 37.6% with epilepsy. In patients without seizures it was 69.7% with febrile convulsions and 55.8% with epilepsy. Some 80.6% of patients with unclassified generalized motor seizures, 11.5% of those with infantile spasms, 2.9% of those with secondary generalized epilepsy other than infantile spasms, 46.4% of those with partial seizures, and 25.0% of those with hemiconvulsive seizures were finally placed in group I. The percentage of patients without seizures was 81.4, 33.0, 34.4, 57.7, and 100%, respectively. As has been suggested, among the first‐year epilepsies, a subgroup with a more favorable prognosis may exist. Further studies regarding the etiology, ictal EEGs, and effectiveness of treatment and long‐term prognosis of these cryptogenic benign infantile convulsions are needed to provide a firm basis for understanding convulsive disorders in the first year of life.

Epileptic Nystagmus Associated with Typical Absence Seizures

Summary: A 10‐year‐old girl was reported who showed horizontal nystagmus in association with typical absence and 3‐cycles/s generalized, bisynchronous spike‐and‐wave discharges. In view of the general concept that epileptic nystagmus is a manifestation of partial seizures, the occurrence of such an association deserves documentation.

Auditory brainstem response: A comparative study of ipsilateral versus contralateral recording in neurological disorders of children

The auditory brain stem response (ABR) was studied in 103 normal and 90 neurologically abnormal children. The ABR was recorded simultaneously in Cz to the mastoid both ipsilateral and contralateral to the stimulated ear. A comparison between the ipsilateral and contralateral recording demonstrated that contralateral recording resulted in a small increase in the latency of peak V, whereas peak III showed a small latency reduction in the large majority of normal and neurologically abnormal children despite relatively large intersubject variability. There were some cases (cerebral palsy, infantile spasms) which demonstrated abnormal ABR only on contralateral recordings. Since most averaging systems have two channels, it is desirable to record the ABR simultaneously on bilateral recordings.

Predictors of Long-Term Outcome of Convulsive Disorders in the First Year of Life: Clinical Usefulness of Five Risk Factors

The clinical usefulness of 5 risk factors (delayed development before the onset of seizures, abnormal EEG, symptomatic etiology, abnormal past histories, nonbrief symmetric generalized tonic and/or clonic convulsions) used to indicate long-term prognosis was investigated in 295 children who had convulsive disorders in the first year of life. All patients were followed up to the age of 6 years or older. Patients without any of these risk factors showed mentally and physically normal development, and 89% became seizure-free. However, only 10% or less of the high-risk group with 3 or more of these factors became mentally and physically normal. The score of these 5 factors was significantly related to the long-term prognosis and was assumed to be a valuable index to prognosis in the early stages.

Prognostic factors of convulsive disorders in the first year of life

Prognostic factors for mental and physical development and seizure control were investigated in 194 patients with convulsive disorders in the first year of life, excluding infantile spasms, neonatal convulsions and occasional convulsions. Thirty-three patients with febrile convulsions were included in the study. All patients were followed up to age six or older. The cases were subdivided into five etiologic groups; prenatal, perinatal, postnatal, doubtful and cryptogenic. Those with delayed development before the onset of seizures, or neurological abnormalities at the first visit had significantly less chance of being seizure-free and attaining normal mental and physical development. The prognosis for seizures and mental and physical development was much better in the patients with brief, symmetric, generalized tonic and/or clonic convulsions. This was also true with cryptogenic cases. There was a significant correlation between the initial EEGs and the long-term prognosis for mental and physical development and seizure control. Normal EEG was associated with a good prognosis. From these data, the neurodevelopmental status before the onset, the clinical features of convulsions and EEG findings in the infantile period were demonstrated to be important prognostic factors in addition to etiology.

EEG Topography in Porencephaly and Arachnoid Cyst

With the topographic EEG mapping method, the correlation between the morphologic abnormality of the brain and its functional changes was investigated in 13 patients with porencephaly and arachnoid cyst verified by CT scan. The spatial distribution of the EEG activity was displayed in the delta, theta, alpha-1, alpha-2, beta-1, and beta-2 frequency bands. The abnormality of EEG topographic images (e.g., an increase of the power of delta activity and/or a decrease of the power of alpha activity in the involved side of the brain) was more marked in cases with porencephaly than those with arachnoid cyst, and in cases with mental retardation or paralysis than among those without.

Intractable Epilepsy in Children

With the recent advent of new diagnostic techniques such as intensive monitoring, there has been an increasing interest in the management of intractable epi1epsy.l According to a recent collaborative study on intractable epilepsy in children, the seizure frequency was unchanged or had increased at the last visit in 212 (13.4%) of 1,581 epileptic children followed up for more than 3 years in five institutions in Japan? This intractable group was compared with 953 patients whose seizures were completely controlled for more than 3 years. It was found that the intractable group was characterized by the higher incidence of those with the onset before 6 months, underlying disorders, preand postnatal etiological factors, mental retardation, motor disabilities and preceding status epilepticus. Our previous study on the prognosis of the first-year epilepsy also disclosed that those who had persistent seizures beyond 6 years were characterized by the higher incidence of delayed development or neurological abnormalities before the onset of seizures, symptomatic etiology, infantile spasms or other types of secondary generalized epilepsy during the first year, evolution into other types of seizures, abnormal EEGs during

Factors Prognostic of Poor Seizure Outcome in Children with Complex Partial Seizures

Among patients with intractable epilepsy, those with complex partial seizures are frequently found not only in adults, but also in chi1dren.l In children, only a few studies have tried to define the clinical features relevant to a seizure prognosis. The present investigation has been undertaken to elicit factors of a prognostic value for the control of complex partial seizures in children. Eighty-seven patients with a regular follow-up of at least 3 years underwent a diagnostic and therapeutic reevaluation for complex partial seizures. The type of epileptic seizures was defined according to the International Classification of Epileptic Seizures.2 The patients were classified according to the results of treatment. Out of the 87 patients, 38 were free from seizures for at least one year and 49 still had seizures. The clinical features of the 38 patients with seizure control and the 49 patients with persistent seizures were compared by the chisquare test with Yates’ correction. A statistical significance was assumed at p values of less than 5%. The compared clinical features were (1 ) age at onset of seizures, ( 2 ) past history of febrile convulsion or status epilepticus, (3 ) experience of other types of seizures, (4) seizure frequency of more than 2 per day, (5) symptomatic etiology, (6) mental retardation, (7) abnormal background activity, and ( 8 ) focus of seizure discharges. The number of clinical features was shown to be of prognostic relevance (Table 1 ). These clinical features included (1 ) age 2 the first seizure 5 4 months, 9 months, 2 years, or 5 10 years, (2) symptomatic etiology, (3) mental retardation, (4) abnormal background activity, and ( 5 ) TI , TS, 0,, or 0, focus or multifocal seizure discharges. The number of these clinical features in each child related significantly to seizure control (Table 2). In 18 patients who had none of these clinical features, only 2 showed a pxsistence of seizures. The persistence of seizures was observed in 8 1.1 %100.0% of 30 patients who had more than three of these clinical features. Tassinari reported that complex partial seizures with a known etiology (symptomatic) have the poorest prognosis as compared to those of an unknown e t i~ logy .~ Patients with mental and psychopathological disturbances have a significantly poorer seizure prognosis as suggested by Tsai4 In addition to the etiology, Lindsay reported eight fac-

Unclassified Frequent Seizures in a Four‐Year‐Old Boy

A 4-year-old boy was uneventfully born after an uncomplicated pregnancy and had no family history of epilepsy or other neuromuscular diseases. He had developed normally until he was 4 months old, when he first experienced peculiar stereotyped attacks consisting of grimacing and head shaking. The seizures occurred every 3 or 4 minutes when awake and continued every day. He was diagnosed elsewhere as having tics and given haloperidol without any effect. He was referred to us at the age of 2 years and 4 months. He was neurologically normal but mentally slightly retarded. An interictal EEG as well as a (JT scan were normal. His seizures were investigated with simultaneous EEG and videotape recordings (Fig.). At first, his movements stopped and his head hung slightly down. Subsequently, his eyelids, especially the left side, began to droop with depression of the left oral angle and an elevation of the right side of the upper lip. A few seconds later, he began to close his eyes recurrently with a left upward nystagmus, which continued for about 10 seconds and was followed by rhythmical head shaking toward the right synchronously with shouting. Thereafter, he returned to the normal previous condition. He retained consciousness at least initially. The ictal EEG revealed semirhythmic low voltage alpha waves, which increased the amplitude and reduced the frequency. Alpha or theta wave bursts were most marked in the occip’tal areas especially when his eyelids began drooping. A rectal administration of 6 mg of DZP climinated his seizure completely for only one hour without inducing sleep. He was placed on oral CZP, which lessened the seizure intensity and stopped the vocalization associated with the attack. DPH, CBZ, VPA and PB modified the seizure minimally but could not reduce its frequency. The second rectal administration of 10 mg of DZP after withdrawal of all anticonvulsants resulted in a complete cessation of attacks for 1.5 hours without affecting the consciousness level of the patient. He still has seizures every day. Despite frequent seizures, however, he has shown no psychomotor deterioration.