Tamiko Negoro

Long‐term Prognosis after Infantile Spasms: a Statistical Study of Prognostic Factors in 200 Cases

A follow‐up study was made on 200 children (115 boys, 85 girls) who had had infantile spasms, in order to compare their present condition over the age of six years with various prognostic factors. 48 of the children (30 males and 18 females) had died, and all the rest were aged six years or older at the time of final follow‐up. 139 of the children had received ACTH therapy: at final follow‐up, spasms had ceased in 43.5 per cent, and about the same proportion showed normal physical development; 23 per cent had normal mental development and 15.4 per cent were attending ordinary schools. Complete recovery (normal mental and physical development and attending ordinary schools) was achieved in only 19 cases (9.5 per cent). Of the cryptogenic cases, 44.4 per cent had made a full recovery.

Clinical features of benign convulsions with mild gastroenteritis.

The aim of this study is to reveal the detailed clinical features of benign convulsions with mild gastroenteritis (CwG). We studied 114 consecutive episodes in 105 patients with CwG between January 1995 and March 2000. CwG was defined as when a patient met the following two conditions: (a) seizures accompanied the symptoms of gastroenteritis without clinical signs of dehydration or electrolyte derangement and (b) the body temperature remained less than 38.0 degrees C before and after the seizures. Patients with meningitis, encephalitis/encephalopathy or apparent history of epilepsy were excluded. The age of onset ranged from 8 to 52 months (mean, 21.1 +/- 8.4 months). Six patients (6%) had a family history of afebrile convulsions and seven (7%) had one of febrile convulsions. The average interval between the onset of gastroenteritis and that of seizures was 2.3 +/- 1.1 days (range, 1-6 days). The average number of seizures during a single episode was 2.6 +/- 1.5 (range, 1-7). Two or more seizures occurred in 86 (75%) of the 114 episodes. Seizures induced by pain and/or crying were seen in 35 (43%) of 82 episodes. Antiepileptic drugs were administered for 96 episodes. Seizures did not cease after the administration of one kind of antiepileptic drug in 56 episodes (58%). Epilepsy developed in none of the patients. All patients exhibited normal psychomotor development. CwG is characterized by a cluster of seizures that are sometimes induced by pain and/or crying. The seizures are rather refractory to antiepileptic treatment, although the seizure and development outcomes are good.

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.

Partial Seizures Evolving to Infantile Spasms

Summary: We describe the clinical and electroencephalographic (EEG) manifestations of four patients with simultaneous EEG‐video‐telemetry recording (VTR) documented partial seizures evolving to infantile spasms. Clinical manifestations of the partial seizures included cessation of activity, staring, automatisms, increased limb tone, and laughter. In each case, partial seizures were followed by a cluster of infantile spasms. Infantile spasms preceded by partial seizures have not been previously documented.

Benign Infantile Epilepsy with Complex Partial Seizures

Benign infantile epilepsy with complex partial seizures is characterized by a high incidence of family history of benign childhood convulsions, normal development prior to onset, infantile onset, no underlying disorders, no neurological abnormalities, normal interictal EEGs, good response to treatment, and complete remission with normal developmental outcome. Seizures often occur in clusters, consisting of motion arrest, decreased responsiveness, staring or blank eyes mostly with simple automatisms, and mild convulsive movements associated with focal paroxysmal discharges, most frequently in the temporal area.

Benign Partial Epilepsy with Secondarily Generalized Seizures in Infancy

Summary: Seven infants with benign idiopathic partial epilepsy presented with apparently generalized tonic‐clonic seizures (GTCs) which proved to be partial seizures evolving to secondarily generalized seizures. All were easily controlled with phenobarbital (PB) or carbamazepine (CBZ), and remained seizure‐free >3 years even after discontinuation of medication, and had a normal developmental outcome. The initial seizure manifestation consisting of motion arrest, staring or blank eyes, or crying associated with focal paroxysmal discharges was followed by GTCs in accordance with generalization of paroxysmal discharges. Four infants had a family history of benign type of childhood convulsions. The term “benign partial epilepsy in infancy” is proposed to combine this syndrome with the previously described benign infantile epilepsy with complex partial seizures.

Infantile spasms: Etiological factors, clinical aspects, and long term prognosis in 200 cases

The etiological factors, clinical aspects and long term prognosis were studied in 200 patients with infantile spasms. Forty-eight (24.0%) died and the rest were aged 6 years or more at the time of final follow-up. In 73 (36.5%) the etiology was prenatal, in 44 (22.0%) perinatal, and in 17 (8.5%) postnatal: 18 cases (9.0%) were cryptogenic. The remaining 48 (24.0%) patients were doubtful cases. The mortality of the pre-and perinatal cases at 35.6% and 34.1% respectively was significantly higher than that in the other etiologic groups (P<0.001).With regard to the onset of spasms, these were very carly in the pre-and perinatal groups, whereas in the postnatal group the onset was late. The onset in the doubtful and cryptogenic groups was in between. Some 44.4% of the cryptogenic cases showed normal mental and physical development after the age of 6 years, whereas less than 10% of the prenatal and perinatal group did so. Almost all of the doubtful cases became mentally subnormal.The incidence of a family history of epilepsy or other convulsive disorders in the first, second and third degree relatives was highest in the cryptogenic cases (40.0%), and lowest in the perinatal cases (9.3%) (P<0.01). The incidence of laughing attacks was highest in the postnatal cases (42.9%), against no such attacks in cryptogenic cases (P<0.01). The attacks seemed to be linked with organic brain lesions. Among various factors related to the etiology of infantile spasms, genetic propensity seemed especially important in cryptogenic cases.

Surgical treatment for pediatric moyamoya disease: use of the superficial temporal artery for both areas supplied by the anterior and middle cerebral arteries.

OBJECTIVE To revascularize ischemic territories of both the anterior cerebral artery (ACA) and the middle cerebral artery (MCA), a simple and effective combined bypass operation was performed in 36 pediatric patients with moyamoya disease during the past 8 years. METHODS The branches of the superficial temporal artery (STA) were used to revascularize the ACA and MCA territories. In children older than 5 years, the parietal branch of the STA was usually used for an end to side anastomosis with a cortical branch of the MCA. In children who were younger than 5 years, the parietal branch of the STA was used for an encephaloduroarteriosynangiosis instead of a direct anastomosis. Encephaloduroarteriosynangiosis, using the proximal part of the intact frontal branch of the STA, and encephalomyosynangiosis, using the temporal muscle, were also performed in all patients to stimulate spontaneous anastomosis. In addition, bilateral frontal burr holes were made in all patients to induce vascularization of the ACA territories from the distal part of the intact frontal branch of the STA. The first operation was performed on the dominant side, then a similar procedure was performed on the opposite side after an interval of at least 3 months. RESULTS Postoperative clinical symptoms and the findings from magnetic resonance imaging, magnetic resonance angiography, angiography, and electroencephalography demonstrated improvement in all patients. CONCLUSION These results suggest that the placement of bilateral burr holes (while leaving the frontal branch of the STA intact), in addition to the STA-MCA anastomosis, encephaloduroarteriosynangiosis, and encephalomyosynangiosis, is very effective in vascularizing the ischemic ACA and MCA territories in pediatric patients with moyamoya disease.

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.

Efficacy of antiepileptic drugs in patients with benign convulsions with mild gastroenteritis

The aim of this study was to clarify the efficacy of antiepileptic drugs during a cluster of seizures in patients with convulsions with mild gastroenteritis (CwG). We retrospectively investigated the details of antiepileptic treatment in 110 consecutive episodes in 103 patients with CwG. The temporal course of the seizures and the use of antiepileptic drugs were investigated in each episode. Drugs were judged as effective when seizure cessation was achieved after administration of the drug. As the first drug, diazepam (DZP)/bromazepam (BZP) was effective in 38%, phenobarbital (PB) in 40%, and lidocaine (LD) in 100%. As the second drug, DZP/BZP was effective in 42%, PB in 69%, and LD in 100%. As the third drug, PB was effective in 70%. When the efficacy of the first doses of PB and LD were compared, the efficacy rate was significantly higher for LD than for PB (P = 0.047). In conclusion, LD was effective for the cessation of seizures in patients with CwG.