Yasuyuki Futagi

Ictal video-EEG recording of three partial seizures in a patient with the benign infantile convulsions associated with mild gastroenteritis

Summary: Purpose: In infants, benign convulsions can be triggered by febrile illness or mild diarrhea such as Rotavirus gastroenteritis. The triggering mechanism of these convulsions is still unknown. In spite of several reports concerning clinical features, the ictal EEG recordings were rarely analyzed by a video‐EEG monitoring system. To reveal a clue for the triggering mechanism of these convulsions, we analyzed the correlation of clinical manifestations and the EEG discharges during the ictal events and compared with previous reports.

Primitive Reflex Profiles in Infants: Differences Based on Categories of Neurological Abnormality

In order to clarify reflex profiles in the first year of life in connection with categories of neurological abnormality, six primitive reflexes, i.e., the crossed extensor reflex, suprapubic extensor reflex, heel reflex, Galant response, asymmetric tonic neck reflex and plantar grasp response, were examined in 458 normal infants, 78 infants with cerebral palsy (CP) and 81 infants with mental retardation (MR), whose diagnoses were confirmed at a later follow-up examination. The change in the mean score for each of these reflexes with age was characteristic for each category or type of neurological abnormality. This implies that a presumptive diagnosis can be made in neurologically high-risk infants by examination of the primitive reflexes. Such reflexes are therefore of specific significance, among other neurological criteria, in infants within the first year of life.

A Japanese patient with the Costello syndrome

The Costello syndrome is characterized by dwarfism, unique cutaneous lesions, distinct facial gestalt, and mental retardation. We present a Japanese patient with the Costello syndrome. She showed high serum IgM level during the early infantile period. Nissens fundplication was carried out to treat severe gastroesophageal reflux. Endocrinological investigations revealed a partial deficiency of growth hormone.

Hypomelanosis of Ito associated with hemimegalencephaly: A clinicopathological study

A girl with hypomelanosis of Ito was studied both clinically and at postmortem examination. She manifested severe epilepsy early after birth. Magnetic resonance imaging demonstrated left-sided hemimegalencephaly. The seizures were secondarily generalized or unilateral initially, followed by infantile spasms with asymmetrical hypsarrhythmia at 1.5 months of age. Frequent complex partial seizures, refractory to anti-epileptic drug treatments appeared at 4 months of age. She died of pneumonia at the age of 14 months. Postmortem examination revealed marked asymmetry of the cerebrum and gyral abnormalities in the left cerebral hemisphere. Histopathologically, severe disorganization of the neuronal cytoarchitecture was evident. Absence of delineation between cortical gray and white matter was evident, as was increase and hypertrophy of the neurons and glial cells. We believe that the association of skin and brain lesions was not one of chance; that is, they may share a common pathogenetic mechanism.

Theta rhythms associated with sucking, crying, gazing and handling in infants

In order to specify the locations and to clarify the electrophysiological significance of the transitory rhythmic theta activities detected on scalp electrodes in infants, we performed simultaneous EEG and video recording with power spectral map analysis in 29 normal infants of less than 1 year of age. The rhythmic theta activities appeared in posterior temporal regions with sucking or crying, in the parietal region with gazing, and in the frontal region with handling. Each specific location of the theta rhythm seemed to correspond to the functional localization in the infants brain. We thus concluded that these rhythmic theta activities might originate from direct cortical activation, or from the cortical activation driven by the neuronal impulses from the limbic system through the connection between that system and the cortex.

Neurodevelopmental outcome in children with fetal hydrocephalus

Thirty-eight children with fetal hydrocephalus treated surgically during the neonatal period were analyzed concerning their neurodevelopmental outcome by means of a follow-up study. The mean gestation at diagnosis was 31.1 weeks, and the mean postconceptual age at the first operation was 36.3 weeks. The ages of the patients at the last follow-up ranged from 3.8 to 11.8 years (mean = 7.4 years of age). During the follow-up period, they underwent neurologic examinations and were also evaluated to determine the developmental quotient or intelligence quotient. The relationships between age at diagnosis or the first operation for hydrocephalus and neurodevelopmental outcome, and between the lateral ventricular width/hemispheral width ratio at diagnosis and age at diagnosis or the first operation, and neurodevelopmental outcome were statistically evaluated. The neurodevelopmental outcome in the patients was normal in three patients, borderline intelligence in one patient, mental retardation in seven patients, and motor disturbance in 27 patients (five of whom were intellectually normal). The etiology was a major determinant of the outcome in the children with early postnatal surgical treatment of fetal hydrocephalus. Furthermore, an early onset and a high lateral ventricular width/hemispheral width ratio at diagnosis of hydrocephalus were significantly correlated with a poor intellectual outcome.

H-reflex study in normal children and patients with cerebral palsy

The H-reflex was studied in 53 children with cerebral palsy, and the results were compared with those obtained for 56 normal control subjects. Pairs of identical stimuli were delivered and the time course of recovery of the amplitude of the H-reflex was determined. Recovery of the H-reflex was increased in the normal control group aged 0 to 12 mos compared to that in the normal control group aged 1 to 9 yrs, especially at interstimulus intervals from 100 to 800 msec. In children with spasticity, marked recoveries of the H-reflex were observed in both age groups, 0 to 12 mos and 1 to 9 yrs, compared to those in normal controls. In the cases of athetosis, recovery of the H-reflex was also marked. In children with ataxia, it was not pronounced and similar to that in normal controls. From these findings, an H-reflex study was considered to be useful for evaluation of central nervous system function in childhood.

Growth suppression in children receiving acetazolamide with antiepileptic drugs.

To clarify the effect of the clinical dosage of acetazolamide on growth in children with epilepsy or febrile convulsion, the standard scores of height and weight in 17 subjects receiving acetazolamide as an adjunct to unchanged monotherapy of antiepileptic drug were compared longitudinally through four phases: before antiepileptic drug administration, with monotherapy of antiepileptic drug, with acetazolamide in addition to monotherapy, and after acetazolamide discontinuation. The standard scores of both height and weight in the subjects were significantly reduced during the phase of acetazolamide administration. During this period, serum concentrations of potassium and total CO2 decreased while that of chloride increased, suggesting the existence of metabolic acidosis in the subjects. For both height and weight, there was no correlation between the degree of standard score reduction during acetazolamide administration and age at the time of acetazolamide initiation, duration of acetazolamide administration, dosages of acetazolamide, and variety of antiepileptic drugs concomitantly administered with acetazolamide. We speculate that metabolic acidosis induced by acetazolamide suppressed the growth of the subjects and that there were large individual differences in the susceptibility to acetazolamide for growth suppression among patients receiving acetazolamide.

Antiepileptic effects of Clobazam in children

Many benzodiazepines used as anticonvulsants have nitrogen radicals in positions 1 and 4. Clobazam has nitrogen radicals in positions 1 and 5. We studied the antiepileptic effect of clobazam in 36 patients with intractable epilepsies in childhood. Their ages were 1 year 1 month to 16 years 5 months (mean 8 years). The mean initial dose was 0.33 mg/kg of daily doses and increased up to 0.71 mg/kg. Nine cases (primary generalized epilepsy 2/2, secondary generalized epilepsy 7/29) were completely free from seizures, and another 9 (secondary generalized epilepsy 8/29, partial epilepsy 1/5) experienced a decrease of 50% or more in seizure frequency. Seizure frequency did not change in 16, and increased in the other 2 (secondary generalized epilepsy 2/29). The antiepileptic effects were observed on the first day to 10th day after clobazam treatment. There were recurrences of seizures in 4 out of 9 patients with complete control of seizures, 1 month alter in 3 and 10 months later in one. Mean serum clobazam level of 7 improved cases was 73 ng/ml and that of 3 cases with no response was 94 ng/ml. Although mild clinical side effects such as drowsiness were observed transiently in 17 cases, no abnormality was found in laboratory investigations performed.

The Grasp Reflex and Moro Reflex in Infants: Hierarchy of Primitive Reflex Responses

The plantar grasp reflex is of great clinical significance, especially in terms of the detection of spasticity. The palmar grasp reflex also has diagnostic significance. This grasp reflex of the hands and feet is mediated by a spinal reflex mechanism, which appears to be under the regulatory control of nonprimary motor areas through the spinal interneurons. This reflex in human infants can be regarded as a rudiment of phylogenetic function. The absence of the Moro reflex during the neonatal period and early infancy is highly diagnostic, indicating a variety of compromised conditions. The center of the reflex is probably in the lower region of the pons to the medulla. The phylogenetic meaning of the reflex remains unclear. However, the hierarchical interrelation among these primitive reflexes seems to be essential for the arboreal life of monkey newborns, and the possible role of the Moro reflex in these newborns was discussed in relation to the interrelationship.