Biomedical subjects
Publications and source records attributed to K Metrakos.
We report two infants with near miss sudden infant death syndrome events exhibiting seizure disorders after caffeine treatment, suggesting there is an infant subgroup diagnosed as near miss sudden infant death syndrome who have apnoea possibly with seizures whose seizure threshold may be lowered by central nervous system stimulants like caffeine.
The clinical and EEG findings were reviewed for 270 juvenile children from the Montreal Children's Hospital Diabetic Clinic in an attempt to correlate the EEG findings at the onset of diabetes mellitus with the future risk of having a convulsion with a hypoglycemic reaction. Compared to a non-diabetic control population, the number of epileptiform EEG abnormalities was significantly increased in the initial EEG of diabetic patients who later had a hypoglycemic convulsion. The initial epileptiform EEG did not help to differentiate those diabetics with recurrent hypoglycemic induced convulsions from diabetics who would have only a single convulsion.
Sixty-five children with seizure disorders, who had been treated with multiple anticonvulsants but were poorly controlled, were selected from the Montreal Children's Hospital Convulsive Disorder Clinic and Neurology Service and were treated with sodium valproate (valproate). All types of seizure disorders were included in the group. Rapid oral absorption of the drug lead to peak plasma levels in one to three hours (later peaks occurring if administered after meals). A mean plasma half-life of 12.8 hours was calculated. Correlation between oral dose and plasma levels was poor. The side effects which occurred in this study were trivial. Drug interactions occurred with phenobarbital, diphenylhydantoin and clonazepam. Sixty percent of patients had a greater than 50% reduction in seizure frequency with sodium valproate, but the best response was in generalized absence seizures. Since all types of seizures responded to some degree, a trial of sodium valproate is warranted in intractable seizure disorders of childhood regardless of classification.
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Fourty-four patients received two doses of 12.5 mg/kg of hydrocortisone or placebo on the first day of life in attempted therapy for respiratory distress syndrome. Follow-up studies were performed on survivors at 5 years of age in ten steroid-treated and seven placebo-treated respiratory distress syndrome subjects. There were no significant differences in growth, intelligence tests, or neurologic examinations in the patients assessed. Abnormal EEGs are present in both groups. Immunologic tests showed no differences in lymphocyte counts, immunoglobulin levels, diphtheria and tetanus antibody titers, or complement components. Diminished percentages of T lymphocytes were found in steroid patients (53%) compared to control subjects (69%). There were also increased percentages of lymphocytes with C3 receptors in steroid patients (20.1%) compared to control patients (13.8%). Episodes of otitis and/or pneumonia were documented in eight of 11 steroid-treated patients between the ages of 1 and 5 years, compared to two of seven patients in the placebo group in the same time period. It is concluded that large doses of steroids on the first day of life may induce lasting immunologic abnormalities and may predispose to an increased incidence of infections.
Plasma levels of 14 amino acids were determined in 44 probands with 3/sec spike-wave epilepsy, 27 of their first-degree relatives, and 22 controls. Six ratios of metabolically related amino acids were also calculated. Statistically significant differences were found for 7/20 variables when the experimental and control probands were compared, and for 6/20 variables when the relatives were compared with controls. Tau, Asp, and Tau/Glu were decreased and Glu was was increased in both the experimental probands and their first degree relatives. When discriminant analysis was used, age- and sex-matched epileptic probands could be distinguished from the age- and sex-matched control probands with 100% accuracy employing only 3 amino acids (Asp, Glu, Val) and one ratio (Thr/Ser).
Growth, neurologic, and ophthalmologic assessments were done in 21 low-birth-weight infants given caffeine for neonatal apnea and in 21 matched control infants. Caffeine significantly decreased the need for and the duration of mechanical ventilation. No difference in growth and development was noted between the control and caffeine-treated infants. A high incidence of cicatricial retrolental fibroplasia was observed in both control (10/21) and caffeine-treated infants (7/18).
In a 10-year follow-up study electroencephalograms (EEGs) of 31 hyperactive and 27 matched control subjects of mean ages 19.17 and 18.59 years respectively showed no significant differences in any of the features assessed. Sequential EEGs, available for only the hyperactive subjects, suggested that a much greater proportion were normal at the 10-year follow-up assessment than at the 5-year follow-up assessment and that the normalization tended to take place mainly in the second 5-year period. This supports the hypothesis that EEG abnormalities of hyperactive persons are those of an immature pattern that tends to normalize with age. Correlation between EEG findings at the 10-year follow-up assessment and global outcome measures was not significant. Initial and 5-year EEGs also failed to predict global outcome at the 10-year follow-up assessment.
Four adolescents had basilar migraine, infrequent cerebral seizures, and severe EEG abnormalities. The clinical course was benign, with normal personality, mentation, and neurologic examination. Almost continuous EEG abnormalities, consisting of rhythmic temporo-occipital sharp and slow wave discharges, or generalized spike and wave complexes, were seen in serial recordings. The rare seizures, either focal or generalized, usually followed a migrainous aura and seemed to be readily controlled with anticonvulsant medication. The complex relationship between classical migraine and epilepsy is illustrated by this syndrome; recognition of its relatively benign course may prevent unnecessary investigation and undue alarm.
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Two families with benign familial neonatal seizures evident over three generations are presented with emphasis on clinical and electroencephalographic features. Benign familial neonatal seizures represent a disorder with the onset of frequent generalized seizures during the first weeks of life. Family history reveals autosomal dominant inheritance of this disorder. The neonates are neurologically normal and outcomes are usually benign. In our families, none of the patients had seizures after the first ten months of life, with long-term follow-up ranging from 10 months to 56 years. The use of antiepileptic medication seems to have little effect on course or eventual outcome. The electroencephalograms, both initially and subsequently, ranged from normal to epileptiform and did not correlate with risk for further seizures in infancy or subsequent epilepsy. Benign familial neonatal seizures are a distinct clinical entity whose key features and favorable prognosis should be appreciated by all those caring for sick neonates. This recognition would result in an early diagnosis and conservative treatment.