Recurrent disseminated vasculomyelinopathy.

The monosymptomatic (recurrent infantile hemiplegia) and the polysymptomatic forms of disseminated vasculomyelinopathy that follow various infections and antigenic challenge to the nervous system were seen in two cases. These cases emphasize the importance of vasculopathy as the initial and obligatory component of the postinfectious and postimmunization neurologic syndromes as well as the clinical and pathological variability of the secondary effects on the nervous system. Recurrent infantile hemiplegia occurred in the first patient. In the second patient, after two episodes of postinfectious myelinoclastic encephalopathy, concurrent acute hemorrhagic leukoencephalopathy and an acute Guillain-Barré syndrome following swine flu vaccination developed.

Progressive bulbar paresis in childhood.

We present a case of progressive bulbar paresis in a 2-year-old child, with appropriate autopsy findings. A review of previously reported cases and a comparison with more extensive literature in Werdnig-Hoffmann disease suggest that Fazio-Londe disease is not unique, but belongs in the spectrum of progressive lower motor neuron disease.

Sydenham Chorea: magnetic resonance imaging reveals permanent basal ganglia injury.

MRI of the brain of a 3-year-old boy performed 3 days after the onset of hemichorea (Sydenham Chorea) revealed abnormal signal and enlargement of the contralateral caudate and putamen. Follow-up imaging 40 months later showed a persistent cystic appearance of the caudate and putamen. This case is the first report of permanent MRI abnormalities after Sydenham Chorea.

Childhood giant axonal neuropathy. Case report and review of the literature.

Giant axonal neuropathy (GAN) is a rare autosomal recessive childhood disorder characterized by a peripheral neuropathy and features of central nervous system involvement. Typically seen are distal axonal swellings filled with 8-10 nm in diameter neurofilaments in central and peripheral axons, and intermediate filament collections in several other cell types. Many neurotoxins produce a morphologically similar neuropathy in humans and experimental animals. Defective nerve fiber energy metabolism has been postulated as a cause in these toxic neuropathies. It is possible that GAN represents an inborn error of metabolism of enzyme-linked sulfhydryl containing proteins, resulting in impaired production of energy necessary for the normal organization of intermediate filaments.

Birth asphyxia and the neonatal brain: what do we know and when do we know it?

In this article, the authors examine whether indicators commonly used to recognized birth asphyxia are specific to asphyxial states, and whether these allow recognition of a severity of asphyxia sufficient to pose a risk of irreversible brain injury. Characteristics recognizable within the first hours after birth are focused on because these characteristics will be of most use in clinical decisions regarding use of potential new therapies for asphyxia.

The natural history of night terrors.

Night terrors are a sleep disorder, resulting from a partial arousal during slow-wave sleep. They usually occur within 2 hours of sleep onset and are characterized by agitation and unresponsiveness to external stimuli. Nineteen children (ten males, nine females) with onset of night terrors before age 7.5 years were studied by means of a questionnaire. Mean observation time (time from onset age to age at survey) was 8.5 years, but longer than 10 years in nine subjects. Seventy percent of the children had their initial frequency of night terrors as their peak frequency, with a tendency for shorter duration of the parasomnia in this group. Children with onset age less than 3.5 years may be expected to attain a peak frequency of at least one episode per week. Children with onset after 3.5 years, but before 7.5 years, may expect to attain a peak frequency of 1-2 episodes per month. There was a mean duration of 3.9 years, with a tendency for longer duration in children with positive family histories of sleep walking. Fifty percent stopped by age 8 years; 36 percent continued into adolescence. No common abnormal behavioral profile or psychopathology was found. Common precipitants of attacks were not identified.

Mitochondrial hydroxyapatite deposits in spinocerebellar degeneration.

We report the presence of crystalline deposits of calcium hydroxyapatite in the mitochondria of 2 children with sporadic spinocerebellar degeneration. The deposits, identified by electron microscopy, were found in the mitochondria of neurons and smooth muscle cells in one patient and in only smooth muscle cells in the second child, but not in other cell types. The calcific nature of the deposits was confirmed by laser microprobe mass analysis. The calcium overload may interfere with mitochondrial function, as has been shown in the cardiomyopathic strain of the Syrian hamster, a model of the cardiomyopathy of Friedreich's ataxia.

Assessment of gestational age using birth certificate data compared with medical record data.

We assessed the extent to which use of medical record data might improve gestational age estimates compared with reliance on the birth certificate alone. Using population-based data from four northern Californian counties, we constructed an algorithm to select the best gestational age estimate from antenatal assessments recorded in medical records. A total of 172 singletons with moderate or severe congenital cerebral palsy from the California Cerebral Palsy Project were compared with 472 randomly selected controls with regard to discrepancies between the algorithm-derived estimated gestational age (bestgest) and an estimate based solely on the last menstrual period as recorded on birth certificates. Agreement between bestgest and birth certificate estimated gestational age was exact or within one week for at least 60% of both cases and controls in each of the three birthweight strata. In general, the greater the birthweight of the babies, the better the agreement. The mean number of weeks of overestimation by the birth certificate was 0.7 weeks for cases and 1.1 weeks for controls in the lowest birthweight group (< 1500 g). When compared with bestgest, clinical examination of the infant also tended to overestimate gestational age. In the < 1500 g birthweight group, cases were twice as likely as controls to have a bestgest of 'low certainty,' but antenatal estimates of 'high certainty' were obtained for at least a third of very low birthweight babies born during the mid-1980s. More widespread use of early ultrasound in more recent birth cohorts may result in a greater proportion of accurate antenatal estimates. When a distinction between immaturity and intrauterine growth retardation is important to the understanding of the aetiology of the outcome under investigation, the use of antenatal estimates from medical records may substantially improve the certainty of the data.

Prenatal and perinatal factors and cerebral palsy in very low birth weight infants.

OBJECTIVE: To identify prenatal and perinatal characteristics associated with cerebral palsy (CP) in infants born weighing < 1500 gm (very low birth weight, VLBW). DESIGN: All 42 VLBW singleton infants with CP born in the period from 1983 to 1985 in a defined population were compared with 75 randomly selected VLBW control infants. RESULTS: Birth in a level I facility was associated with increased risk of CP (odds ratio (OR) 6.3, 95% confidence interval (CI) 1.8, 19), as was birth within 3 hours of the mother's first admission for delivery (OR 3.2, CI 1.4, 7.4). Delivery occurred within 3 hours of admission to a level I facilty in 24% of VLBW children with CP and no control children (OR (0.5 added to each cell of 2 x 2 table) 49, CI 3.1, 204). Chorionitis was associated with increased risk in children born more than 5 hours after admission (OR 4.3, CI 1.1, 13). Chorionitis followed by neonatal seizures occurred in 14% of VLBW children with CP (in 25% with spastic diplegia) and in no control child (OR (0.5 added to each cell of 2 x 2 table) 26, CI 1.6, 116). Preeclampsia was associated with decreased risk (OR 0.08, CI 0.02, 0.67), as was use of magnesium sulfate (OR 0.14, CI 0.05, 0.51) administered for preeclampsia or preterm labor. Other risk factors for CP included gravidity greater than one (OR 3.9, CI 1.2, 11), short interbirth interval (OR 4.1, CI 1.3, 12), and vaginal bleeding on the day of admission (OR 2.9, CI 1.2, 7.4). CONCLUSIONS: In this population-based study, almost one fourth of the CP in VLBW children occurred in infants delivered in level I facilities soon after their mothers' admissions. Another 14% was in children who had neonatal seizures after birth to women with chorionitis. No control subject experienced either of these sequences.