myelin-basic-protein and Infant--Newborn--Diseases

Melatonin demonstrates neuroprotective properties in adult models of cerebral ischemia, acting as a potent antioxidant and anti-inflammatory agent. We investigated the effect of melatonin in a 7-d-old rat model of ischemia-reperfusion, leading to both cortical infarct and injury in the underlying white matter observed using MRI and immunohistochemistry. Melatonin was given i.p. as either a single dose before ischemia or a double-dose regimen, combining one before ischemia and one 24 h after reperfusion. At 48 h after injury, neither a significant reduction in cortical infarct volume nor a variation in the number of TUNEL- and nitrotyrosine-positive cells within the ipsilateral lesion was observed in melatonin-treated animals compared with controls. However, a decrease in the density of tomato lectin-positive cells after melatonin treatment was found in the white matter underlying cortical lesion. Furthermore, we showed a marked increase in the myelin basic protein-immunoreactivity in the cingulum and in the density of mature oligodendrocytes (APC-immunoreactive) in both the ipsilateral cingulum and external capsule. These results suggest that melatonin is not able to reduce cortical infarct volume in a neonatal stroke model but strongly reduces inflammation and promotes subsequent myelination in the white matter.

Topics: Animals; Brain Infarction; Humans; Immunohistochemistry; In Situ Nick-End Labeling; Infant, Newborn; Infant, Newborn, Diseases; Magnetic Resonance Imaging; Melatonin; Myelin Basic Protein; Myelin Sheath; Myelitis; Neuroprotective Agents; Oligodendroglia; Plant Lectins; Rats; Reperfusion Injury

We studied the spongy myelinopathy of glycine encephalopathy in five patients by using specific antisera. The walls of the vacuoles were stained with the myelin basic protein but not with the myelin associated glycoprotein or the glial fibrillary acidic protein immunostains. The pattern suggested that the vacuoles originated in compact myelin and not from the adaxonal portion of the sheath or from glial processes. Ultrastructural study revealed myelin vacuoles resulting from intraperiod splitting, and there were unusual intranuclear and cytoplasmic inclusions in skeletal muscle in two cases. In addition to the action of glycine as an inhibitory neurotransmitter, structural alterations of myelin may be important in the pathogenesis of the neurologic disorder of glycine encephalopathy.

Topics: Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases, Metabolic; Female; Glial Fibrillary Acidic Protein; Glycine; Humans; Infant, Newborn; Infant, Newborn, Diseases; Male; Microscopy, Electron; Myelin Basic Protein; Myelin P0 Protein; Myelin Proteins; Myelin Sheath; Nerve Tissue Proteins; Vacuoles

GM1 ganglioside concentration was measured by radioassay technique in individual samples of lumbar cerebrospinal fluid from 20 neonatal and 17 older pediatric patients. The lumbar CSF GM1 ganglioside concentration of neonates (76.6 +/- 27.4 ng/ml) is greater than that of older infants and children (31.9 +/- 22.2 ng/ml). The lower range of GM1 ganglioside concentration of CSF from older pediatric patients is similar to the previously reported adult CSF values. The mean CSF GM1 ganglioside concentration in pediatric patients with active neurologic disease (53.1 +/- 30.0 ng/ml) is greater than that of children without central nervous system pathology. The temporal evolution and magnitude above baseline values of lumbar CSF GM1 ganglioside concentration in three neonates was correlated with the clinical status of these patients.

Topics: Adolescent; Child; Child, Preschool; Female; G(M1) Ganglioside; Gangliosides; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Male; Myelin Basic Protein; Radioimmunoassay