1593 Cerebral Spinal Fluid (Csf) Protein as an Indication of Intraventricular Hemorrhage (Ivh)

Abstract

Eighteen preterm infants with severe progressive post-hemorrhagic hydrocephalus (PPH) following IVH were managed with serial lumbar punctures (LP) according to the following protocol. Daily serial LP with measurement of opening and closing pressure (OP,CP) were performed for no more than four weeks. Sufficient CSF was removed with each LP to lower OP by half. If OP became normal (5 80 mm H20) and remained so for two successive days, LP was deferred for 48 hours and then repeated. If OP remained 5 80 nun H20 LP management was discontinued and the patient observed. If hydrocephalus progressed after discontinuation of LP or symptoms of increased intracranial pressure developed during LP management? shunt was effected.Of the 18 infants thus far studied, 11 responded to LP management. OP in 10 of the 11 was 5 80 mm H20 by three weeks; none of the non-responders demonstrated OP 5 80 mm Hz0 prior to three weeks of therapy (p=0.0002). Initial CSF protein and sugar and response of CSF protein and sugar to LP were not predictive of response.In conclusion, attainment of OP of 2 80 nun H20 for 3successive days within a 21 day period of serial LP management is highly predictive of PPH response to LP management. Mannosidosis is noteworthy among lyaosomal storage diseases affecting the nervous system for having a low mortality and a stable clinical course. Comparative studies of oligosaccharides were carried out on serum and urine of a 31-year-old man with mannosidosis (Arch Neurol 34:45, 1977) whose neurologic status has been stable since the age of 8 years. A mannose-containing trisaccharide with two mannose and one N-acetyl glucosamine residues was predominant in urine (304 mg/l), in agreement with other reported cases. By use of a sensitive new high pressure chromatographic procedure, a very low level of this trisaccharide was detected in serum (0.1-0.4 nmol/ml), with respective urinary values from 161 to 558 nmol/ml. The urinary clearance of trisaccharide was on average 16.1 times the glomerular filtration rate of creatinine, suggesting that the renal tubular cell is the immediate source of urinary oligosaccharides. The substrate burden in brain is much less in mannoaidosis (0.11 umol/g; J Pediatr 75:360, 1969) than it is in fucoaidoais (19.9 umol/g; J Neurochem 27:733, 1976), despite the fact that both diseases reflect a failure to catabolize glycoproteins. Our data suggest that upon maturation of the kidney a mannoae receptor is elaborated which, by removing mannose-rich oligosaccharides from the circulation, favors the elimination of excess oligosaccharides and lowers the substrate burden for brain. The pathogenesis of Pelizaeua-Merzbacher (P-M) disease is unknown. A 17-year-old male, whose brother died at 12 years with pathological findings of classical P-M disease, had congenital nystagmus, ataxia, dementia, and progressive quadriparesis. Electronystagmographic testing showed prolonged latency and slow phase duration during rotation and caloric stimulation. These findings suggest ongoing involvement of myelinated fib...