Myelopathy with severe structural derangement associated with combined modality therapy.

Myelopathy is a known complication of radiation therapy, but its association with chemotherapy has been less well documented. The authors report the case of a 12-year-old boy with medulloblastoma who had been followed for an 8 1/2-year period. In the context of accepted doses of intrathecal methotrexate and spinal irradiation, he developed an extensive myelopathy. At postmortem he was found to have profound fibrosis of the leptomeninges and necrosis with structural derangement of the spinal cord. It is speculated that these findings are secondary to a combination of radiation acting in synergism with methotrexate. The pertinent literature relating to myelopathy associated with radiation and chemotherapy is reviewed.

Sphenoethmoidal encephalocele: a case report with review of the literature.

A rare case of sphenoethmoidal encephalocele with detailed pathological studies is reported. Only a few cases have been documented and were reviewed. The etiology and embryology of the congenital malformation remains speculative with an agreement on their development at an early embryonic stage. The mass presenting in the nasopharyngeal cavity remains often undetected unless causing respiratory embarrassment and is frequently misdiagnosed. Thorough radiological investigations detect the bone defect and outline the extent of the herniated mass and associated cerebral abnormalities. Small lesions have been successfully repaired. Larger ones prove less amenable to surgery and carry a high mortality.

Brain changes in newborns, infants and children with congenital heart disease in association with cardiac surgery. Additional observations.

The histopathologic pattern in the Central Nervous System in 12 out of 21 new-borns and young infants, below 2 months of age, operated for various congenital heart defects, is that of recent necroses in the deep and subcortical white matter of the pallium, seen from a few hours to 8 days following completion of surgery. Type and location of these lesions of ischemic coagulative necrosis, resembling findings in the perinatal respiratory distress syndrome, seem to depend on shock-like states, the low output syndrome of the left heart with hypovolemia and hypotension. The cause of death in all these infants was cardio-respiratory failure. The question to what extent glial scars replacing necroses within the mostly non-myelinated fiber systems of the pallium will lead to defective maturation of the brain in later infancy and childhood remains a challenge for neurologic and psychological investigation, even if the heart defect has been successfully repaired by the surgeon. Post-mortem data of the CNS in 24 older infants and children below 9 years of age in association with cardia surgery include thrombembolic infarctions, also, after catheterization alone, diffuse or segmental cortical necroses, and cortical scars, especially in children older than 1 year, confirming data recently reported from this laboratory.