Osmotic Myelinolysis with Malignant Cerebellar Edema Occurring after DDAVP-Induced Hyponatremia in a Child.
Journal - Pediatric neurosurgery (Switzerland )
Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are dire neurological disorders, characterized by severe damage to the myelin sheath of neurons, which typically result from rapid correction or overcorrection of systemic hyponatremia. For many years, both conditions have been considered universally fatal, though survivors have been reported more recently. Pediatric cases are rare. We present a 13-year-old boy with panhypopituitarism secondary to repair of a nasofrontal encephalocele in infancy, managed on long-term corticosteroid, deamino arginine vasopressin and thyroid hormone. He presented with severe hyponatremia (116 mEq/l), which during correction rapidly and unexpectedly increased to 176 mEq/l, resulting in profoundly impaired consciousness. Brain imaging revealed multiple bilateral changes in the basal ganglia, thalamus, pons and cerebral white matter, consistent with both CPM and EPM. Malignant cerebellar edema necessitated emergent suboccipital craniectomy, with subsequent improvement in level of consciousness and imaging postoperatively. However, he succumbed to acute cardiorespiratory arrest 8 weeks later. Nine similar cases from the literature are reviewed.Copyright © 2010 S. Karger AG, Basel.
Effects of surgical excision and radiation on medulloblastoma cell invasiveness.
Journal - The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques (Canada )
OBJECTIVES: Surgical resection and adjuvant radiation are mainstays of medulloblastoma (MB) patient management. We utilized a novel 3-dimensional assay to identify how (a) radiation, (b) excision of the primary tumour aggregate, and (c) both treatments combined influence MB cell invasiveness. METHODS: Five MB cell lines (UW228-1, 2 and 3; Daoy, and Madsen) were implanted onto a 3-dimensional, type I collagen gel assay to assess tumour invasion distance over five days, in response to (1) needle-assisted excision of the central cell aggregate; (2) pre-exposure to single-dose and fractionated dose irradiation in doses from 6-25 and 8-24 Gy, respectively; and (3) excision plus either single-dose or fractionated radiation. RESULTS: Within hours, individual MB cells detached from the surface of the cell aggregates and invaded the collagen matrix, to distances up to 1200 microm and at rates up to 300 microm daily. The UW228-1 cell line was less invasive than the other cell lines and was dropped from further analysis. In the four remaining lines, a dose-dependent decline in tumour invasiveness was identified, both for single-dose and fractionated radiation, which achieved statistically decreased invasion distances at higher doses, especially of fractionated irradiation. Excision of the central tumour aggregate tended towards exerting a late effect on cell invasion, but exerted no significant influence on the radio-sensitivity of residual cells. CONCLUSIONS: Both single-dose and fractionated dose irradiation appear to inhibit MB cell invasiveness in a dose-dependent manner, whereas excision of the central cell aggregate exerts no effect on residual invading cells.
|ISSN : ||0317-1671|
|Mesh Heading : ||Cell Line, Tumor Cell Survival Cellular Structures Dose-Response Relationship, Radiation Extracellular Matrix Humans Medulloblastoma Neoplasm Invasiveness Neoplasm Transplantation Radiation Tolerance Time Factors pathology metabolism pathology radiation effects pathology methods|
|Mesh Heading Relevant : ||Models, Biological surgery therapy pathology|
Do intracranial neoplasms differ in Ollier disease and maffucci syndrome? An in-depth analysis of the literature.
Journal - Neurosurgery (United States )
OBJECTIVE: Ollier disease (OD) and Maffucci syndrome are closely related, very rare syndromes, that are both associated with multiple sites of chondrodysplasia. They differ primarily with the additional association of Maffucci syndrome with vascular abnormalities, including hemangiomas. Both are associated with chondrosarcomas and other nonsarcomatous neoplasms (NSN), but Maffucci syndrome is thought to have greater malignancy potential. We examined whether OD and Maffucci syndrome differ in the number, histology, in the location of intracranial malignancies, and in the demographics of such patients, as reported in the medical literature. METHODS: Relevant cases were identified by electronic searches on PubMed, SciSearch, Scientific Commons, Springer Link, and Google. Translate DotNet and Babelfish were used to translate non-English text. Unpaired Student's t tests were used to compare OD and Maffucci syndrome, and chondrosarcoma and NSN patients for mean age; Pearson chi analysis was used for comparisons of gender distribution, geographical distribution (by continent), site of lesion, and for OD versus Maffucci syndrome, tumor type (chondrosarcoma versus NSN). RESULTS: Forty-six patients with 47 intracranial malignancies were identified: 24 with OD, including 6 with a chondrosarcoma and 18 with an NSN; and 22 with Maffucci syndrome, including 13 with a chondrosarcoma, 8 with an NSN, and 1 with both. The 2 syndromes were statistically different in the distribution of chondrosarcomas versus NSNs (P = 0.002). All chondrosarcomas originated at the base of the cranium. All 18 NSNs in OD were of glial cell origin, but only 5 of 9 NSNs in Maffucci syndrome were glial; other tumors included pituitary adenoma, olfactory neuroblastoma, malignant chordoma, and spindle cell hemangioendothelioma. Patients with OD were more than 10 years younger than their Maffucci syndrome counterparts (24.7 versus 34.9 years; P = 0.002), as were patients with OD and chondrosarcoma versus those with Maffucci syndrome and chondrosarcoma (24.7 versus 36.2 years; P = 0.035). The 2 syndromes did not differ in overall sex distribution. OD and Maffucci syndrome differed in geographical distribution, with 10 Maffucci syndrome but no OD patients with malignancy either in Asia (7 patients) or South America (3 patients). Among NSNs, OD and Maffucci syndrome did not differ as to site of lesion within the brain. CONCLUSION: OD and Maffucci syndrome differ with respect to the distribution of intracranial malignancies by histology, and geographical and age distribution of cases, with OD patients younger by approximately a decade, and Maffucci syndrome patients more likely to live in Asia or South America.
Bilateral Decompressive Craniectomy for Refractory Intracranial Hypertension in a Child with Severe ITP-Related Intracerebral Haemorrhage.
Journal - Pediatric neurosurgery
We report a 13-month-old infant who developed acutely elevated intracranial pressure (ICP) as a result of a spontaneous intracerebral haemorrhage (ICH), secondary to idiopathic thrombocytopenic purpura (ITP). Her ICP remained severely elevated despite aggressive medical measures, with persistent obtundation, right hemiparesis and a dilated left pupil. Bilateral decompressive craniectomies (DCs) were performed, which resulted in a rapid decline in ICP. Ultimately, the patient regained consciousness and went on to complete neurological recovery. Tragically, she died of non-neurological, ITP-related complications 9 months later. In our review, we identified no other instances of bilateral DCs reported in the management of an infant with ITP and/or an ICH. We addressed three central questions: (1) Is there any value of DCs in children, and especially in infants, with elevated ICP? (2) Is there any value of DCs in the setting of non-traumatic ICH? And (3) is there any rationale for the use of bilateral versus unilateral DCs?Copyright © 2009 S. Karger AG, Basel.