Pinar Ozisik

Medulloblastoma in a child with the metabolic disease L-2-hydroxyglutaric aciduria

L-2-Hydroxyglutaric aciduria (LHGA) is a rare autosomal recessively inherited neurodegenerative disorder. It is characterized by psychomotor retardation, progressive ataxia and typical magnetic resonance imaging findings, and presents in early infancy. On the other hand, medulloblastomas are very common solid tumors of childhood and infancy. We present a 3-year-old boy with LHGA who developed a medulloblastoma during the course of the disease. There has been no previous report of the coexistence of medulloblastomas with LHGA. Central nervous system tumors are encountered in children with other metabolic neurodegenerative disorders. The aim of this paper is to focus on the difficulties in the diagnosis and treatment of an intracranial tumor in a child already neurologically impaired due to metabolic neurodegenerative disease.

Prolonged cerebral salt wasting syndrome associated with the intraventricular dissemination of brain tumors. Report of two cases and review of the literature

Hyponatremia is a frequent event in neurosurgery practice and is usually associated with subarachnoid hemorrhage, head trauma, infections and neoplasms. The two common clinical manifestations are the inappropriate secretion of antidiuretic hormone (SIADH) and the cerebral salt wasting syndrome (CSWS), which were usually attributed to each other due to identical clinical presentation. In contrast to the better-recognized SIADH, there has not been a uniform consensus over the humoral and neural mechanisms of CSWS and functional aspects of renal response. In this article, we report on 2 cases of a primitive neuroectodermal tumor with prolonged CSWS manifested during the intraventricular dissemination of primary disease and the high catabolic stage.

Systemic administration of interleukin-10 attenuates early ischemic response following spinal cord ischemia reperfusion injury in rats.

BACKGROUND The aim of this experimental study was to investigate the early effects of interleukin-10 (IL-10) and interleukin-1beta antagonist (anti-IL-1beta) against cellular damage, inflammatory reactivity, lipid peroxidation (LPO), and myeloperoxidase (MPO) activity induced by spinal cord ischemia reperfusion injury (IRI). METHODS Thirty-two single strain female Albino rats were divided into four groups: control (sham-operated), IRI-alone, IL-10-treated (100 mug/kg), and anti-IL-1beta-treated (1 mg/kg) groups after IRI. IRI was induced by balloon occlusion of the aorta and simultaneous hypovolemia during occlusion. The animals were sacrificed at 24 h. Histopathological and ultrastructural analyses, biochemical studies for determination of LPO and MPO activity and Comet assays (single cell electrophoresis for detecting DNA single strand breaks) were performed in all study groups. RESULTS Compared with the levels of control (sham-operated) animals, IRI produced a significant increase in the levels of LPO and MPO activity, and prominent tissue damage characterized by leukocyte infiltration, edema and neuronal and glial damage in the affected spinal cord in 24 h. The administration of IL-10 decreased LPO and MPO activity, and suppressed initial inflammatory response in the first 24 h. The effects of anti-IL-1beta were limited to decrease in LPO activity without considerable evidence of cellular preservation. CONCLUSIONS These data suggest that systemic administration of IL-10 attenuates the early ischemic response, and may restrict the tissue damage in the first 24 h after spinal cord ischemia reperfusion injury. Anti-IL-1beta has no considerable effect in this time window. The results of this preliminary study promote further studies with longer time windows on the effects of anti-inflammatory cytokines in spinal cord IRI.

Dural sinus thrombosis after cardiopulmonary bypass

Cerebral venous thrombosis in various clinical conditions, such as congenital heart disease (CHD) and hyper-coagulable states, have been recognized previously. However, dural sinus thrombosis in a pediatric patient with Antiphospholipid Syndrome (APLS) after the repair of a ventricular septal defect (VSD) has not been reported yet. A child who underwent an operation for the surgical repair of VSD under cardiopulmonary bypass (CPB) developed a stroke after the procedure. A cranial computer tomography (CT) scan of the patient showed dural sinus thrombosis and severe cerebral edema. APLS and CPB were thought to be the only risk factors of dural sinus thrombosis. This observation warrants attention and screening should be taken into consideration in patients with APLS who carry a higher risk for dural sinus thrombosis after CPB.