Source:Pediatric Neurology
Author(s): Fatema Al Amrani, Roy Dudley, Luis E. Bello-Espinosa, Bernard Rosenblatt, Myriam Srour, Guillaume Sébire
ObjectiveThe observation of the dramatic response to intravenous immunoglobulin (IVIG) of a child from our center with intractable epilepsy (IE) due to focal cortical dysplasia (FCD) prompted us to perform a meta-analysis on the efficacy of IVIG in this condition.BackgroundFCD is a common cause of IE. Microglial activation and up-regulation of neuroinflammatory pathways have been documented in brain specimen from surgically treated IE/FCD patients. IVIG was used for decades to treat patients with IE, however there is no evidence regarding its efficacy, possibly due to the pathophysiological heterogeneity of patients included in most of the previous studies.Design/MethodsA search for studies in patients from 0-18 years was performed in databases. We found 4 observational studies - prospective or retrospective - including FCD patients with IE treated by IVIG. The primary outcome was reduction of seizure frequency by more than 50%.ResultsA total of 8 patients were included in this meta-analysis. The IVIG doses ranged from 0.2 to 1 g/kg/day repeated from three to six times over 1 to 14 months (median: 5 months). IVIG was associated with reduced seizure frequency in six out of eight patients (p<0.05). Among these six patients, the reduction of seizure frequency lasted for 9 months to 9 years (median: 3.7 years). There were either no or mild adverse effects of IVIG infusion including post-infusion paresthesia (n=1), and transient increase in temperature (n=1).ConclusionsDespite obvious limitations, mainly due to small number of patients, and to selection biases, this study suggests that based on the available data IVIG might be effective in the treatment of IE secondary to FCD. Further therapeutic trials are mandatory to further clarify the efficacy of IVIG in this condition.
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