Acute disseminated encephalomyelitis in a child following plasmodium vivax malaria

Acute Disseminated Encephalomyelitis [ADEM] is a multifocal, monophasic, acute demyelinating disease of the brain and spinal cord, which is commonly preceded by viral infections and occasionally bacterial infections or immunizations. Its occurrence following malarial infection, especially Plasmodium vivax Malaria is very uncommon. We report an 11-year girl who presented with clinical features of encephalopathy and generalized convulsions, 10 days following complete recovery from the Plasmodium vivax Malaria. Diagnosis of ADEM as a complication of Plasmodium vivax Malaria was made based on acute onset of neurological events, characteristic findings on Magnetic Resonance Imaging [MRI] of brain and prompt response to corticosteroid therapy. Follow-up MRI, 6 months after discharge, showed complete resolution of change found on the initial MRI. To the best of our knowledge, only two such cases have been reported in the English literature till date

Etiology, clinical spectrum and outcome of metabolic liver diseases in children

To determine the etiology, clinical spectrum and outcome of metabolic liver diseases [MLD] in children admitted in a tertiary care hospital of Eastern India. An observational study. Paediatric Liver Clinic and Paediatrics Inpatient Department of Nilratan Sircar Medical College and Hospital, Kolkata, Eastern India, from April 2009 to March 2011. All children aged 0 - 12 years having characteristic clinical features along with diagnostic hallmark of any MLDs were included in this study and data were collected on a pre-designed proforma. After appropriate management and discharge, all patients were followed-up for next 6 months. Fifty one children with mean age 4.34 +/- 3.78 years [range 2 days - 12 years], male: female ratio 1.55:1, were studied. The etiologies were Wilson's disease [33.33%, n = 17]; glycogen storage disorder [23.53%, n = 12]; galactosemia [19.61%, n = 10]; non-alcoholic fatty liver disease [11.76%, n = 6]; Gaucher disease [5.88%, n = 3]; mucopolysaccharidoses [3.92%, n = 2] and familial hyperlipoproteinemia type-I [1.96%, n = 1]. Jaundice [n = 24] and hepatomegaly [n = 47], was the commonest symptom and sign respectively. Of the 17 non-responders, most were Wilson's disease [n = 7] cases. There was statistical difference in outcome with respect to INR > 1.3 at diagnosis [p = 0.026]. High index of suspicion, early detection and screening, simple dietary modification and cost effective drugs along with good compliance are sufficient to treat and even prevent evolution of most causes of the MLDs