Cause Of Acute Encephalopathy Research Articles

Chemokine profiling of Japanese encephalitis virus-infected mouse neuroblastoma cells by microarray and real-time RT-PCR: Implication in neuropathogenesis

Japanese encephalitis (JE) is one of the leading causes of acute encephalopathy affecting children and adolescents in the tropics. JE virus (JEV) infection causes prominent neurological sequelae in approximately one-third of the survivors. In humans, the inflammatory response of CNS consequent to JEV induced viral encephalitis is mediated through chemokines released by various cells of CNS. In the present study, the chemokine profiles of mouse neuroblastoma cells (N2A) following JEV infection was analyzed by cDNA microarray followed by real-time RT-PCR. Eighty mRNA transcripts belonging to various functional classes exhibited significant alterations in gene expression. There was considerable induction of genes involved in apoptosis and anti-viral response. Modified levels of several transcripts involved in proinflammatory and anti-inflammatory processes exemplified the balance between opposing forces during JEV pathogenesis. Other genes displaying altered transcription included those associated with host translation, cellular metabolism, cell cycle, signal transduction, transcriptional regulation, protein trafficking, neurotransmitters, neuron maturation, protein modulators, ER stress and cytoskeletal proteins. The infection of neurons results in the synthesis of proinflammatory chemokines, which are early important mediators of leukocyte recruitment to sites of viral infection. Our results clearly suggest the implication of chemokines in neuropathogenesis of JEV infection leading to neurological sequelae. Pro- and anti-inflammatory agents targeted against chemokines such as CXCL10 may provide possible therapeutic modalities that can mitigate the morbidity associated with JEV infection of the CNS.

Transient bilateral blindness and posterior reversible encephalopathy syndrome: A rare complication of enuresis treatment

Enuresis is a common paediatric problem which is sometimes treated with anticholinergic drugs. We report a 4-year-old girl who presented with acute bilateral blindness, a focal seizure and hypertension 10 days after commencing oxybutynin to treat enuresis. Magnetic resonance imaging brain showed features of posterior reversible encephalopathy syndrome, a recognised but rare complication of hypertension in children. Discontinuing the oxybutynin leads to complete neurological recovery associated with normalisation of her blood pressure. We believe this case represents a rare complication of anticholinergic therapy. Posterior reversible encephalopathy syndrome is a treatable and reversible cause of acute encephalopathy with blindness, as long as an early diagnosis is made.

Hashimoto’s encephalopathy in an adolescent boy

Hashimoto's encephalopathy is an under-recognized cause of acute encephalopathy both in children and adults. We hereby describe a 12.5 yr old boy with this rare disorder that presented with an acute onset of episodic psychosis with hallucinations along with seizures and had elevated antithyroid antibodies. Symptoms improved with thyroxine replacement and anticonvulsants and EEG normalized 3 mth into follow up. Hashimoto's encephalopathy should be considered in patients with unexplained encephalopathy and seizures, as prompt recognition and management can lead to an excellent outcome.

Epilepsias mioclónicas en la infancia

Malaria is one of the main health problems in the Third World. Plasmodium falciparum infects as many as 300 million people, causing up to three million deaths each year, most of which occur in African children. Cerebral malaria is the most common lethal complication of P. falciparum infection in children and is defined by three criteria: disturbances of consciousness, presence of P. falciparum parasitaemia and absence of other causes of acute encephalopathy. Cerebral malaria is a medical emergency and parenteral quinine is the most recommended treatment because of the frequency of chloroquine-resistant strains. Mortality is as high as 50 per cent and residual disability is present in about 20 per cent of survivors. We want to warm Spaniard neuropaediatricians about the existence of cases of cerebral malaria in our country in order to get a better diagnose and treatment for those children. A retrospective medical scores review of 20 hospitalised children diagnosed of malaria from 1990 to 1998. We selected three cases with neurological signs and we analysed clinical onset, EEG, neuroimaging, and permanent sequels. All patients had acute encephalopathy with fever, obtundation and seizures. They all presented residual disability (mainly hemiparesis). We must know better about cerebral malaria because of an increasing incidence of imported malaria due to emigration from African countries and Spaniard tourism to areas of endemic paludism.

Le acidure organiche

Organic acidurias are congenital metabolic disorders variously involving the metabolic pathways, mainly the catabolism of aminoacids and fatty acids with accumulation of one or more organic acids in blood or urine. This group of diseases includes over 50 specific forms which, although rare, are the most common cause of acute encephalopathy in infancy. Single or recurrent episodes of metabolic failure are the most frequent presenting symptom in infancy, often in the first weeks of life. Onset may also occur later with features of chronic encephalopathy and complex clinical features including seizures, dystonia, hypotonia and in some cases myopathies associated with liver and kidney failure. Early diagnosis of acute forms is important since some organic acidurias can be treated thus preventing often fatal episodes of metabolic failure or ongoing neurological damage. Definitive diagnosis requires a search for pathological metabolites in urine using gas chromatography and mass spectroscopy and confirmation of enzyme deficit. Neurological investigation will show features indicative of metabolic disease in acute forms and in children presenting with progressive encephalopathy. Neurological features are heterogeneous, but MR findings will offer information for subsequent biochemical tests and sometimes suggest diagnosis, especially signal changes in the basal nuclei and subcortical white matter.

Glutaric aciduria type I: A common cause of episodic encephalopathy and spastic paralysis in the Amish of Lancaster County, Pennsylvania

We have diagnosed type I glutaric aciduria (GA-I) in 14 children from 7 Old Order Amish families in Lancaster County, Pennsylvania. An otherwise rare disorder, GA-I appears to be a common cause of acute encephalopathy and cerebral palsy among the Amish. The natural history of the disease, which was previously unrecognized in this population, is remarkably variable and ranges from acute infantile encephalopathy and sudden death to static extrapyramidal cerebral palsy to normal adult. Ten patients first manifested the disease between 3 and 18 months at the time of an acute infectious illness. Four of these children died in early childhood, also during acute illnesses. However, there has been little progression of the neurological disease after age 5 years in the surviving children and intellect usually has been preserved, even in children with severe spastic paralysis. When well, patients have plasma glutaric acid concentrations ranging from 4.8 to 14.2 mumol/liter (nl 0-5.6 mumol/liter) and urinary glutaric acid concentrations from 12.5 to 196 mg/g creatinine (nl 0.5-8.4 mg/g creatinine). We have found that GA-I can be diagnosed in the Amish by measurement of urinary glutaric acid concentrations using isotope-dilution gas chromatography/mass spectrometry, whereas the diagnosis can easily be missed by routine urine organic acid gas chromatography.(ABSTRACT TRUNCATED AT 250 WORDS)

Aluminium and the Pathogenesis of Senile Plaques in Alzheimer's Disease, Down's Syndrome and Chronic Renal Dialysis

Click to increase image sizeClick to decrease image sizeKey Words: Alzheimer's diseasealuminiumsenile plaquesDown's syndromerenal dialysis