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Severe Encephalopathy (severe + encephalopathy)

Distribution by Scientific Domains

Medical Sciences	80%

Selected Abstracts

Ictal Pattern of EEG and Muscular Activation in Symptomatic Infantile Spasms: A Videopolygraphic and Computer Analysis

EPILEPSIA, Issue 12 2002
Francesca Bisulli
Summary: ,Purpose: To investigate ictal muscular phenomena characterizing symptomatic infantile spasms (ISs) and their relation to ictal EEG. Methods: Four children with severe encephalopathy, neurologic impairment, and refractory ISs related to different dysplastic lesions, underwent videopolygraphic recordings collecting surface electromyogram (EMG) activity from several cranial and limb muscles to evaluate the pattern of muscular recruitment, duration, and side-to-side asymmetry of ISs. Acquired data were stored for off-line analysis by a computerized polygraphic system. Results: Spasms were characterized by a complex pattern of muscular activation. A constant or rostrocaudal propagation pattern was lacking in all patients. Intervals between the onset of EMG activity in different muscles in each spasm were very long: ,100,200 ms. Two patients, with hemispheric cortical dysplasia and agenesis of the corpus callosum, had asymmetric and asynchronous spasms in which the EMG onset of the muscles contralateral to the affected hemisphere constantly anticipated that of the ipsilateral ones. Backaveraging of EEG activity disclosed a high-amplitude EEG complex on the same side as the dysplastic lesion, preceding or succeeding the contralateral muscle activity onset. In the other two patients with diffuse cortical dysplasia, no EEG transient was related to EMG activity onset. Conclusions: Despite clinical similarity between spasms in the same patient, our data demonstrate the complexity and heterogeneity of these motor phenomena. Our findings cannot be explained simply in terms of cortical or reticular generators like other motor phenomena such as cortical myoclonus or startle reflex. [source]

Mechanism of the protective effect of hypothermia on ammonia toxicity in astrocytes

JOURNAL OF NEUROCHEMISTRY, Issue 2002
C. Zwingmann
Ammonia is a key factor in the pathogenesis of hepatic encephalopathy (HE). Acute ammonia treatment causes energy failure of astrocytes, which are able to compensate partly by increased anaerobic metabolism as a means of making up for the energetic shortfall. As hypothermia offers protection from severe encephalopathy and lactate accumulation in liver failure, we investigated the mechanism by which hypothermia protects against ammonia toxicity by multinuclear NMR spectroscopy. 12 h exposure to 5 mm NH4CL decreased the phosphocreatine (PCr)/creatine (Cr) and ATP/ADP ratios to 65 and 76% of control, increased synthesis and release of glutamine to 200,250% and led to a significant stimulation of glycolytic activity reflected by increased uptake and consumption of glucose and accumulation of de novo synthesized intra- and extracellular lactate to 161 and 230% of control. The protective effect of mild hypothermia was evident from inhibiton of lactate accumulation and restoration of ammonia-induced depletion of PCr/Cr. Moderate hypothermia led to an increase of PCr/Cr ratio and inhibited lactate synthesis to 14% of normothermic control, but did not prevent the ATP decrease. While hypothermia inhibited glycolytic flux, intracellular glutamine remained elevated. The results suggest that hypothermia-induced protection against ammonia toxicity results from reduction of cellular energy demand leading to inhibition of anaerobic glucose metabolism and a compensatory stimulation of mitochondrial energy production. Acknowledgements:, Funded by CIHR Canada. [source]

Encephalopathy after furosemide use in nephrotic syndrome

JOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 3 2007
Ajay P Sharma
Abstract: An 18-year-old girl with nephrotic syndrome presented with a life-threatening respiratory failure and a severe encephalopathy, shortly after being started on furosemide. The evaluation confirmed furosemide as the sole culprit for the clinical manifestations. This case highlights an unpredictable dose,response relationship of furosemide. The pathophysiological basis, differential diagnosis and clinical implications of the observed findings are discussed. [source]

Wernicke's encephalopathy in a malnourished surgical patient: clinical features and magnetic resonance imaging

ACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 10 2005
M. Nolli
We report a clinical and neuroradiological description of a severe case of Wernicke's encephalopathy in a surgical patient. After colonic surgery for neoplasm, he was treated for a long time with high glucose concentration total parenteral nutrition. In the early post-operative period, the patient showed severe encephalopathy with ataxia, ophthalmoplegia and consciousness disorders. We used magnetic resonance imaging (MRI) to confirm the clinical suspicion of Wernicke's encephalopathy. The radiological feature showed hyperintense lesions which were symmetrically distributed along the bulbo-pontine tegmentum, the tectum of the mid-brain, the periacqueductal grey substance, the hypothalamus and the medial periventricular parts of the thalamus. This progressed to typical Wernicke,Korsakoff syndrome with ataxia and memory and cognitive defects. Thiamine deficiency is a re-emerging problem in non-alcoholic patients and it may develop in surgical patients with risk factors such as malnutrition, prolonged vomiting and long-term high glucose concentration parenteral nutrition. [source]

A new case of pyruvate dehydrogenase deficiency due to a novel mutation in the pdx1 gene

ANNALS OF NEUROLOGY, Issue 2 2003
Runu Dey PhD
We report a case of neonatal congenital lactic acidosis associated with pyruvate dehydrogenase E3-binding protein deficiency in a newborn girl. She had a severe encephalopathy, and magnetic resonance imaging of the brain showed large subependymal cysts and no basal ganglia lesions. She died 35 days after birth. We detected a novel homozygous deletion (620delC) in the PDX1 gene, which encodes for the E3BP subunit of the pyruvate dehydrogenase complex. [source]