Metabolic encephalopathy structural

The metabolic encephalopathies comprise a series of neurological disorders not caused by primary structural abnormalities rather, they result from systemic illness, such as diabetes, liver disease and renal failure. Metabolic encephalopathies usually develop acutely or subacutely and are reversible if the systemic disorder is treated. If left... 

Asterixis, a jerky relaxation of tonically contracted postural muscles, was observed in some patients with structural lesions of the brain or metabolic encephalopathy who were taking levodopa, but not in patients with Parkinson s disease (10). 

Odell,G.B.K. and Schutta,H.S. (1985). Bilirubintnct dXopalAiy.laCembralEnergyMetabolism and Metabolic Encephalopathy. Ed., by McCandless, D.W., Plenum, NY, USA Onishi, S., Itoh, S., and Isobe, K. (1986). Wavelength dependence of the relative rate constants for the main geometric and structural photoisomeiization of hUirubin IXa bound to human serum albumin. Biochem. J. 236 23-29... 

One example of reversible metabohc encephalopathy is that seen in thiamine deficiency. Both animal models and humans with pure thiamine deficiency develop highly specific neurological symptoms. These symptoms can be reversed completely, or dramatically improved, often within hours, by the administration of thiamine. These results are instrumental in leading to the concept of metabolic encephalopathy , a disorder without structural brain changes. From a historical standpoint, there are increasing numbers of such disorders, which are amenable to successful treatment. Sadly, for example, in the case of kemicterus, managed health care has led to an increase in the number of cases due to the early release from hospitals of newborn infants after birth, even before the onset of jaundice. 

In summary, this book on metabohc encephalopathies is meant to combine and correlate animal and human studies. It is hoped that increased awareness of the importance of early diagnosis and treatment of these disorders may result in a lowering of the incidence of structural changes, and morbidity. These disorders hold a special fascination for both basic scientists and chnical investigators because they are accessible, treatable, and there exist good animal models for study. Therefore, this book pulls together basic and chnical neuroscience issues in the treatment of specific metabolic encephalopathies. 

Opsoclonus is a disorder of eye movement characterized by multidirectional saccades. Opsoclonus is usually associated with myoclonus, constituting OM, and there are often coexisting signs of cerebellar dysfunction and encephalopathy. The causation of OM is complex, including viral, metabolic, toxic, and structural disorders. Paraneoplastic OM was first described in children. Neuroblastoma is found in 50% of the pediatric OM cases, but on the whole, only 2-3% of all children with neuroblastic tumors have paraneoplastic OM [74, 75]. 

Biochemical disease involves a departure from the normal in some body component, e.g. an enzyme, a structural protein or a membrane transport mechanism. Some biochemical diseases are almost entirely environmental in origin, e.g. lead encephalopathy, but at present attention is largely directed to genetically determined conditions—the inborn errors of metabolism and other molecular diseases. In some cases it is the interaction between genotype and external environment which brings about the signs and symptoms, in others, such as the gangliosidoses, no modification of the external environment can appreciably alter the course of the disease. 

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