Biochemical neurological disease

Chapter 6 Biochemical Neurological Disease in Children. L, L Woolf. 

D2. Delmotte, R, and Gonsette, R., Biochemical findings in multiple sclerosis. IV. Isoelectric focusing of the CSF gamma globulins in multiple sclerosis (262 cases) and other neurological diseases (272 Cases). J. Neurol. 215, 27-37 (1977). 

Stourac, R, and Bednafova, J., Intrathecal, antiviral and oligoclonal IgG synthesis in multiple sclerosis and its significance in differential diagnosis of neurological diseases. Clin. Biochem. Metab. 8(29), 204-208 (2000). 

Addison disease only Asymptomatic Any Adrenocortical dysfunction with no neurological disease Biochemical defect with no symptoms... 

In addition to the attempts described below to interfere with the onset or progression of neurological disease, it is also important to carry out endocrine testing on all biochemically identified males to assess their adrenocortical axis. In particular, all patients who have adrenal insufficiency require adrenal hormone replacement to prevent life-threatening complications of insufficiency. 

Steinlein OK, Bertrand D (2008) Neuronal nicotinic acetylcholine receptors from the genetic analysis to neurological diseases. Biochem Pharmacol 76 1175-1183... 

Neurons are more sensitive to OP-induccd biochemical damage or cytotoxicity than are astroglia and oligodendroglia, but developmental stages in the context of fetal base.s for adult neurological disease need to be fully considered. 

Wernicke Korsakoff syndrome (WKS) is the neurological disorder most clearly linked to thiamine deficiency in humans. WK develops in a subset of chronic alcoholics, who are vitamin deficient because so many calories are consumed as alcohol instead of normal diet, and a diet rich in carbohydrates increases the metabolic demand for thiamine. Thiamine dependent enzymes were diminished in the brains of patients who died with WKS, but not in alcoholic controls (Butterworth et al., 1993). Transketolase in fibroblasts from those patients who develop WKS syndrome binds TPP more avidly than the control lines. The Km was nearly ten times higher in patients with WKS. Thus, these patients have an abnormahty of transketolase that would be clinically unimportant if the diet was adequate (Blass and Gibson, 1977, 1979). The latter demonstrate a predisposing biochemical mutation to a neurological diseases that is only revealed by inadequate diet. 

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