The cognitive profile and CSF biomarkers in dementia with Lewy bodies and Parkinson's disease dementia.

OBJECTIVE: Dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD) may be viewed as different points on a continuum reflecting the regional burden and distribution of pathology. An important clinical consideration is overlapping Alzheimer's disease (AD) pathology, since it has been reported that associated AD pathology in DLB shortens survival and leads to a more rapid cognitive decline. We aimed to investigate cerebrospinal fluid (CSF) biomarkers and the associated cognitive profile in DLB and PDD. METHODS: Clinically diagnosed DLB (n=47) and PDD (n=17) patients from a clinical follow-up programme were investigated. All performed mini mental state examination (MMSE) and went through lumbar puncture at baseline. CSF concentrations of total τ (T-τ), τ phosphorylated at threonine 181 (P-τ(181) ) and the 42 amino acid isoform of amyloid ß, Aß42 were determined. RESULTS: We found significant differences in T-τ and Aß42, with a higher level of T-τ and a lower level of Aß42 in DLB compared to PDD. The combination of T-τ with Aß42 showed better discrimination between DLB and PDD than either of the measures alone. In DLB, a CSF profile more like the one seen in AD was significantly correlated with worse performance on the orientation and memory of the MMSE. CONCLUSION: The correlation suggests a possible link between a higher degree of AD pathology and a profile of more temporal disabilities on cognitive tests in DLB. This might aid in identifying a subgroup of patients with a greater burden of AD pathology in a clinical setting and could have important implications for prognosis.

Strain differences to effects of aging on concentrations of amino acids in cerebrospinal fluid between Sprague Dawley rat and Wistar Kyoto rat.

Age-related alterations and differences of weights and those of amino acid concentrations in the cerebrospinal fluid (CSF) were evaluated between Sprague Dawley (SD) rats and Wistar Kyoto (WKY) rats from eight to twenty weeks of age. The weights of SD rats were heavier than WKY rats at all ages. The age-related alterations of the CSF concentration of many amino acids within each strain were significant but showed no significant trend with age. Between the strains, the concentration differences of excitatory and inhibitory amino acids were not frequent although the concentrations of arginine, alanine and threonine were significantly higher in SD rats than in WKY rats. These results suggest that the different CSF concentrations of amino acids may relate to characteristics of rat strains.

Inhibitory and excitatory amino acids in cerebrospinal fluid of neurolathyrism patients, a highly prevalent motorneurone disease.

Neurolathyrism is caused by overconsumption of seeds containing 3-N-oxalyl-L-2,3-diaminopropanoic acid (beta-ODAP). Amino acids levels of cerebrospinal fluid (CSF) were studied in 50 patients with neurolathyrism and 12 healthy volunteers. The levels of excitatory amino acids glutamate and aspartate were 281% and 71% respectively of control values. The concentration of inhibitory amino acids glycine and taurine were 277% and 198% respectively of the levels in CSF from control individuals. There was a significant correlation between the level of glycine and the duration of the disease. We also found increased levels of threonine, serine and alanine. In contrast to reports on other motor neurone diseases where an increase of isoleucine was observed we found a significant decrease of isoleucine. The results suggest a disturbance of amino acid metabolism due to excitotoxic damages caused by beta-ODAP, a dietary excitatory amino acid.

Decrease of plasma sulfur amino acids in essential hypertension.

In order to evaluate the correlation between sulfur amino acids (derived mainly form animal protein in the diet) and blood pressure, free amino acids, including sulfur amino acids such as taurine and methionine, were determined in the plasma and cerebrospinal fluid (CSF) of twelve normotensive subjects and twelve patients with essential hypertension under nutritional control after at least 10 days of standard hospital diet (total calorie and protein content: 2100-2300 Cal per day and 78-83 g per day, respectively). The results obtained were as follows: plasma taurine, serine, methionine and threonine were significantly lower in patients with essential hypertension than in normotensive patients. The levels of plasma taurine, serine, methionine and total sulfur amino acids in individuals correlated inversely to systolic blood pressure. No difference was observed in the CSF levels of free amino acids in normotensive and hypertensive patients. As taurine, methionine and serine are involved in the metabolism of sulfur amino acids, these observations support the view that the decrease in plasma sulfur amino acids may be a factor contributing to elevated blood pressure.

Elevated levels of amino acids in the CSF of motor neuron disease patients.

Amino acid levels in the cerebrospinal fluid of patients with motor neuron disease were compared with an age-matched control group receiving diagnostic myelography. Five amino acids were significantly elevated in the cerebrospinal fluid of the motor neuron disease patients compared to the controls. These were isoleucine, glycine, alanine, phenylalanine, and threonine, which were increased by 60, 58, 38, 26, and 25% respectively. A significant increase was also obtained when the amino acids with nonpolar R groups were grouped together (34%). The significance of these findings is discussed in terms of amino acid changes that occur in both normal aging and other neurological conditions.

Plasma and cerebrospinal fluid amino acid concentrations in phenylketonuria during the newborn period.

Plasma and cerebrospinal fluid amino acid values were determined in 29 infants 9 to 30 days of age with a confirmed diagnosis of phenylketonuria. Phenylalanine concentrations in plasma and cerebrospinal fluid were markedly elevated; the degree of elevation in the cerebrospinal fluid had a significant relationship to that of the plasma. The only other significant deviations in the plasma were reductions in the threonine and tyrosine values. Cerebrospinal fluid threonine, alanine, and arginine concentrations were reduced, whereas those of serine, isoleucine, and histidine were elevated. This combined deficiency and excess of amino acids in the central nervous system may have a significant effect on protein synthesis at a time in life when this synthesis and turnover is most active.

[Normal range of free amino acids in cerebrospinal fluid of children (author's transl)]. / Verhalten der freien Aminosäuren im Liquor cerebrospinalis von Kindern

UNLABELLED: Free amino acids were determined quantitatively in cerebrospinal fluid of 80 boys and 69 girls (ages 1 month-14years). On the basis of the cliniclal criterions these children were considered to be ""healthy''. The total number of examined children was 2000. Cerebrospinal fluid was mixed with an equal volume of 5% sulphosalicylic acid and the amino acids were separated by ion exchange column chromatography. RESULTS: 1. The sexspecific investigation of the 1 month -2 years old children showed that the boys had a statistical significant hgher concentration of lysine and that no statistical significant differences existed between the groups of 2-14 years old children. 2. The age specific comparison revealed that the amino acid concentrations ofaspartic acid, citrulline, histidine, lysine, serine, threonine and tyrosine were in the cerebrospinal fluid of the younger boys statistical significant higher as compared to the older boys and that the concentration of serine in the cerebrospinal fluid of the younger girls was statistical significant higher than in the group of older girls.