Steroids in Duchenne muscular dystrophy--deflazacort trial

We conducted a double blind controlled trial in 28 Duchenne muscular dystrophy (DMD) patients with Deflazacort (DF), an oxazoline derivative of prednisolone which reduces its side-effects. Myometric muscle strength measurements, Scott Score and timed tests showed statistically significant improvement for the treated group (P less than 0.05). Side-effects after 9 months of treatment included mild cushingoid appearance in four patients (28%) and moderate in only one (7%), increased appetite in seven (50%), increased body hair in four (28%), irritability and hyperactivity in three (21%). Increased body weight was not prominent and was controlled with dietary measures. No patient had to be withdrawn from medication. More research and long-term follow-up are needed in order to establish the mechanism of improvement and the consequences of long-term steroid administration in DMD. In this regard DF appears as an alternative to prednisone preserving its benefits but with fewer side-effects.     

Altered excitatory and inhibitory amino acid receptor binding in hippocampus of patients with temporal lobe epilepsy.

We examined binding to excitatory amino acid and inhibitory amino acid receptors in frozen hippocampal sections prepared from surgical specimens resected from 8 individuals with medically refractory temporal lobe epilepsy. The excitatory receptors studied included N-methyl-D-aspartate (NMDA), strychnine-insensitive glycine, phencyclidine, and quisqualate. The inhibitory receptors studied were gamma-aminobutyric acid type A (GABAA) and benzodiazepine. Excitatory and inhibitory amino acid receptor binding were differentially altered in the patients with temporal lobe epilepsy in comparison to 8 age-comparable autopsy control subjects, and changes in receptor binding were regionally selective in four areas. Binding to phencyclidine receptors associated with the NMDA channel was reduced by 35 to 70% in all regions in the hippocampi of the patients. In contrast, binding to the NMDA recognition site and its associated glycine modulatory site was elevated by 20 to 110% in the cornu ammonis (CA) 1 area and dentate gyrus of the hippocampus of the patients. Binding to these sites was unaffected in area CA4. Binding to the quisqualate-type excitatory amino acid receptor was unchanged in all regions except the stratum lacunosum moleculare CA1, where it was increased by 63%. GABAA and benzodiazepine receptor binding was reduced by 20 to 60% in CA1 and CA4, but unchanged in dentate gyrus. The data indicate that excitatory and inhibitory amino acid receptors are altered in the hippocampus of patients with temporal lobe epilepsy.     

Increased lymphocyte beta-adrenergic receptor density in progressive multiple sclerosis is specific for the CD8+, CD28- suppressor cell.

Beta-adrenergic receptor density on T cells from healthy humans is greatest on suppressor cells (CD8+, CD28-) and the effect of catecholamines, secreted by the sympathetic nervous system, predominates on this subset. The sympathetic skin response, a measure of sympathetic nervous system function, is absent in most patients with chronic progressive multiple sclerosis (MS). We measured beta-adrenergic receptor density on suppressor cells, cytotoxic cells, and monocytes from patients with chronic progressive MS and healthy control subjects. Control receptor density on suppressor cells was 2.8 +/- 0.3 fmol/10(6) cells versus a density of 5.1 +/- 0.7 fmol/10(6) cells for patients. Cytotoxic cell (CD8+, CD28+) receptor density was 1.4 +/- 0.4 fmol/10(6) cells in control subjects and 0.9 +/- 0.3 fmol/10(6) cells in the patients. Monocytes displayed beta-adrenergic receptor densities of 2.6 +/- 0.4 fmol/10(6) cells in normal individuals and 2.7 +/- 0.4 fmol/10(6) cells in the patient group. CD8 lymphocyte beta-adrenergic receptor densities in patients with relapsing-remitting and those with stable MS were not different from control values, yet were significantly less than the values for patients with chronic progressive MS. We find that mononuclear cells from healthy control subjects and patients with chronic progressive MS proliferate in response to 200 units/ml of recombinant human interleukin-2 (IL-2) similarly. However, IL-2 treatment increased beta-adrenergic receptor density on normal mononuclear cells, but failed to increase it on mononuclear cells from patients with chronic progressive MS.(ABSTRACT TRUNCATED AT 250 WORDS)     

Test-retest reliability in EEG frequency analysis.

This study was performed to gain a better understanding of EEG frequency analysis test-retest reliability in normal healthy adults, and to evaluate factors which could influence the measured inter-record differences. Nineteen subjects underwent serial EEG recordings at 5 min and 12-16 week intervals. Records were visually edited using a standardized protocol, and FFT frequency analysis performed on segments of 60, 40, or 20 sec total length. Correlation coefficients for broad band features averaged 0.92 over the 5 min retest interval and 0.84 over the 12-16 week interval. There was essentially no difference between correlation coefficients of absolute and relative power features. Coefficients based on 60 sec records were marginally higher than those of 40 or 20 sec records. On the other hand, test-retest percent differences were typically lower for relative as opposed to absolute power features, and 60 sec records showed consistently lower percent differences than did 40 or particularly 20 sec records. Peak alpha frequency and mean frequency were the most stable EEG features at either interval. Montage had significant effects on test-retest differences at the 12-16 week interval. A significant association between intra-record and inter-record variability could not be demonstrated.     

Screening recommendations for the elderly.

BACKGROUND. Studies have documented the potential contributions of preventive health care programs. Yet little is known about which screening tests should be included in public health programs for older persons. This study offers recommendations regarding these tests. METHODS. The recommendations come from synthesizing the findings of the US Preventive Services Task Force, the literature, and the consensus of experts in geriatrics, gerontology, and health policy research. The literature was evaluated to identify methodologically sound studies of the prevalence of selected disorders and benefits and availability of screening procedures for those disorders. Experts from various fields specializing in the care of the elderly formed panels to assist in evaluating the literature and providing further information from gerontological and public health perspectives. RESULTS. We recommend vision testing for refractive error; inspection of the skin surface for fungal infection and skin cancer, drug eruptions, and xerosis; a history for symptoms of xerosis; audiometric testing for presbycusis; surveys for hearing loss; otoscopic inspection for cerumen impaction; dental examination for caries; measurement of blood pressure for hypertension; and breast examination and mammography for cancer. CONCLUSIONS. Our study suggests that these screening procedures are useful for public health screening programs. More information is needed on the effects of screening services on the health and functioning of older persons.