Frank R. Brown

Immunologic dysfunction: simultaneous study of alzheimer's and older down's patients ☆

Peripheral blood lymphocytes from Alzheimers disease patients, older patients (aged 25-59 years) with Downs syndrome and closely age-matched healthy controls were tested in vitro for DNA synthesis in response to stimulation by phytohemagglutinin, pokeweed mitogen and OKT3 monoclonal antibody to T3 antigen. All were significantly decreased in Downs and Alzheimers patients relative to healthy controls. The autologous mixed lymphocyte reaction (AMLR), unstimulated or stimulated with anti-T3. in the 2 patient groups did not differ significantly from the healthy controls. We also quantified the proportions of the T cells staining positive for surface membrane orosomucoid, and found their levels to be either higher or lower than normal in Downs patients, but not in Alzheimers patients. These results demonstrate the existence of defective cellular immune functions in both elderly Downs patients and Alzheimers patients.

A case study of the cognitive and behavioral deficits of temporal lobe damage in herpes simplex encephalitis

Herpes simplex viral encephalitis is a fairly common nonepidemic encephalitis which produces severe neurological sequelae in survivors. Most viral infections of the central nervous system produce diffuse damage, but the herpes simplex virus demonstrates a predilection for localization in the temporal and orbitofrontal regions of the brain. This case study illustrates the highly significant language difficulties, marked memory deficits, and propensity for physical aggression following temporal lobe damage brought about by herpes encephalitis, and presents the usefulness of a new diagnostic measure in delineating such a variable cognitive pattern.

Adrenal and testicular function in 14 patients with adrenoleukodystrophy or adrenomyeloneuropathy.

Testicular and adrenal function were evaluated in 12 patients with adrenoleukodystrophy and 2 patients with adrenomyeloneuropathy. Although only 5 subjects had clinical symptoms suggesting adrenal insufficiency, an additional 5 showed laboratory evidence of reduced adrenal reserve. 9 of the 14 patients developed neurological deficits prior to the onset of clinical or biological adrenal insufficiency. In the remaining 5 patients, adrenal insufficiency antedated the appearance of neurological symptoms; 2 of these 5 patients had only laboratory evidence of hypoadrenocorticism, and 3 had both clinical and laboratory abnormalities. None of the prepubertal patients had detectable signs of testicular insufficiency, while 3 of the 7 pubertal/adult patients had elevated serum LH or FSH levels. This mild testicular deficiency was seen only in association with clinical adrenal insufficiency and significant neurological impairment.

Diagnosis and Management of Learning Disabilities

As known, adventure and experience about lesson, entertainment, and knowledge can be gained by only reading a book. Even it is not directly done, you can know more about this life, about the world. We offer you this proper and easy way to gain those all. We offer many book collections from fictions to science at all. One of them is this diagnosis and management of learning disabilities that can be your partner.

Effect of proteolipid protein on central nervous system myelin membrane fluidity

The effect which intrinsic (proteolipid) protein has on fluidity of central nervous system myelin membrane was measured through differences in temperature-dependent anisotropy of the lipid-soluble fluorescence probe, 1,6-diphenyl-1,3,5-hexatriene (DPH), in multilamellar vesicles (MLV) prepared from total myelin lipids in the presence and absence of proteolipid protein. Very little difference was observed in the anisotropies of DPH incorporated into intact myelin membrane vesicles compared with MLV reconstituted from total myelin lipid plus proteolipid protein but excluding myelin basic protein. In contrast, a significant decrease (P less than 0.01) in anisotropy was observed when MLV prepared from total myelin lipids depleted of proteolipid protein were compared with vesicles containing proteolipid protein. Given the different distributions of myelin basic protein and proteolipid protein suggested by freeze-fracture, neutron and X-ray diffraction studies, and the fact that the hydrophobic DPH probe is known to distribute in the non-polar regions of lipid bilayers, we interpret the marked decrease in anisotropy when proteolipid protein is excluded from MLV to suggest that at least part of the proteolipid is distributed in the hydrocarbon region of the MLV. These findings are consistent with the earlier physical studies and recent postulations that extensive hydrophobic segments exist in proteolipid protein and that these hydrophobic segments are buried in the myelin lipid bilayer and alternate with hydrophilic extra-membrane segments.

Maternal Docosahexaenoic Acid (DHA) Supplementation Does Not Affect Neurodevelopmental Outcome of Breast-fed Term Infants at One Year of Age• 1584

Maternal Docosahexaenoic Acid (DHA) Supplementation Does Not Affect Neurodevelopmental Outcome of Breast-fed Term Infants at One Year of Age• 1584

Early Pediatric Neurodevelopmental Profile and Laboratory Workup of Children with Autism and Pervasive Developmental Disorder-Not Otherwise Specified (PDD-NOS)

Early Pediatric Neurodevelopmental Profile and Laboratory Workup of Children with Autism and Pervasive Developmental Disorder-Not Otherwise Specified (PDD-NOS)

Concurrent and Predictive Validity of the Clinical Linguistic and Auditory Milestone Scale (CLAMS) and the Mental Developmental Index (MDI) of the Bayley Scales of Infant Development (BSID)

Concurrent and Predictive Validity of the Clinical Linguistic and Auditory Milestone Scale (CLAMS) and the Mental Developmental Index (MDI) of the Bayley Scales of Infant Development (BSID)

Rate of Developmental Progress in the Second Year is Compared to the First Year of Life in Premature Infants † 1325

Correction for prematurity in early developmental assessment of high risk infants continues to be a dilemma. Correction for prematurity in the first year of life is a generally accepted practice, however, correction in the second year is more controversial. To determine whether “catch up” still occurs in the second year of life, 75 premature infants born prior to 31 weeks gestation (mean gestational age = 28 weeks with a range of 24 to 31 weeks) were followed serially at 4 month intervals during the first 2 years of life. The Clinical Adaptive Test (CAT)/Clinical Linguistic and Auditory Milestone Scale (CLAMS) and the Gross Motor scale of the Revised Gesell Developmental Screening Inventory were used to determine each infants developmental function in visual problem solving, language and gross motor domains at each visit. Interval gain in the first year was compared with interval gain in the second year using a paired t test for each of the developmental domains. The rate of developmental progress in the second year was greater than in the first year in all domains. Differences between rates of progress in year one versus year two were significant for language(mean interval gain in year one = 84%, mean interval gain in year two = 111%, N = 64, t = -4.865, p <.0001) and gross motor skills(mean interval gain in year one = 90%, mean interval gain in year two = 101%, N = 62, t = -2.59, p = 0.01). There was not a statistically significant difference between rate of progress in years one and two for visual problem solving skills (mean interval gain in year one = 106%, mean interval gain in year two = 112%, N = 68, t = -1.539,p = 0.13). This data suggests that there is still “catch up” occurring in year two, especially in gross motor and language skills. Therefore, gestational age at the time of delivery should still be considered when doing developmental evaluations in the second year of life.

BIOCHEMICAL FEATURES OF A PATIENT WITH ZELLWEGER-LIKE SYNDROME WITH NORMAL PTS-1 AND PTS-2 PEROXISOMAL PROTEIN IMPORT SYSTEMS: A NEW PEROXISOMAL DISEASE. ♦ 626

The peroxisomal disorders represent a group of inherited metabolic disorders that derive from defects of peroxisomal biogenesis and/or from dysfunction of single or multiple peroxisomal enzymes. We described earlier an 8 1/2 year-old with a history of progressive developmental delay, micronodular cirrhosis, and elevated very long chain fatty acids in plasma and skin fibroblasts. These findings were felt to be compatible with both neonatal adrenoleukodystrophy (nALD) and Zellweger syndrome (ZS). This patient is now 21 years old and his clinical course, inconsistent with either nALD or ZS, led us to examine his peroxisomal status in light of a possible new peroxisomal disease. The normal levels of bile acid precursors found in this patient suggest that peroxisomal beta-oxidation is functional. The activities of dihydroxyacetone phosphate acyltransferase and oxidation of lignoceric acid and phytanic acid were 14, 17, and 15% of the control, respectively. This partial activity for oxidation and the normal levels of bile acid precursors suggests that this patient has peroxisomes containing beta-oxidation enzymes. Western blot analysis of subcellular organelles showed that beta-oxidation enzyme proteins are present at normal levels in catalase-negative peroxisomes of density equivalent to normal peroxisomes. The presence of acyl-CoA oxidase and 3-ketoacyl-CoA thiolase in catalase-negative peroxisomes suggests that both peroxisomal targeting signal-1 (PTS-1), and peroxisomal targeting signal-2 (PTS-2)-mediated protein transport processes into peroxisomes are normal in this patient. These findings of catalase-negative peroxisomes of normal density and normal PTS-1 and PTS-2 import machinery with partial peroxisomal functions clearly demonstrate that this patient differs from those with known disorders of peroxisomal biogenesis.