Distinct Subtypes of Behavioral Variant Frontotemporal Dementia Based on Patterns of Network Degeneration.

IMPORTANCE: Clearer delineation of the phenotypic heterogeneity within behavioral variant frontotemporal dementia (bvFTD) will help uncover underlying biological mechanisms and improve clinicians' ability to predict disease course and to design targeted management strategies. OBJECTIVE: To identify subtypes of bvFTD syndrome based on distinctive patterns of atrophy defined by selective vulnerability of specific functional networks targeted in bvFTD using statistical classification approaches. DESIGN, SETTING AND PARTICIPANTS: In this retrospective observational study, 90 patients meeting the Frontotemporal Dementia Consortium consensus criteria for bvFTD underwent evaluation at the Memory and Aging Center of the Department of Neurology at University of California, San Francisco. Patients underwent a multidisciplinary clinical evaluation, including clinical demographics, genetic testing, symptom evaluation, neurologic examination, neuropsychological bedside testing, and socioemotional assessments. All patients underwent structural magnetic resonance imaging at their earliest evaluation at the memory clinic. From each patient's structural imaging scans, the mean volumes of 18 regions of interest (ROI) constituting the functional networks specifically vulnerable in bvFTD, including the salience network (SN), with key nodes in the frontoinsula and pregenual anterior cingulate, and the semantic appraisal network (SAN), anchored in the anterior temporal lobe and subgenual cingulate, were estimated. Principal component and cluster analyses of ROI volumes were used to identify patient clusters with anatomically distinct atrophy patterns. Data were collected from from June 19, 2002, to January 13, 2015. MAIN OUTCOMES AND MEASURES: Evaluation of brain morphology and other clinical features, including presenting symptoms, neurologic examination signs, neuropsychological performance, rate of dementia progression, and socioemotional function, in each patient cluster. RESULTS: Ninety patients (54 men [60%]; 36 women [40%]; mean [SD] age at evaluation, 55.1 [9.7] years) were included in the analysis. Four subgroups of patients with bvFTD with distinct anatomic patterns of network degeneration were identified, including 2 salience network-predominant subgroups (frontal/temporal [SN-FT] and frontal [SN-F]), a semantic appraisal network-predominant group (SAN), and a subcortical-predominant group. Subgroups demonstrated distinct patterns of cognitive, socioemotional, and motor symptoms, as well as genetic compositions and estimated rates of disease progression. CONCLUSIONS AND RELEVANCE: Divergent patterns of vulnerability in specific functional network components make an important contribution to the clinical heterogeneity of bvFTD. The data-driven anatomic classification identifies biologically meaningful anatomic phenotypes and provides a replicable approach to disambiguate the bvFTD syndrome.

Growth, tolerance and biochemical measures in healthy infants fed a partially hydrolyzed rice protein-based formula: a randomized, blinded, prospective trial.

OBJECTIVE: To evaluate growth, tolerance and plasma biochemistries in infants fed an experimental rice protein-based infant formula. DESIGN: Randomized, blinded, 16 week parallel feeding trial of 65 healthy infants fed either an experimental partially hydrolyzed rice protein-based infant formula fortified with lysine and threonine (RPF, n = 32), or a standard intact cow's milk protein-based formula (CMF, n = 33) as a control. Assessments occurred at enrollment (average 2 days), 2, 4, 8, and 16 weeks of age. RESULTS: Growth as indicated by weight, length, and head circumference was not different between the 2 formula groups. All plasma biochemistries for both groups were within reference normal range. However, RPF group had lower phosphorus and urea nitrogen, lower essential amino acids except threonine, which was higher, and lower ratio of essential (including semi-essential) to non-essential amino acids. Differences in the concentrations and ratios of amino acids became less as feeding progressed with age. Plasma total protein, albumin, prealbumin, calcium, magnesium, and alkaline phosphatase were not different between groups. CONCLUSION: Healthy infants fed an experimental partially hydrolyzed rice protein-based formula had normal growth, tolerance, and plasma biochemistry comparable to those of infants fed a standard intact milk protein-based formula, despite some differences in amino acid profiles.

Safety and efficacy of a premixed, rice-based oral rehydration solution.

UNLABELLED: The authors compared the safety and efficacy of a ready-to-use, premixed, rice-based oral rehydration solution (R-ORS) with a glucose-based oral rehydration solution (G-ORS), each containing 75 mmol/L sodium, in Mexican children with acute diarrhea for less than 5 days. METHODS: One hundred eighty-nine boys 3 to 24 months old admitted to the hospital with acute diarrhea and signs of dehydration were randomly assigned to receive either G-ORS or R-ORS. Intake and output were measured every 3 hours. RESULTS: In the group treated with R-ORS, significantly fewer patients required supplemental intravenous fluids during the rehydration phase compared to the G-ORS group (1% v 8.7%; P < 0.01). Mean stool output, percent weight gain, ORS intake, urine output, and number of patients who vomited during rehydration were similar in the two groups. The mean total stool output after the first 24 hours of maintenance phase was significantly lower in the R-ORS group than in the G-ORS group. CONCLUSIONS: The authors found rice-based ORS to be safe, and its use reduced the rate of intravenous fluid therapy in comparison with the use of a glucose-based ORS.