TEMPORALLY GRADED SEMANTIC MEMORY LOSS IN ALZHEIMER'S DISEASE: CROSS-SECTIONAL AND LONGITUDINAL STUDIES

Abstract

Semantic knowledge of famous names and words that entered popular North American culture at different times in the 20th century was examined in 16 patients with mild-to-moderate Alzheimer's disease (AD), 12 of whom were re-tested 1 year later. All patients showed evidence of temporally graded memory loss, with names and words from the remote past being relatively better preserved than recent names and words. There was considerable between-patient variability with respect to severity of semantic impairment. Most patients exhibited losses extending back 30-40 years; however, two mildly impaired (MMSE >28) patients showed deficits restricted to the last 10-15 years. At the 1-year follow-up, patients not only exhibited more severe deficits overall, but the temporally graded period of loss extended further back in time, suggesting that this deficit reflects a loss of previously intact knowledge and not merely faulty encoding or lack of exposure to the material. The extensive period of graded semantic loss exhibited by most patients contrasts with the temporally limited retrograde semantic loss typical of medial temporal lobe amnesia. We propose that short periods of temporally graded semantic memory loss can be explained by damage to medial temporal structures, but that extensive periods of graded loss occur only with additional damage to neocortical tissue. This pattern contrasts with that of autobiographical memory loss, which is often ungraded and extends for the person's entire lifetime, even when damage is restricted to the medial temporal lobes.     

The prediction of body fatness by BMI and skinfold thicknesses among children and adolescents

Background: Although the body mass index (BMI, kg?m^?2) is widely used as a measure of adiposity, it is a measure of excess weight, rather than excess body fat. It has been suggested that skinfold thicknesses be measured among overweight children to confirm the presence of excess adiposity.

Objective: The present study examined the additional information provided by skinfold thicknesses on body fatness, beyond that conveyed by BMI-for-age, among healthy 5- to 18-years old (n?=?1196).

Methods and procedures: Total body dual-energy X-ray absorptiometry (DXA) provided estimates of % body fat, and the sum of two skinfolds (triceps and subscapular) was used as an indicator of the overall skinfold thickness.

Results: As assessed by the multiple R²s and the residuals of various regression models, information on the skinfold sum significantly (?p?<?0.001) improved the prediction of body fatness beyond that obtained with BMI-for-age. For example, the use of the skinfold sum, in addition to BMI-for-age, increased the multiple R²s for predicting % body fat from 0.81 to 0.90 (boys), and from 0.82 to 0.89 (girls). The use of the skinfold sum also reduced the overall prediction errors (absolute value of the residuals) for % body fat by 20–30%, but these reductions varied substantially by BMI-for-age. Among overweight children, defined by a BMI-for-age ≥95th percentile, the skinfold sum reduced the predication errors for % body fat by only 7–9%.

Conclusions: Although skinfold thicknesses, when used in addition to BMI-for-age, can substantially improve the estimation of body fatness, the improvement among overweight children is small.     

Case report of instantaneous resolution of juvenile macular degeneration blindness after proximal intercessory prayer

An 18-year-old female lost the majority of her central vision over the course of three months in 1959. Medical records from 1960 indicate visual acuities (VA) of less than 20/400 for both eyes corresponding to legal blindness. On fundus examination of the eye there were dense yellowish-white areas of atrophy in each fovea and the individual was diagnosed with juvenile macular degeneration (JMD). In 1971, another examination recorded her uncorrected VA as finger counting on the right and hand motion on the left. She was diagnosed with macular degeneration (MD) and declared legally blind. In 1972, having been blind for over 12 years, the individual reportedly regained her vision instantaneously after receiving proximal-intercessory-prayer (PIP). Subsequent medical records document repeated substantial improvement; including uncorrected VA of 20/100 in each eye in 1974 and corrected VAs of 20/30 to 20/40 were recorded from 2001 to 2017. To date, her eyesight has remained intact for forty-seven years.     

Facilitation of ventricular tachyarrhythmia induction by isoproterenol

Ventricular tachyarrhythmia induction was facilitated during infusion of isoproterenol in 21 of 60 patients with ventricular tachycardia (VT) or ventricular fibrillation (VF) in whom programmed electrical stimulation alone failed to reproducibly induce sustained ventricular tachyarrhythmias. Of 44 patients with no ventricular tachyarrhythmias induced before isoproterenol infusion, 11 had a sustained ventricular tachyarrhythmia and 1 patient had unsustained VT induced by isoproterenol alone or by programmed stimulation during the infusion. In 9 of 16 patients in whom nonreproducible or unsustained ventricular tachyarrhythmias were induced before isoproterenol infusion, more reproducible or more sustained ventricular tachyarrhythmias were induced during the infusion. Tachyarrhythmia induction was facilitated by isoproterenol in 20 of 40 patients with sustained VT clinically, but in only 1 of 20 patients with unsustained VT or VF clinically. Among patients with sustained VT clinically, those with exercise-provoked VT and those who had not been tested with stimulation at a second right ventricular site or in the left ventricle were more likely to have induction facilitated by isoproterenol. Drugs effective against induction of isoproterenol-facilitated ventricular tachyarrhythmias were identified in 13 of 25 trials. These drugs were effective during a mean follow-up of 17 months in 7 of 9 long-term trials.     

Malignant Rhabdoid Tumor of the Floor of Mouth: First Reported Case in the Oral Cavity of an Adult

Malignant rhabdoid tumors (MRTs) are exceedingly rare lesions. To our knowledge, only 2 cases have been reported in the oral cavity, with both examples occurring in infants. The current case is the third reported case of MRT of the oral cavity and the first reported case to occur in an adult at this location. The following report describes the clinical, histologic and immunohistochemical features of this tumor.     

Failure of recombinant human interleukin-3 therapy to induce erythropoiesis in patients with refractory Diamond-Blackfan anemia [see comments]

In two previous studies, we observed that recombinant human interleukin- 3 (IL-3) induced an increase in marrow burst-forming unit-erythroid- derived colonies in vitro in some patients with Diamond-Blackfan anemia (DBA). To determine whether a similar erythropoietic response could be induced in vivo, we treated 13 patients with DBA (aged 4 to 19 years) with two preparations of IL-3. All patients had absent absolute reticulocyte counts and markedly reduced to absent recognizable bone marrow erythroid elements; patients with circulating reticulocytes in the previous 12 months were excluded from study. All patients except 1 had failed steroid therapy and had been transfusion-dependent since infancy; 1 patient was maintained on high-dose prednisone at the time of enrollment. On the first arm of the study, IL-3 (Immunex Corp, Seattle, WA) was administered subcutaneously using a dose escalation regimen of 125 to 500 micrograms/m2/day in divided dosage at 12-hour intervals, coadministered with 1.5 mg/kg/d of oral ferrous sulphate. Of the 13 patients that entered the trial, 4 stopped prematurely because of adverse side effects. In the other 9 evaluable cases, reticulocytes increased transiently in 1 patient from 0 to 65 x 10(9)/L after 35 days of IL-3 therapy at 250 micrograms/m2, but transfusion dependency persisted. One transient peak in absolute reticulocyte count was noted in 6 other patients, but no erythroid response was observed after completion of a full course of IL-3. Oral prednisone at 0.5 mg/kg/d was then coadministered with IL-3 at 500 micrograms/m2 to 5 of the patients without effect, and treatment was stopped. In 2 patients, a second preparation of IL-3 (Sandoz Canada Inc, Dorval, Quebec, Canada) was initiated in a dose escalation regimen of 2.5 to 10 micrograms/kg and was coadministered with ferrous sulphate. No erythroid response was observed in either patient, and in one of the two, alternate-day subcutaneous recombinant erythropoietin at 300 U/kg was administered for 3 weeks in combination with daily IL-3 at 10 micrograms/kg, but no increased erythropoiesis was seen. Significant increases in white blood cell and eosinophil counts during administration of both preparations of IL-3 were observed in all patients. These data show that the response of DBA patients to IL-3 in vivo is heterogeneous and cannot be predicted from in vitro studies. The absence of a corrective effect of IL-3 in these patients with DBA indicates that a deficiency of the cytokine is not central in the pathogenesis of the disorder.     

Risk factors in early life as predictors of adult heart disease: the Bogalusa Heart Study

The adult heart diseases, coronary artery disease and essential hypertension, are now clearly recognized to begin in childhood. The evidence comes from autopsy studies of cardiovascular-renal changes in the first two decades of life. Cardiovascular risk factors can be identified in children just as in adults and these have a high correlation with the anatomic disease. This relationship underscores the importance of risk factor screening of children. Of interest is that clinical risk factors tend to persist within a rank (track) so that studies in childhood can be predictive of future levels. Behavior and lifestyle of eating, cigarette smoking, alcohol intake, and use of oral contraceptive pills influence risk factors in children. Familial aggregation of risk factors are also noted. Studies of apolipoproteins, B and A-I, have identified subsets of children that have a greater frequency of paternal myocardial infarction. The findings from the Bogalusa Heart Study and other epidemiological studies of children show the need to begin prevention of adult heart disease in early life. Approaches to prevention should include high risk families and children and a public health or population approach. Cardiovascular health education for elementary school children should be directed to children in the general population in an effort to encourage them to adopt healthy life styles.