Growth hormone deficiency, short stature, and juvenile rheumatoid arthritis in a patient with autoimmune polyglandular syndrome type 1: case report and brief review of the literature.

Autoimmune polyglandular syndromes (APSs) include a cluster of autoimmune and nonautoimmune conditions which have been classified into subtypes. APSs type 1 is characterized by at least two of the following: chronic mucocutaneous candidiasis, chronic hypoparathyroidism, and autoimmune Addison's disease (AD). We report the chronological history of a female patient who presented with features most consistent with APS type 1, along with growth hormone deficiency and juvenile rheumatoid arthritis (JRA). In terms of her autoimmune diagnoses, she first presented with JRA at three years of age, then hypocalcemia and hypoparathyroidism at five years of age, type 1 diabetes (DM 1) at age eleven years, adrenal insufficiency at age fourteen years, recurrent mucocutaneous candidiasis as a teenager, growth hormone deficiency at age fourteen years leading to significant short stature, primary amenorrhoea, and hypogonadism, and finally alopecia at age twenty-six years. In addition to this, she has suffered other nonautoimmune medical problems including a Tetralogy of Fallot with a surgical repair at age six years. On review of the medical literature, we found no other previously reported case with this unique combination of medical problems.

MR properties of excised neural tissue following experimentally induced demyelination.

Changes in the magnetic resonance (MR) parameters of demyelinated neural tissue were measured in vitro using an experimental animal model. A tellurium (Te) diet was applied to weanling rats to induce the demyelination process in the sciatic nerve. The quantitative MR parameters, such as T(1), T(2) relaxation time constants and magnetization transfer (MT) were measured each day after applying the Te diet (up to 7 days) and were found to be substantially different from those of normal nerves. An increase in the average T(1) and T(2) was observed along with a decrease in the MT ratio (MTR) and the quantitative MT parameter M(0B), which describes the semisolid pool of protons. Most of the MR parameters correlated very well with the myelin fraction of neural tissue evaluated by quantitative histopathology. The T(2) relaxation spectrum provided the most efficient quantitative assessment of changes in neural tissue microstructure and its analysis resulted in a powerful tool to distinguish the processes of demyelination and inflammation. In comparison, the MT measurements were less successful.

Effect of feeding patterns on performance of a visuospatial memory task in the beagle dog: a novel cognitive-based protocol for assessing satiety.

The assessment of appetite suppressing effects, or satiating effects, of drugs or other treatments is typically based on the measurement of food consumption and body weight. The present study describes a novel cognitive-based protocol for assessing satiety in the dog based on response latency and performance accuracy on a canine test of spatial working memory, the three-component delayed-non-matching-to-position task (3cDNMP). We hypothesized that satiety, produced by providing food prior to testing, would reduce motivation to respond quickly and accurately on this food-reinforced task. Dogs were first over-trained on a variable-delay version of the 3cDNMP task. They were then pre-fed with either a single or a double portion of food prior to being tested on the same task. Pre-feeding slowed response latency, but had no effect on performance accuracy. A more pronounced increase in response latency was observed in young dogs than in old dogs when offered double portions of food. These results suggest, first, that spatial working memory capability is independent of motivation; second, that satiety is age sensitive; and third, that a cognitive protocol can provide a reliable method for evaluating the satiating effects of various foods and other compounds in the dog.

Histological and magnetic resonance analysis of sciatic nerves in the tellurium model of neuropathy.

Ingestion of tellurium (Te), a toxic element, produces paralysis of the hind limbs in weanling rats that is due to temporary, segmental demyelination of the sciatic nerves bilaterally. Weanling rats were fed a 1.1% elemental Te diet and sacrificed at various time points for histological and magnetic resonance (MR) analysis of the sciatic nerves. No controls exhibited impairments of the hind limbs, whereas Te-treated animals became progressively impaired with increased Te exposure. Toluidine blue-stained nerve sections of Te-treated animals showed widened endoneurial spaces, disrupted myelin sheaths, swollen Schwann cells, and a few instances of axonal degeneration. Te decreased healthy myelin by 68% and increased percent extracellular matrix by 45% on day 7. MR experiments showed a decrease in the area of the short T2 component, an increase in average T1, and an increase in the position of the intermediate T2 component in Te-treated nerves. The correlation coefficient for healthy myelin and average T1 was 0.88 and that for healthy myelin and the area underneath the short T2 component was 0.77. The area of the short T2 component has been postulated as the best measure of the process of demyelination.

MR properties of excised neural tissue following experimentally induced inflammation.

Changes in the MR parameters of inflamed neural tissue were measured in vitro. Tumor necrosis factor-alpha (TNF-alpha) was injected into rat sciatic nerves to induce inflammation with negligible axonal loss and demyelination. The MR parameters, such as T1/T2 relaxation and magnetization transfer (MT), were measured 2 days after TNF-alpha injection and were found to be substantially different from those of normal nerves. The average T1/T2 relaxation times increased, whereas the MT ratio (MTR) and the quantitative MT parameter M0B (which describes the semisolid pool of protons) decreased. The MR parameters correlated very well with the extracellular volume fraction (EM) of neural tissue evaluated by quantitative histopathology. The multicomponent T2 relaxation was shown to provide the best quantitative assessment of changes in neural tissue microstructure, and allowed us to distinguish between the processes of inflammation and demyelination. In comparison, the MT measurements were less successful due to competing contributions of demyelination and pH-sensitive changes in the MT effect.