Role of DAT‐SPECT in the diagnostic work up of Parkinsonism

The diagnosis of idiopathic Parkinson's disease (PD) can be achieved with high degrees of accuracy in cases with full expression of classical clinical features. However, diagnostic uncertainty remains in early disease with subtle or ambiguous signs. Functional imaging has been suggested to increase the diagnostic yield in parkinsonian syndromes with uncertain clinical classification. Loss of striatal dopamine nerve terminal function, a hallmark of neurodegenerative Parkinsonism, is strongly related to decreases of dopamine transporter (DAT) density, which can be measured by single photon emission computed tomography (SPECT). The use of DAT‐SPECT facilitates the differential diagnosis in patients with isolated tremor symptoms not fulfilling PD or essential tremor criteria, drug‐induced, psychogenic and vascular Parkinsonism as well as dementia when associated with Parkinsonism. This review addresses the value of DAT‐SPECT in early differential diagnosis, and its potential as a screening tool for subjects at risk of developing PD as well as issues around the assessment of disease progression. © 2007 Movement Disorder Society     

Renin secretion by the spontaneously diabetic rat

Renal function studies and measurements of in vivo plasma renin activity (PRA), kidney renin content, and renin secretion by isolated, perfused kidneys were performed in spontaneously diabetic and nondiabetic BioBreeding/Worcester (BB/W) rats. Diabetic animals evidenced hyperglycemia, glycosuria, and plasma volume expansion. After dietary sodium deprivation, plasma volume fell to levels equivalent to those of sodium-deprived, nondiabetic rats. Dietary sodium deprivation evoked a larger proportional increase in PRA among diabetic than nondiabetic animals, although PRA before sodium restriction was equivalent in the two groups. Basal renin release (RR) was higher from isolated, perfused kidneys from diabetic rats than from nondiabetic kidneys. Diabetic kidneys, moreover, displayed increased kidney renin content (KRC). By contrast, while isoproterenol (10(-5) M) stimulated a nearly fivefold increment in RR from nondiabetic, perfused kidneys, a negligible effect was observed in diabetic kidneys. The dose-response curve of renin secretion (as a proportion of total renal content) in response to isoproterenol was shifted downward. Hence, while KRC and spontaneous RR by isolated, perfused kidneys were increased, the increment in PRA with salt depletion and the renin-secretory response to isoproterenol in vitro were impaired. We propose that specific defects in renin secretion, in particular, the response to beta-adrenergic stimulation, may be operative in diabetes.     

The influence of high dietary aluminum on brain microtubule polymerization in mice

One of the possible mechanisms that has been proposed to underlie the deleterious effects of excess aluminum on brain function is an impairment in the normal formation of the cytoskeletal network. Based on recent reports that aluminum can promote the in-vitro polymerization of purified tubulin, in the present study we characterized the effects of high dietary aluminum on in-vitro microtubule formation in brain supernatants. Mice were fed diets containing aluminum 25-1000 micrograms/g for up to 10 weeks. Tubulin polymerization in high-speed brain supernatants was not found to be affected by dietary aluminum. However, we observed that the addition of aluminum in vitro stimulated microtubule assembly in brain supernatants from mice fed control diets, as had been previously reported. Thus, impaired brain microtubule function is not an early general biochemical lesion in aluminum toxicosis.     

Proteolytic Processing Mechanisms in the Biosynthesis of Neuroendocrine Peptides: The Subtilisin-like Proprotein Convertases

The recent discovery of a novel family of precursor processing endoproteases has greatly accelerated progress in understanding the complex mechanisms underlying the maturation of prohormones, neuropeptides, and many other precursor-derived proteins. At least six members of this family have been found thus far in mammalian species, several having alternatively spliced isoforms, and related enzymes have been identified in many invertebrates, including molluscs, insects, nematodes, and coelenterates. The proprotein convertases are all dependent on calcium for activity and all possess highly conserved subtilisin-like domains with the characteristic catalytic triad of this serine protease (ordered Asp, His, and Ser along the polypeptide chain). Two members of this family, PC2(SPC2) and PC1/PC3(SPC3), appear to play a preeminent role in neuroendocrine precursor processing. Both convertases are expressed only in the brain and in the extended neuroendocrine system, while another important family member—furin/PACE (SPC1)—is expressed more ubiquitously, in almost all tissues, and at high levels in liver. SPC2 and SPC3 exhibit acidic pH optima and other properties which enhance their activity in the acidic, calcium-enriched environment of the dense-core secretory granules of the regulated pathway in neuroendocrine cells, while furin has a neutral pH optimum and is localized predominantly to the trans Golgi network where it is retained by a C-terminal transmembrane domain. Furin processes a wide variety of precursors in the constitutive pathway, such as those of growth factors, receptors, coagulation factors, and viral glycoproteins. Recent findings on the processing of proopiomelanocortin, proinsulin, proglucagon, and several other neuroendocrine precursors by SPC2 and SPC3 are discussed, along with information on the structure, properties, evolution, developmental expression, and regulation or the convertases. An inherited defect in the fat/fat mouse which affects the processing of proinsulin, and probably also many other prohormones, due to a point mutation in carboxypeptidase E has recently been identified and has begun to provide new insights into the functional integration of the individual processing steps.     

Impact of Ingested Liquids on 24-Hour Ambulatory pH Tests

A prospective investigation of the impact ofingested liquids on 24-hr pH test scores was conducted.Eighty-two patients contributed 142 samples. The liquidsused were coffee/tea (N = 35), water (N = 32), fruit juice (N = 29), cola (N = 34), and beer (N =12). The pH of cola, juice, and beer are approximately3.0. The parameters studied included: total test time,total drink time, total minutes of pH < 4.0 during drink, minutes of pH < 4.0 10 min beforedrink, and minutes of pH < 4.0 10 min followingdrink. Analysis was performed using one-way ANOVA andrepeated measures. Age of patients, total test time, and total time pH < 4.0 were notsignificantly different (P > 0.05). The total time toconsume the drink was significantly greater (P <0.05) for beer than all other liquids. The total time(7.7 ± 6.0 min) pH < 4.0 for cola wassignificantly different (P < 0.023) than beer (3.3± 3.7 min), tea/coffee (1.4 ± 6.5 min),and water (1.1 ± 2.5 min). The percentage oftotal time pH < 4.0 was not significantly different (P >0.05) among any of the liquids. The percentage of timepH < 4.0 during the drink was the highest for cola(63 ± 47%) and juice (51 ± 57%); water,coffee/tea, and beer were not significantly different (P> 0.05). Although the impact of cola and juice werethe greatest, none of these had an impact that exceeded0.5%. The lack of impact of beer appears to be due to the increased period of time it takes toconsume. We conclude that the impact of ingested fluidsis minimal and can probably be disregarded in mostpatient groups.     

Intra-abdominal desmoplastic small cell tumor presenting as an acute abdomen after trauma

The intraperitoneal mass most commonly encountered after blunt abdominal truama is a hematoma. However, one must also consider unusual bulky tumors that can have imaging characteristics similar to those of hematoma. The most typical of these neoplasms is lymphoma, but a desmoplastic small cell tumor also may be observed. The presentation and imaging findings of a desmoplastic small tumor are described.     

Biliary cystadenoma with mesenchymal stroma of the liver: Correlation between unusual MR appearance and pathologic findings

We reported a case of the biliary cystadenoma of the liver. The cystic mass had labulation and septation and showed marked hyperintensity on T1-weighted images and hypointensity on T2-weighted images; MR findings were very unusual for cystadenoma. The content of the cystic mass was jelly-like, thick mucinous fluid without intracystic hemorrhage. We concluded that these unusual signal intensities of the cyst were due to hyperproteinous mucinous fluid.     

Terminal acute myelogenous leukemia in a patient with congenital agranulocytosis

Congenital agranulocytosis terminating in acute myelogenous leukemia has been previously reported in only two cases of adolescent males. We describe the clinical and laboratory features of a 13-year-old male with congenital agranulocytosis, treated with G-CSF with initial good neutrophil response, who subsequently developed acute myeloid leukemia. This rare complication may define a preleukemic subset of patients for whom G-CSF therapy is ineffective. The diagnostic challenges of this case are presented.