Fulminant subacute sclerosing panencephalitis in association with pregnancy

Subacute sclerosing panencephalitis developed during pregnancy in a 27-year-old woman and immediately after delivery in an 18-year-old woman. In both, disease took an acute and fulminant course culminating in a vegetative state within several weeks. It is suggested that the relative older age of disease presentation and the unusually rapid neurologic deterioration were partially due to immunologic and hormonal alterations of pregnancy.     

Audiotaped versus written administration of the Situational Competency Test

The Situational Competency Test (SCT) is an audiotaped, verbal role-play measure of coping responses to eight drinking and eight non-drinking situations. The present investigation evaluated the comparability of response scores to audiotaped versus written administrations of the SCT in two samples of alcohol clients. The written administration of the SCT yielded significantly shorter durations of response in both clinical samples, but there were no other significant differences between the administration methods on the other scoring dimensions. Correlations of the audiotaped and written summary scores revealed no consistently significant or meaningful associations. Given the differences in duration, the generally poor correlations between summary scores, and the loss of the latency measure in the written format, we recommend retention of the audiotaped administration for use in research.     

The quality of acute stroke units on a nation-wide level: the Austrian Stroke Registry for acute stroke units

Concepts for stroke units that cover the acute phase vary. Therefore, the network of acute stroke units that is being set up in Austria in a uniform way is of general interest. This nationwide network has been established in accordance to evidence-based recommendations and prespecified criteria for available resources. The location for such a unit follows a maximum of 90-min isochrones (transport time) to the hospital. The quality of the network is currently documented and the results are reported. A nationwide stroke registry was prospectively performed on 15 stroke units that were already functional in this network. The aim was to document the quality performance of Austrian stroke units, focusing on rapid admissions, ready availability of investigations and therapies performed. Outcome measures were Barthel scale, Rankin score and percentages of complications. Between August 1998 and December 2000, 2,313 patients with ischemic stroke or with primary intracerebral hemorrhage admitted to an Austrian stroke unit within 24 h after onset of symptoms were prospectively included. Forty-three percent of the patients had a moderate or severe stroke. Fifty-seven percent of all patients were admitted to the stroke unit within 3 h after the onset of symptoms. Twenty-seven percent of these patients were brought in by ambulance accompanied by an emergency physician. Two percent of patients were admitted by helicopter. Fifty-four percent of patients had their first brain imaging within 30 min after admission, another 26% within 3 h. Intravenous thrombolysis was performed in 4.1% of patients. The overall stroke-unit mortality was about 6.8% and mortality at 3 months was 12.9%. The outcome at 3 months showed a modified Rankin Scale score of 0 or 1 in 47% of patients, denoting none or mild impairment. This network of acute stroke units is highly efficient in terms of rapid admissions, short intrahospital delays, as well as rapid use of readily available investigations. Stroke units seem to be well accepted by the general public and the medical community because our data show that all types of strokes are treated in Austrian stroke units, including severe strokes. The total number of concurrently treated acute strokes in other institutions across Austria is not known and no formal comparison with other systems of hospitalized care was undertaken, therefore further research is necessary.     

Neuronal plasticity in the nigrostriatal system of the rat after unilateral removal of vibrissae

The vibrissae of rats were shaved from one side of the face daily for 10 days. To see whether or not this treatment had an effect on crossed and uncrossed striatal afferent connections from the midbrain, the tract tracer horseradish peroxidase was applied to the caudate-putamen on day 11. When the tracer was deposited on the side opposite the vibrissae removal, more labeled cells were found in the contralateral substantia nigra than when it was applied on the same side as the vibrissae removal, or placed in animals with intact vibrissae. Unilateral removal of vibrissae did not affect uptake of the tracer by the cells which give rise to the homolateral nigrostriatal projections. These changes in HRP labeling in the crossed projection from the substantia nigra were seen after 10 days of unilateral removal of the vibrissae; i.e., at a time when the animals have had opportunity to learn to compensate for vibrissotomy-induced behavioral asymmetries.     

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.     

Inhibition and superactivation of the calcium-stimulated isoforms of adenylyl cyclase

It was shown previously that chronic exposure to opiate agonists increases adenylyl cyclase (AC) activity, a phenomenon termed AC superactivation (or supersensitization). More recently, we showed that acute G_i/o-coupled receptor activation inhibits the activity of several AC isozymes, including Ca²⁺/calmodulin-stimulated AC-I and -VIII, whereas chronic receptor activation induces their superactivation. Here, we report that both acute μ-opioid receptor-induced inhibition and chronic induced superactivation of AC-I and -VIII are pertussis toxin sensitive. In addition, we show that proteins that interfere with the activity of {ie195-2} subunits ({ie195-3} scavengers) strongly attenuate the acute inhibition of AC-I and -VIII and the superactivation of AC-I, and abolish the superactivation of AC-VIII. Based on these results, we suggest that {ie195-4} is involved in the acute inhibition and chronic agonist-induced superactivation of AC types I and VIII.     

Time-dependent changes of insufficiency fractures of the sacrum: intraosseous vacuum phenomenon as an early sign

Sacral insufficiency fractures develop over a period of time and show time-dependent changes. We report on 15 CT examinations of 5 patients with early-stage insufficiency fractures of the sacrum. In 4 patients only irregular sclerosis without distinct fracture lines was present in 7 of 8 fractures. Of these 4 patients; 3 exhibited intraosseous gas inclusions in a ventral part of a lateral mass; 5 of 8 fractures disclosed a ventral cortical break. When distinct fracture lines had developed in 1 patient, intraosseous vacuum phenomenon had disappeared. Fracture lines evolve over weeks to months and show central bone absorption. The fractures can heal as demonstrated in 4 of 6 fractures in 3 patients, can persist over 1 year without significant changes or can progress to pseudoarthrosis with bone destruction similar to neuropathic joint disease. Intraosseous vacuum phenomena can persist to this stage. Intraosseous vacuum phenomenon is recognized as a potential finding in the early stage of sacral insufficiency fracture, which also is true for irregular sclerosis and ventral cortical disruption. Correspondence to: A. Stäbler