Slowly progressive hemiparesis in childhood as a consequence of Rasmussen encephalitis without or with delayed-onset seizures

Five young children developed slowly progressive hemiparesis as the initial manifestation of Rasmussen encephalitis (RE). Three have remained seizure free over an observational period of 1.3–1.9 years. In the remaining two patients, seizures occurred after 0.5 and 0.6 years respectively. We suggest that RE might be presently underdiagnosed and should be suspected in cases of new onset hemiparesis. In this series, three out of five patients showed oligoclonal bands on examination of cerebrospinal fluid (CSF) which represented additional diagnostic hints towards an immune-mediated condition. According to recently published formal diagnostic criteria, evidence of progressive cerebral hemiatrophy or bioptic identification of RE-typical inflammation confirms the diagnosis in such cases. Long-term immunotherapy is recommended in order to prevent further tissue loss and functional decline.     

Holographic interferometry forIn Vitro investigation of bioprosthetic valves

Dysfunction of heart valve prostheses—mechanical as well as biological—is a common problem in cardiac surgery. The reasons for the valve failures are still not well understood. Biological valves especially have an unsatisfactory durability; degeneration and calcification very often lead to the failure of the valves. In our opinion, hidden defects present in the valve material prior to implantation of the valves is a plausible explanation for the dysfunction. Hitherto there has been no technique to detect these defects without destructing the specimen. Holographic interferometry proved to be applicable forin vitro evaluation of mechanical heart valve prostheses. In the present paper we describe application of this method to biological valves. Nine porcine bioprostheses and four fresh porcine aortic valves were investigated by means of holographic interferometry. In eight of nine bioprostheses, the results showed irregularities of the leaflet structure which depend on anomalies of the connective tissue of the leaflets of the valves. To make sure that these findings are not due to normal variations of the morphology, the investigations were carried out with fresh and unfixated porcine aortic valves. In the latter, no such anomalies of the structure were detected. The results obtained confirm the above hypothesis on the origin of the later valve dysfunction. Thus, holographic interferometry tests of bioprostheses prior to their implantation prevent the use of potentially dysfunctional valves.Presented at the 35th World Congress, International College of Angiology, Copenhagen, Denmark, July 1993     

Automated CTA quantification of internal carotid artery stenosis: a pilot trial.

PURPOSE: To evaluate the feasibility and accuracy of automated analysis software for use with multidetector computed tomographic angiography (CTA) in the exact grading of internal carotid artery stenosis. METHODS: A retrospective pilot trial was performed using CTA datasets from 87 stenotic carotid arteries in 46 consecutive patients (34 men; median age 73.5 years) with known cerebrovascular disease. Internal carotid artery (ICA) stenosis was graded according to NASCET criteria by 2 experienced vascular radiologists in consensus using axial source images as well as curved planar reformations and digital subtraction angiography (DSA). These results were then compared to those obtained from the automated CTA analysis software and the results of manually adapted automated CTA analysis. RESULTS: Measurements from automated CTA analysis as well as manually adapted automated CTA analysis correlated significantly to those of axial/reformatted CTA and DSA (r=0.53 and r=0.82, r=0.58 and 0.70, respectively, all p<0.05). Compared to axial/reformatted CTA measurements, automated CTA analysis had a median difference of -16%, while manually adapted automated CTA had a difference of -10%. Corresponding differences in a comparison with DSA were +4% and -2%, respectively. Circumferential calcification or kinking of the ICA origin did not significantly interfere with these differences (all p>0.05). Sensitivities for the detection of ICA stenosis >70% by manually adapted automated CTA analysis and automated measurement were 44.2% and 34.9%, respectively, versus axial/reformatted CTA. Compared with DSA as the gold standard, the sensitivities were 54.2% and 62.5%, respectively. Specificities for both methods and gold standards all exceeded 90%. CONCLUSION: Commercially available automated CTA analysis is a feasible tool, but sensitivities are still not sufficient for clinical application.     

Brucellosis of the spine with a synchronous Staphylococcus aureus pyogenic elbow infection

Staphylococcus aureus osteomyelitis and pyogenic arthritis has a different pattern in the elderly than in the young. The axial skeleton is the most frequent site of infection and treatment is usually by intravenous antibiotics. We report a case ofStaph. aureus septic arthritis of the elbow with concomitant osteomyelitis of the spine that was thought to be due toStaph. aureus, but culture of debrided material from the lesion grewBrucella in culture. We suggest that in the elderly it is advisable to obtain a tissue culture diagnosis and not to instigate therapy based on positive blood cultures or a concomitant infection.     

Role of angiotensin II in dynamic renal blood flow autoregulation of the conscious dog

The influence of angiotensin II (ANGII) on the dynamic characteristics of renal blood flow (RBF) was studied in conscious dogs by testing the response to a step increase in renal artery pressure (RAP) after a 60 s period of pressure reduction (to 50 mmHg) and by calculating the transfer function between physiological fluctuations in RAP and RBF. During the RAP reduction, renal vascular resistance (RVR) decreased and upon rapid restoration of RAP, RVR returned to baseline with a characteristic time course: within the first 10 s, RVR rose rapidly by 40 % of the initial change (first response, myogenic response). A second rise began after 20–30 s and reached baseline after an overshoot at 40 s (second response, tubuloglomerular feedback (TGF)). Between both responses, RVR rose very slowly (plateau). The transfer function had a low gain below 0.01 Hz (high autoregulatory efficiency) and two corner frequencies at 0.026 Hz (TGF) and at 0.12 Hz (myogenic response). Inhibition of angiotensin converting enzyme (ACE) lowered baseline RVR, but not the minimum RVR at the end of the RAP reduction (autoregulation-independent RVR). Both the first and second response were reduced, but the normalised level of the plateau (balance between myogenic response, TGF and possible slower mechanisms) and the transfer gain below 0.01 Hz were not affected. Infusion of ANGII after ramipril raised baseline RVR above the control condition. The first and second response and the transfer gain at both corner frequencies were slightly augmented, but the normalised level of the plateau was not affected. It is concluded that alterations of plasma ANGII within a physiological range do not modulate the relative contribution of the myogenic response to the overall short-term autoregulation of RBF. Consequently, it appears that ANGII augments not only TGF, but also the myogenic response.     

Dopamine receptor agonists alter gap prestimulus modulation

An innocuous sensory event (a prestimulus) that briefly precedes a startle-eliciting stimulus (SES) will reduce the amplitude of the subsequently elicited reflex. In three experiments brief silent periods in otherwise continuous noise (gaps) were used as prestimuli to investigate the effects of the D₁ dopamine receptor agonist (±)-SKF-38393 (SKF) and the dopamine D₂ receptor group agonist (−)-quinpirole hydrochloride on gap inhibition of the rat’s acoustic startle reflex. Gap durations of 4 and 50 ms were analyzed. Quinpirole (0–1.6 mg/kg) had a biphasic effect on gap inhibition. Lower doses increased gap inhibition, an effect that peaked at the 0.4 mg/kg dose. For higher doses, inhibition returned to control levels for the 4-ms long gap, but remained elevated for the 50-ms long gap. SKF had no effect on gap inhibition, and haloperidol (0.2 mg/kg) reversed the quinpirole-induced increase of gap inhibition. These data implicate the D₂ dopamine receptor group in gap inhibition of startle modulation. The results are discussed in terms of the effects of catecholamine agonists on attention. Received: 25 July 1995/Final version: 28 April 1997