Long-term outcome of natalizumab-associated progressive multifocal leukoencephalopathy in Austria: a nationwide retrospective study.

BACKGROUND/OBJECTIVE: The use of natalizumab (NAT) in multiple sclerosis (MS) may be complicated by progressive multifocal leukoencephalopathy (PML), a rare and life-threatening opportunistic brain infection. We aimed to analyze the course of MS after PML recovery together with the long-term outcome of NAT-associated PML (NAT-PML) in Austria. METHODS: Retrospective study based on identification of cases in the nationwide Austrian MS treatment registry (AMSTR) and MS centers with review of patient records. The expanded disability status scale (EDSS) was used to measure neurological disability and outcome. RESULTS: As of December 2022, we identified 15 NAT-PML cases in Austria; only 20% occurred after 2016, when increased vigilance commenced. Two patients did not survive acute PML, and an additional patient died five years later, yielding a mortality rate of 20%. Seizures occurred exclusively in patients with pronounced EDSS increase. Gadolinium (Gd)-enhancement on brain magnetic resonance imaging (MRI) on PML suspicion was associated with minor changes of post-PML neurological disability. Long-term follow-up of up to 132 months (median 76 months) was available in 11/15. The overall median EDSS increased from 3.5 at pre-PML to 6.5 at the last assessment. Regarding inflammatory MS-related disease activity during the observation period, one single individual experienced an MS relapse and another patient had two Gd-enhancing brain lesions. Three patients converted to progressive MS within three years from PML and the EDSS further increased in 6/11. CONCLUSIONS: The number of NAT-PML cases is decreasing over time. While many patients accumulated severe persistent neurological deficits compared to pre-PML, inflammatory MS-related disease activity after PML recovery was rare.

3D detection of periodic limb movements in sleep.

The standard polysomnographic method for detecting periodic limb movements in sleep (PLMS) includes measuring the electromyography (EMG) signals from electrodes at the left and right tibialis anterior muscles. This procedure has disadvantages as the cabling affects the patients quality of sleep and the electrodes tend to come off during the night, deteriorating data quality. We used contactless monitoring of body movements by a 3D time-of-flight camera mounted above the bed. Changes in the 3D silhouette indicate motion. Contactless detection of PLMS has several substantial advantages over the EMG and provides more complete and more specific diagnostic data: (1) Motor events caused by other leg muscles than tibialis anterior muscles are fully captured by the 3D method, but missed by EMG. (2) 3D does not react to tonic muscle contractions, where such contractions cause strong deflections in EMG which are annotated as limb movements by most PSG apparatus. Another aspect turned out to be of high practical relevance: Deflections in EMG traces are frequently caused by poor electrode contacts, potentially causing false movement annotations. This can lead to substantial overestimation of the automatically computed PLM index. Contactless sensing completely avoids such problems.

Neuromyelitis optica in Austria in 2011: to bridge the gap between neuroepidemiological research and practice in a study population of 8.4 million people.

BACKGROUND: In 2008 the Austrian Task Force for Neuromyelitis Optica (NMO) started a nation-wide network for information exchange and multi-centre collaboration. Their aim was to detect all patients with NMO or NMO spectrum disorders (NMO-SD) in Austria and to analyse their disease courses and response to treatment. METHODS: (1) As of March 2008, 1957 serum samples (of 1557 patients) have been tested with an established cell based immunofluorescence aquaporin-4 antibody (AQP4-ab) assay with a high sensitivity and specificity (both >95%). All tests were performed in a single reference laboratory (Clinical Dept. of Neurology of the Innsbruck Medical University). (2) A nation-wide survey with several calls for participation (via email newsletters, articles in the official journal of the Austrian Society of Neurology, and workshops) was initiated in 2008. All collected data will be presented in a way that allows that every individual patient can be traced back in order to ensure transparency and to avoid any data distortion in future meta-analyses. The careful and detailed presentation allows the visualization and comparison of the different disease courses in real time span. Failure and response to treatment are made visible at one glance. Database closure was 31 December 2011. All co-operators were offered co-authorship. RESULTS: All 71 NMO- or NMO-SD patients with AQP4-ab positivity (age range 12.3 to 79.6 years) were analysed in detail. Sex ratio (m:f = 1:7) and the proportion of patients without oligoclonal bands in cerebrospinal fluid (86.6%) were in line with previously published results. All identified patients were Caucasians. CONCLUSIONS: A nationwide collaboration amongst Austrian neurologists with good network communications made it possible to establish a database of 71 AQP4-ab positive patients with NMO/NMO-SD. This database is presented in detail and provides the basis for further studies and international cooperation in order to investigate this rare disease.

No influence on selected parameters of human visual perception of 1970 MHz UMTS-like exposure.

In recent years several studies regarding possible effects of radio frequency (RF) electromagnetic fields (EMFs) on cognitive brain function were reported. In many of these studies on awake humans the working tasks were presented visually to the test subjects, e.g., on a computer screen. Therefore, the question of where in the chain of visual perception, brain processing and response a possible effect could be induced seems to be of interest. In this study, possible effects of exposure to a generic 1.97 GHz UMTS-like signal on human visual perception were investigated in a double blinded, crossover study including 58 healthy volunteer subjects (29 male, 29 female), aged 29 +/- 5.1 years (mean +/- SD). Each test subject underwent a battery of four different clinical tests three times (two different exposure levels and sham exposure) to assess selected parameters of visual perception. The generic signals applied to the subjects' head represented the RF emissions of an UMTS mobile phone under constant receiving conditions and the under condition of strongly varying transmit power, i.e., the signal envelope contained low frequency components. In the high exposure condition the resulting average exposure of the test subjects in the cortex of the left temporal lobe of the brain was 0.63 W/kg (1 g averaged SAR) and 0.37 W/kg (10 g averaged SAR). Low exposure condition was one tenth of high exposure and sham was at least 50 dB (corresponding to a factor of 100,000) below low exposure. Statistical evaluation of the obtained test results revealed no statistically significant differences in the investigated parameters of visual perception between the exposure conditions and sham exposure.