Search

Neuromyelitis optica (NMO) is characterised by a particular pattern of the optic nerves and the spinal cord. Long-term MRI follow-up studies of spinal NMO lesions are rare, or limited by short observation periods. In nine patients with definite NMO or recurrent longitudinally extensive transverse myelitis (LETM) with NMO-IgG serum antibodies, repeated MRI examinations of the spine were carried out over a period of up to 11 years and evaluated regarding the changes over time in this retrospective study. In eight patients spinal cord lesions were located centrally, involving the grey and white matter. In the first examination after clinical onset changes resembled a stroke of the anterior spinal artery in two patients. Symmetrical signal alterations within the grey matter were observed. In one patient this pattern was transient, but it remained in the other. During the chronic stage, either a variable degree of spinal cord atrophy and high signal alterations, or almost complete remission of the lesions, was observed. Spinal MRI of patients with NMO myelitis can resemble a stroke. MRI of acute NMO stages did not allow a prediction of the clinical outcome. To a variable degree, NMO left behind typical defects which correlated with the clinical outcome. [ABSTRACT FROM AUTHOR]

Background: There is a lack of knowledge of disease course, prognosis, comorbidities and potential treatments of elderly MS patients., Objective: To characterize the disease course including disability progression and relapses, to quantify the use of DMTs and to identify comorbidities and risk factors for progression in elderly multiple sclerosis (MS) patients., Methods: This is a retrospective study of 1200 Austrian MS patients older than 55 years as of May 1st, 2017 representing roughly one-third of all the MS patients of this age in Austria. Data were collected from 15 MS centers including demographics, first symptom at onset, number of relapses, evolvement of disability, medication, and comorbidities., Results: Median observation time was 17.1 years with 957 (80%) relapsing and 243 (20%) progressive onsets. Average age at diagnosis was 45 years with a female predominance of 71%. Three-hundred and twenty-six (27%) patients were never treated with a DMT, while most treated patients received interferons (496; 41%) at some point. At last follow-up, 420 (35%) patients were still treated with a DMT. No difference was found between treated and never-treated patients in terms of clinical outcome; however, patients with worse disability progression had significantly more DMT switches. Pyramidal onset, number of comorbidities, dementia, epilepsy, and psychiatric conditions as well as a higher number of relapses were associated with worse outcome. The risk of reaching EDSS 6 rose with every additional comorbidity by 22%. In late and very-late-onset MS (LOMS, VLOMS) time to diagnosis took nearly twice the time compared to adult and early onset (AEOMS). The overall annualized relapse rate (ARR) decreased over time and patients with AEOMS had significantly higher ARR compared to LOMS and VLOMS. Four percent of MS patients had five medications or more fulfilling criteria of polypharmacy and 20% of psychiatric drugs were administered without a matching diagnosis., Conclusions: In this study, we identified number of comorbidities, pyramidal and cerebellar signs, and a higher number of relapses as unfavorable prognostic factors in elderly MS patients filling gaps of knowledge in patients usually underrepresented in clinical trials and may guide future therapeutic studies., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

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., (© 2023. The Author(s).)

Background and Purpose: The benefit of thrombectomy (TE) for acute ischemic stroke (AIS) in patients suffering basilar artery occlusion (BAO) is still unclear. Our aim was to analyze functional outcome after 3 months in BAO compared to anterior circulation large vessel occlusion (ACLVO) in a nationwide registry., Methods: Patients enrolled into the Austrian Endostroke Registry from 2013 to 2018 were analyzed. We used propensity score matching to control for imbalances and to compare patients with BAO and ACLVO. The primary outcome was favorable functional outcome after 3 months measured by the modified Rankin Scale (mRS) (0-2). Multivariate models were applied to estimate the effect of localization (BAO vs ACLVO)., Results: In total, 2288 patients underwent TE for AIS with proximal vessel occlusion, of these 267 with BAO. Two hundred and sixty-four patients with BAO were matched to 264 patients with ACLVO. Baseline characteristics were well-balanced. The 90-day mortality did not significantly differ between patients with BAO and ACLVO. In a multivariate logistic regression model, we did not detect a significant difference in functional outcome between BAO and ACLVO (odds ratio for favorable outcome defined as mRS = 0-2: 1.19; 95% confidence interval (CI) = 0.78-1.81; p  = 0.42). In patients with an onset-to-door-time ⩾270 min, TE of BAO was associated with poor functional outcome defined as mRS 3-6 (odds ratio (OR) = 3.97; 95% CI = 1.32-11.94; p  = 0.01) as compared to ACLVO., Conclusion: In this study, functional outcome did not differ after TE in patients with BAO and ACLVO overall; however, we detected an association of BAO with poor outcome in patients arriving late.

This guideline comprises the state of science at the time of the editorial deadline. In view of the high turnover of knowledge the guideline is designed as a living guideline. The main objective was to provide a tool for the use in primary care, being considered well suited as a first point of entry and for the provision of care. The guideline gives recommendations on the differential diagnosis of symptoms following SARS-CoV‑2 infection, on their therapeutic options, as well as for guidance and care of the patients concerned. It also offers advice concerning return to daily life and rehabilitation. Long COVID being a very variable condition, we chose an interdisciplinary approach., (© 2021. The Author(s).)

Multiple sclerosis is a complex, autoimmune-mediated disease of the central nervous system characterized by inflammatory demyelination and axonal/neuronal damage. The approval of various disease-modifying therapies and our increased understanding of disease mechanisms and evolution in recent years have significantly changed the prognosis and course of the disease. This update of the Multiple Sclerosis Therapy Consensus Group treatment recommendation focuses on the most important recommendations for disease-modifying therapies of multiple sclerosis in 2021. Our recommendations are based on current scientific evidence and apply to those medications approved in wide parts of Europe, particularly German-speaking countries (Germany, Austria, Switzerland)., (© 2021. The Author(s).)

Background: Injury of the recurrent laryngeal nerve and consequent disorder of vocal fold movement is a typical complication in thyroid and parathyroid surgery. During postoperative laryngoscopy we observed not only a complete standstill (vocal fold paralysis), but also a hypomobility (paresis). In this prospective study, we investigated the difference in incidence and prognosis as well as risk-factors, intraoperative neuromonitoring, and symptoms between vocal fold paralysis and vocal fold paresis., Methods: Data were prospectively collected and analyzed in a single high-volume thyroid center between 2012 and 2016. Vocal fold paresis was defined as hypomobility in abduction or adduction, a reduction in range and speed of vocal fold movement. Vocal fold paralysis was defined as asymmetry and missing purposeful vocal fold movement., Results: The study included 4,707 surgeries and 7,992 at-risk nerves at risk. Vocal fold paralysis was diagnosed in 374 patients (4.68% of 7,992 nerves at risk) and vocal fold paresis in 114 patients (1.43%). Exclusively in the paralysis group, 36 patients (0.45%) developed permanent loss of vocal fold function (P < .001). In follow-up, vocal fold paresis patients regain normal vocal fold function significantly earlier than vocal fold paralysis (mean duration: 6.96 ± 6.506 vs 10.77 ± 7,827 weeks) and presented with significantly less symptoms like hoarseness, diplophonia, dysphagia, and dyspnea (68.8% vs 95.9 %). In intraoperative neuromonitoring, vocal fold paresis showed a significantly higher postresectional N. vagus amplitude than vocal fold paralysis patients (0.349 mV vs 0.114 mV, P < .001)., Conclusion: After thyroidectomy, vocal fold paresis must be distinguished from vocal fold paralysis and should be implemented as a separate outcome parameter in the postoperative quality assessment., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background and Purpose- Posterior circulation stroke (PCS) account for 20% of all ischemic strokes. There is limited evidence whether functional outcome of PCS is comparable to that of anterior circulation stroke (ACS). We aimed to analyze whether 3-month functional outcome is different in PCS and ACS. Methods- Patients with acute ischemic stroke prospectively enrolled within the Austrian Stroke Unit Registry were stratified by infarct localization according to the Oxfordshire Community Stroke Project Classification. Propensity score matching was used to control for covariate imbalances and to match patients with PCS and ACS. Patients were matched for stroke severity, recombinant tissue-type plasminogen activator treatment, and demographic and vascular risk factors. Main outcomes were the distribution of modified Rankin Scale after 3 months and multiple proportional odds models to estimate the influence of the infarct localization on the functional outcome. Results- From a total of 90 484 patients enrolled within the Austrian Stroke Unit Registry, 9208 (4604 PCS/4604 ACS) were matched, of those 954 (477 in each group) were treated with recombinant tissue-type plasminogen activator. We detected a significant shift towards better 3-month functional outcome in patients with ACS compared with PCS (odds ratio [OR], 1.19; 95% CI, 1.1-1.28; P<0.0001). In particular, functional outcome was worse in PCS with onset-to-door-time >270 minutes (OR, 1.34; 95% CI, 1.17-1.54; P<0.0001) and in PCS with unknown onset-to-door-time (OR, 1.26; 95% CI, 1.13-1.42; P<0.0001); however, we did not detect any difference in functional outcome between ACS and PCS in patients with an onset-to-door-time ≤270 minutes (1-180 minutes: OR, 0.92, 95% CI, 0.78-1.09, P=0.3554; 181-270 minutes: OR, 1.04, 95% CI, 0.79-1.37, P=0.7689). In patients treated with recombinant tissue-type plasminogen activator, functional outcome was not significantly different between PCS and ACS. Conclusions- PCS was associated with worse outcome compared with ACS in patients arriving later than 4.5 hours at hospital or in those with unknown onset of symptoms. Our results urge for implementation of symptoms found in the posterior circulation into preclinical patient-triage tools.

Therapeutic effect of recombinant tissue-plasminogen activator (rt-PA) is time dependent. There is limited evidence whether localization of stroke within the posterior circulation (PCS) is associated with a treatment delay. We aimed to analyze within a nationwide multicenter cohort whether duration of pre- and intra-hospital patient management differs between patients with PCS and anterior circulation strokes (ACS). We studied onset-to-door-times (ODT) and door-to-needle-times (DNT) of all patients with acute ischemic stroke (IS) enrolled in the Austrian Stroke Unit Registry according to infarct localization. Classification into PCS and ACS was based on clinical presentation applying the criteria used in the Oxfordshire Community Stroke Project. Relationships between ODT, respectively, DNT and explanatory variables were modeled by multivariate linear regression. Between 2003 and 2015, 71010 patients with IS were enrolled, 11,924 with PCS and 59,086 with ACS. Overall, the ODT was significantly longer in PCS: median (IQR): 170 (25th, 75th‰: 79,420) min versus 110 (60,240); p < 0.001; this finding held true in multivariable analysis. In 10535 rt-PA-treated patients (1022 PCS/9832 ACS), ODT and DNT were significantly longer among those with PCS: ODT: median: 80 min (55,120) versus 72 (50,110), p < 0.001; DNT: 57 (35.90) versus 45 (30.67), p < 0.001. In the multivariate model, PCS was significantly associated with delay in the DNT. In conclusion, in this large nationwide cohort, patient management was significantly slower in PCS as compared to ACS. Increasing awareness about these delays and further elaboration of the underlying causes may translate into higher proportions of patients with PCS receiving rt-PA., Competing Interests: Compliance with ethical standards Conflicts of interest The authors declare no conflict of interest.

Objective: It is not clear whether risk scores for early stroke recurrence after TIA that have been mainly established in outpatient and emergency department settings are valid on the background of highly specialized stroke unit care., Methods: ABCD2 and ABCD3-I scores have been prospectively documented in a cohort of patients admitted to Austrian stroke units within 24 hours of symptom onset with TIA or minor stroke (NIH Stroke Scale score <4)., Results: A total of 5,237 TIA and minor stroke patients met inclusion criteria, with 3-month follow-up data available on 2,457. Early and 3-month stroke were observed in 2.4% and 4.2% of the study population. The probability of early stroke during the stroke unit stay (median 2 [interquartile range 1-3] days) steadily increased from 0% to 4.8% and 0% to 16.7% with increasing ABCD2 and ABCD3-I score points, respectively. On 3-month follow-up, stroke risk increased from 0% to 8.0% and 0% to 23.8% with increasing ABCD2 and ABCD3-I score points, respectively. Of the individual score components, age, blood pressure, and diabetes were not related to early or 3-month stroke, whereas clinical presentation (C), symptom duration (D), and cerebral as well as carotid imaging (I) were and accounted for the information provided by the full scores., Conclusions: Standard ABCD2 and ABCD3-I scores are useful instruments to estimate the probability of early and 3-month stroke in TIA and minor stroke patients treated at specialized stroke units, with C, D, and I being the most important score components in this setting., (© 2016 American Academy of Neurology.)

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.

During the last 10 years recombinant interferon-beta-1a administered subcutaneously has been the subject of several clinical trials in relapsing remitting multiple sclerosis (RRMS), in secondary progressive MS (SPMS), as well as in clinically isolated syndromes. All of them met the criteria of evidence level class I. Consistent evidence for moderate immunomodulatory effects on clinical parameters of disease activity was gained, and even higher efficacy of IFN-beta-1a sc. on MRI activity of multiple sclerosis was proven. Indirect evidence confirmed the hypothesis of a dose-response curve for IFN-beta-1a formulations in MS. The higher efficacy of IFN-beta-1a 44 microg sc. TIW, however, also includes more adverse events such as injection site reactions, flu-like symptoms and a moderate immunogenicity. Current evidence does not allow a recommendation of IFN-beta-1a sc. as most effective first line therapy, because also the individual patient's choice in the route of administration and long-term effects of neutralizing antibodies to IFN-beta-1a sc. must be taken into account. In the long-term, IFN-beta-1a showed a beneficial safety-tolerability profile with 50 % of patients sticking to the initial immunomodulatory treatment. There were no teratogenic effects, IFN-beta-1a sc. did not enhance depressive symptoms. Data on inhibition of the progression of disease, however, remained inconclusive. Probable beneficial effects of IFN-beta-1a sc. on cognitive function or "chronic fatigue" have not been investigated yet.

Background: About half of out-of-hospital cardiac arrest survivors experience secondary anoxic brain damage. Neurological outcome can be influenced by rehabilitative treatment approaches, but the nature and severity of persistent disabilities remain unclear. The aim of the study was to explore persistent neuropsychiatric symptoms, global function and life situation of these patients, and to evaluate quality of life in families., Methods: 25 months after inpatient rehabilitation, 12 individuals (mean age=51 years; ten M: two F) attended a cross-sectional interdisciplinary follow-up assessment with their carers. Function was investigated by clinical rating scales, neuropsychological standard tests, and clinical psychological inventories. Family members were asked about quality of life and satisfaction with social support., Results: All patients had deficits in at least one or more cognitive areas such as orientation, memory, alertness, and awareness. Three different clinical syndromes were observed: very severe intellectual and physical impairment, (two), mild to moderate dementia, (five), and amnesic syndrome, (five). Prevalence of multiple disabilities, was high. A striking discrepancy was found between self and proxy rating of disabilities (P<0.01). Family members faced dramatically altered life situations after CA; 60% of spouses suffered from psychosomatic problems, 50% complained of lack of social support., Conclusion: Despite optimal in-hospital treatment, severe anoxic brain damage resulted in permanent cognitive decline, impaired awareness and self care ability. Families felt isolated, and more than half need more support to prevent burn out.

This study investigates the remote effects of botulinum toxin injections by examining the motor unit architecture of noninjected distant muscles. In 21 dystonia patients treated with botulinum toxin (n = 11, mean cumulative dose = 815 mU; n = 10, mean cumulative dose = 7,207 mU) and 10 control individuals, a blinded single-fiber electromyography of the vastus lateralis muscle was performed. The main outcome measure was fiber density (FD), thus measuring the effect of different cumulative doses on remote reinnervation. FD was normal in all patients treated with botulinum toxin. FD did not differ between the three groups studied. No relationship was found between FD and cumulative dose. Therefore, in this specific patient population, muscles remote to the site of injection showed no FD change months after the injection. We conclude that there was no evidence of remote reinnervation and remodelling of motor units with cumulative chemodenervation., (Copyright 2000 S. Karger AG, Basel.)

In 28 patients of a neurological rehabilitation unit of a hospital the use of enteral nutrition via percutaneous endoscopic gastrostomy (PEG) tubes was reviewed. During a total observation period of 5,172 days no life-threatening complications occurred. Minor complications were observed in 12 patients (43%) in the first 2 weeks after the insertion and in 5 patients (18%) afterwards. The nutritional status stabilized in all subjects. Transient PEG feeding was performed in 11 patients (39%) with a mean duration of 150 days. We conclude that hesitation in the application of PEG feeding in neurological rehabilitation should be abandoned. The timing and monitoring of PEG feeding in patients undergoing neurological rehabilitation for acute remitting neurological disorders is discussed.

Focal white-matter lesions in the brain have been frequently found in patients with inflammatory bowel disease. These findings have been discussed as a novel extraintestinal manifestation of inflammatory bowel disease. In this case report we delineate the clinical relevance of such lesions by presenting a patient with ulcerative colitis and his associated neurological symptoms. The cerebral lesions were shown by magnetic resonance imaging, whereupon a successful treatment with corticosteroids was initiated.

Modern rehabilitation is becoming more and more "social integration" instead of "going back to work". Therefore rehabilitation is also a matter in chronic disease and in old people. Parkinson patients are somewhat disabled in nearly every aspect of their life, although the extent is related to the stage of the disease. Moreover, symptoms do not respond equally to drug treatment, balance (with succeeding falls) and swallowing being special problems for anti-parkinsonian drug treatment, but also vegetative symptoms, dysarthria, motor skills etc. Apart from medication patients get relief also from adjuvant therapy like physiotherapy, occupational therapy, and speech therapy, which all can lead to improvement of quality of life. Rehabilitation needs team effort. Patient and family supporting groups (like Parkinson Disease Society and others) are an important factor for all needs of neurological rehabilitation.

Only recently it has become possible to label and visualize dopamine D2 receptors in the living human brain using an [123I]labeled ligand and the SPECT technique. Iodobenzamide (S(-)IBZM) is a substance with high affinity and high specificity for D2 receptors and was used in controls and in patients with different extrapyramidal disorders. After the i.v. administration of 5mCi (185 MBq) of [123I] labeled S(-)IBZM data collection was performed with a rotating double head scintillation camera between 60 and 110 minutes. In a semiquantitative approach a ratio was calculated between mean counts/pixel in the striatum and a region in the lateral frontal cortex, which gives an index of receptor density. In a group of 21 controls this ratio is 1.73 +/- 0.09. A highly significant age-related decline is found in controls and in 57 patients with PD. PD patients without L-DOPA or dopamine agonist treatment do not differ from controls (1.72 +/- 0.09; n = 18), whereas patients under dopaminergic therapy show a significantly lower binding ratio (1.65 +/- 0.13; n = 39; p = 0.017) suggesting receptor downregulation. Comparing the striatum ipsi- and contralateral to clinical symptoms in 18 hemiparkinsonian patients shows slightly but significantly higher values on the contralateral side, indicating receptor supersensitivity (1.71 +/- 0.11 vs. 1.66 +/- 0.09; p = 0.0014). No correlation between D2 receptor binding and clinical stage, duration of disease or duration of dopaminergic therapy exists. Markedly reduced ratios are measured in 7 patients with progressive supranuclear palsy and multiple system atrophies and in 18 patients with Huntington's disease (1.40 +/- 0.09 and 1.37 +/- 0.12 respectively; p = 0.0001).(ABSTRACT TRUNCATED AT 250 WORDS)

78 patients with essential tremor (ET) were investigated to uncover correlation and discrepancies between functional (motor) disabilities and subjective impairment. Various self-rating scales (Zung, v. Zerssen etc.) were used for the assessment of the latter: 2/5 of the patients rated themselves as severely impaired; 1/3 was depressive. Patients who showed nearly the same functional (motor) disability felt very differently subjectively impaired. Semiquantitative clinical scores of action tremor correlated best with the subjective impairment in activities of daily living. Objective measurements of motor disability were performed with the "Motorische Leistungsserie nach Schoppe" (motor performance test) and showed good correlation to the subjective impairment in simple tasks of every day life, such as drinking from a glass, eating soup, and writing. Asking for the subjective impairment in these tasks allows to estimate the objective disability correctly. This may be of value in long-term studies of essential tremor.