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Regional differences in acute ischemic stroke (AIS) care are still reported for Germany but not fully understood. We aimed to analyze the impact of the level of urbanization on the application of proved treatment procedures and the incidence of hospitalized AIS cases in Germany.The level of urbanization of the 401 districts and district-free cities in Germany was defined according to the Federal Institute for Building, Urban Affairs and Spatial Research classification. Using nationwide administrative data, the application of intravenous thrombolysis (IVT), mechanical thrombectomy (MT), and the incidence of hospitalizations for AIS, were analyzed according to the place of residence of the patients. Data included all patients hospitalized for AIS in 2019 (n = 224,274) and sociodemographic information of 83,019,213 residents in Germany.Sparsely populated districts had a significantly higher incidence of hospitalizations for AIS than cities (age-standardized: 271.8 versus 242.3 per 100,000 inhabitants, P 0.001), but a lower use of MT (5.7% versus 8.4%, P 0.001). In the adjusted negative binomial regression model, rurality remained an independent predictor for MT use and the incidence of AIS hospitalizations. The proportion of IVT did not significantly differ between sparsely populated districts and cities (15.6% versus 17.2%, P = 0.021).Although there is a dense network of stroke units in Germany, the level of urbanization still influences AIS care, as we found a lower proportion of MT and a higher incidence of AIS hospitalizations in Germany's rural areas.

Objectives The clinical spectrum of amyotrophic lateral sclerosis (ALS) is characterized by a considerable variation. Different phenotypes have been described by previous studies. We assessed clinical variability and prognostic relevance of these phenotypes in a prospective, population-based cohort of ALS patients in Rhineland-Palatinate, Germany. Methods Incident ALS cases, diagnosed between October 2009 and September 2012, were prospectively enrolled and classified according to established ALS phenotype classification (bulbar, classic, flail arm, flail leg, pyramidal, respiratory). Survival probability was described using Kaplan–Meier method. Moreover, the influence of an additional frontotemporal dementia (FTD) was analysed. Results Phenotypes of all 200 patients were determined. Bulbar and classic phenotypes accounted for 75% of all cases. Deterioration of functional impairment during disease progression was lowest in flail leg and pyramidal variants, and most pronounced in bulbar and classic phenotypes. A poor survival prognosis was observed for bulbar, classic or respiratory phenotypes. Patients with an additional FTD showed an even worse outcome. Conclusions Results suggest that ALS is a heterogeneous disease, as ALS phenotypes differ in disease progression and survival time. Patients classified as suffering from bulbar, classic and respiratory ALS, as well as those with an additional FTD, show a marked reduction of survival time.

We report a novel heteroplasmic point mutation G8299A in the gene for mitochondrial tRNA Lys in a patient with progressive external ophthalmoplegia complicated by recurrent respiratory insufficiency. Biochemical analysis of respiratory chain complexes in muscle homogenate showed a combined complex I and IV deficiency. The transition does not represent a known neutral polymorphism and affects a position in the tRNA acceptor stem which is conserved in primates, leading to a destabilization of this functionally important domain. In vitro analysis of an essential maturation step of the tRNA transcript indicates the probable pathogenicity of this mutation. We hypothesize that there is a causal relationship between the novel G8299A transition and progressive external ophthalmoplegia with recurrent respiratory failure due to a depressed respiratory drive.

Activated monocytes may contribute to the pathogenesis of ischemic stroke. We tested the hypothesis that release products and procoagulant activity of monocytes are increased in acute ischemic stroke. In patients on days 1, 3 and 7 after ischemic stroke and in age- and sex-matched healthy control subjects, we assessed plasma levels of interleukin 8 (IL-8) and neopterin (enzyme linked immunosorbent assay, ELISA) and investigated superoxidanion release (ferricytochrome C reduction), procoagulant activity (one-stage clotting assay) and tissue factor (TF) gene transcription (reverse transcriptase polymerase chain reaction) by monocytes. As compared to control subjects (n=23), IL-8 levels were increased on day 1 after stroke (n=22; p=0.005) and remained elevated on days 3 and 7. Neopterin levels were elevated on days 3 and 7 (p0.05, respectively) but not on day 1. Neopterin and IL-8 were not correlated with monocyte counts. Superoxid anion production by stimulated and unstimulated monocytes was not different between groups. TF mRNA could neither be detected in monocytes from patients investigated within 12 h after ischemia (n=12) nor in control subjects (n=10) and procoagulant activity of cells was similar in both groups. Our results indicate increased monocyte activation after ischemic stroke although not all activation parameters were elevated. We found no support for the hypothesis that circulating monocytes express TF and possess increased procoagulant activity. Elevated IL-8 may contribute to stroke pathophysiology by activating polymorphonuclear leukocyte (PMNL) activation early after ischemia.

Chronic infection may increase the risk for ischemic stroke. Presently, it is insufficiently established whether Helicobacter pylori infection represents a risk factor for ischemic stroke. We analyzed IgG antibodies against H. pylori in 109 patients with acute cerebral ischemia and 82 age- and sex-matched control patients with non-vascular and non-inflammatory neurological diseases. Antibody titers were significantly higher in patients than in control subjects ( p =0.007). H. pylori seropositivity tended to be more common in patients (odds ratio (OR) 1.55, 95% confidence interval (ci) 0.87–2.76), but this trend was further attenuated in multivariate analysis (OR 1.42; 95% 0.75–2.67) with hypertension, diabetes mellitus, current or previous smoking, previous cerebral ischemia and low socioeconomic status. H. pylori seropositivity increased the odds for cerebral ischemia of atherothrombotic origin in univariate (OR 3.63; 95% ci 1.37–9.65) and multivariate analysis (OR 3.53; 95% ci 1.09–11.4). H. pylori seropositivity may be an independent risk factor for stroke of atherothrombotic origin.

Previous studies showed that elevated body temperature early after ischemic stroke is associated with severe neurological deficit and a poor outcome. The aim of this study was to analyse the prevalence and putative etiology of febrile body temperature (>/=38.0 degrees C) early after stroke and to investigate the association between body temperature, stroke severity and outcome. We investigated 119 consecutive patients who were admitted within 24 h after ischemic stroke. Patients were examined for infection before ischemia using a standardized questionnaire and received daily clinical examination after stroke. In case of fever, standardized radiological and microbiological examinations were performed. Fever within 48 h after stroke was observed in 30 (25.2%) patients. The probable cause of fever was infective or chemical aspiration pneumonia (n=12), other respiratory tract infection (n=7), urinary tract infection (n=4), viral infections (n=3) or insufficiently defined (n=5). (One patient had two potential causes of fever.) In thirteen of these patients, infection was most probably acquired before stroke. Fever newly developed more often during day 1 to 2 than day 3 to 7 after stroke (P=0.016). Fever was associated with a more severe deficit on admission independent from age, vascular diseases and risk factors (odds ratio 9.6; 95% confidence interval 3.1-29). Fever is a frequent complication early after stroke and in the majority of cases, it can be explained by infection or chemical aspiration pneumonia. In about half of the infected patients, infection was most probably acquired before stroke. Fever was associated with a more severe neurological deficit on admission.

Background and purpose This study aimed to characterize the time course of inflammatory parameters after acute ischemic stroke. Methods We serially determined high sensitivity C-reactive protein (CRP), fibrinogen, and leukocyte counts at 10 time points between days 1 and 90 after ischemic stroke and in control subjects. Results CRP did not significantly change, whereas fibrinogen increased after stroke. At all time points, CRP and fibrinogen were higher than in healthy control subjects, but not risk factor control subjects. The leukocyte count declined after stroke and was significantly elevated as compared to both control groups only on day 1 but not later. NIHSS levels were positively correlated with CRP and fibrinogen at all time points. Larger infarcts were associated with a higher CRP and leukocyte counts on day 90. Treatment with aspirin was associated with lower values for all three inflammatory parameters in the subacute phase after ischemia. Conclusions The course after stroke was different between the parameters of inflammation. Only the leukocytes followed the paradigm of an acute phase response.

During the terminal incubation period of severe mumps infection, a 40-year-old patient suffered from large infarction in the right middle cerebral artery territory. The proximal right internal carotid artery (ICA) was occluded on angiography. Computed tomography of the neck detected a hemorrhage located ventromedial to the right common carotid artery. Four months later the right ICA was partly recanalized. Carotid surgery revealed an atherosclerotic plaque and a vessel wall, which was fragile and less compact than usually. A strong inflammatory reaction to mumps infection may have contributed to the pathogenesis of the cervical hemorrhage and to acute thrombosis and occlusion of the ICA.

Tay-Sachs disease is a genetically determined neurodegenerative disorder, resulting from mutations of the hexosaminidase (Hex) A gene coding for the alpha-subunit of beta-D-N-acetyl-hexosaminidase. Clinically, there is severe encephalomyelopathy leading to death within the first few years of life. Hex A activity is usually absent in tissue and body fluids of these patients. Juvenile and adult Hex A deficiencies are less severe but rare variants with some residual Hex A activity. All these variants are most prevalent among Ashkenazi Jews. We describe a non-Jewish family in which four adult brothers and sisters had markedly reduced Hex A activities and onset of symptoms in the second decade of life. The phenotypical expression was remarkably homogeneous, consisting in a combination of slowly progressive motor neuron disease, ataxia and ocular motor disturbances. None of the patients were demented at this stage of their illness. Magnetic resonance studies showed severe cerebellar atrophy, but were otherwise normal. Hex A deficiency was established by biochemical measurements in the serum and skin fibroblasts using the fluorogenic substrates 4-MUG and 4-MUGS as well as by gel electrophoresis. Molecular genetic studies revealed that the patients are compound heterozygotes for the 'adult' mutation Gly269 --> Ser and the 'infantile' 4-base insertion in exon 11 of the Hex A gene.