Tobacco use and risk of acute stroke in 32 countries in the INTERSTROKE study: a case-control study.

Background: Smoking is a major risk factor for the global burden of stroke. We have previously reported a global population attributable risk (PAR) of stroke of 12.4% associated with current smoking. In this study we aimed to explore the association of current tobacco use with different types of tobacco exposure and environmental tobacco smoke (ETS) exposure on the risk of stroke and stroke subtypes, and by regions and country income levels. Methods: The INTERSTROKE study is a case-control study of acute first stroke and was undertaken with 13,462 stroke cases and 13,488 controls recruited between January 11, 2007 and August 8, 2015 in 32 countries worldwide. Association of risk of tobacco use and ETS exposure were analysed with overall stroke, ischemic and intracerebral hemorrhage (ICH), and with TOAST etiological stroke subtypes (large vessel, small vessel, cardioembolism, and undetermined). Findings: Current smoking was associated with an increased risk of all stroke (odds ratio [OR] 1.64, 95% CI 1.46-1.84), and had a stronger association with ischemic stroke (OR 1.85, 95% CI 1.61-2.11) than ICH (OR 1.19 95% CI 1.00-1.41). The OR and PAR of stroke among current smokers varied significantly between regions and income levels with high income countries (HIC) having the highest odds (OR 3.02 95% CI 2.24-4.10) and PAR (18.6%, 15.1-22.8%). Among etiological subtypes of ischemic stroke, the strongest association of current smoking was seen for large vessel stroke (OR 2.16, 95% CI 1.63-2.87) and undetermined cause (OR 1.97, 95% CI 1.55-2.50). Both filtered (OR 1.73, 95% CI 1.50-1.99) and non-filtered (OR 2.59, 95% CI 1.79-3.77) cigarettes were associated with stroke risk. ETS exposure increased the risk of stroke in a dose-dependent manner, exposure for more than 10 h per week increased risk for all stroke (OR 1.95, 95% CI 1.69-2.27), ischemic stroke (OR 1.89, 95% CI 1.59-2.24) and ICH (OR 2.00, 95% CI 1.60-2.50). Interpretation: There are significant variations in the magnitude of risk and PAR of stroke according to the types of tobacco used, active and ETS exposure, and countries with different income levels. Specific strategies to discourage tobacco use by any form and to build a smoke free environment should be implemented to ease the global burden of stroke. Funding: The Canadian Institutes of Health Research, Heart and Stroke Foundation of Canada, Canadian Stroke Network, Swedish Research Council, Swedish Heart and Lung Foundation, The Health & Medical Care Committee of the Regional Executive Board, Region Västra Götaland, and through unrestricted grants from several pharmaceutical companies with major contributions from Astra Zeneca, Boehringer Ingelheim (Canada), Pfizer (Canada), MERCK, Sharp and Dohme, Swedish Heart and Lung Foundation, UK Chest, and UK Heart and Stroke.

Risk Factors for Stroke in the Young (18-45 Years): A Case-Control Analysis of INTERSTROKE Data from 32 Countries.

BACKGROUND: It is not clear whether conventional vascular risk factors are responsible for most strokes in patients younger than 45 years of age. Our objective was to evaluate the association of common risk factors with stroke in individuals under 45 years. METHODS: INTERSTROKE was a case-control study carried out in 32 countries between 2007 and 2015. Patients presenting within 5 days of symptom onset of a first stroke were enrolled as cases. Controls were age and sex matched to cases and had no history of stroke. Cases and controls underwent similar evaluations. Odds ratios (ORs) and population attributable risks (PARs) were calculated to determine the association of various risk factors with all stroke, ischemic stroke, and intracranial hemorrhage, for patients 45 years of age or younger. FINDINGS: 1,582 case-control pairs were included in this analysis. The mean age of this cohort was 38.5 years (SD 6.32). Overall, 71% strokes were ischemic. Cardiac causes {OR: 8.42 (95% confidence interval [CI]: 3.01-23.5)}; binge drinking of alcohol (OR: 5.44 [95% CI: 1.81-16.4]); hypertension (OR: 5.41 [95% CI: 3.40-8.58]); ApoB/ApoA1 ratio (OR: 2.74 [95% CI: 1.69-4.46]); psychosocial stress (OR: 2.33 [95% CI: 1.01-5.41]); smoking (OR: 1.85 [95% CI: 1.17-2.94]); and increased waist-to-hip ratio (OR: 1.69 [95% CI: 1.04-2.75]) were the most important risk factors for ischemic stroke in these young cases. For intracerebral hemorrhage, only hypertension (OR: 9.08 [95% CI: 5.46-15.1]) and binge drinking (OR: 4.06 [95% CI: 1.27-13.0]) were significant risk factors. The strength of association and population attributable risk (PAR) for hypertension increased with age (PAR 23.3% in those <35 years of age, 50.7% in 35-45 years of age). INTERPRETATION: Conventional risk factors such as hypertension, smoking, binge drinking of alcohol, central obesity, cardiac causes, dyslipidemia, and psychosocial stress are important risk factors for stroke in those younger than 45 years of age. Hypertension is the most significant risk factor in all age groups and across all regions and both stroke subtypes. These risk factors should be identified and modified in early adulthood to prevent strokes in young individuals.

Real-life anti-smoking practices among Polish neurologists.

Purpose: The aim of the present study was to assess attitudes of Polish neurologists towards cigarette smoking and their real-life anti-smoking practices. Methods: A study questionnaire was constructed, and distributed among Polish neurologists (n = 101; 73% females). More than two thirds (70%) of the study group worked in in-patient neurological wards with separate stroke units. Results: Seventy five percent of the study group documented the smoking status of their patients in medical files. Two thirds of the study group collected data on patient's cigarette smoking during each visit. Only 54% and 22% of study participants routinely assessed the severity of tobacco dependence and diagnosed tobacco dependence according to the ICD-10 criteria, respectively. Two thirds of physicians declared routinely using any anti-smoking intervention, but only 12% used the recommended 5'A (Ask, Advice, Assess, Assist, Arrange) model of behavioral intervention and only 11% introduced Evidence Based Medicine (EBM)-supported pharmacotherapy. The vast majority of study participants (80%) did not try to increase their professional skills in anti-smoking interventions. Conclusions: Real-life anti-smoking practices among Polish neurologists are generally unsatisfactory and do not follow EBM-based guidelines. The low percentage of neurologists who diagnose and treat nicotine dependence may negatively impact the efficacy of secondary stroke prevention in Poland.

Association of adiponectin, leptin and resistin with inflammatory markers and obesity in dementia.

The aim of the study was to determine the role of adiponectin, leptin and resistin in various types of dementia and to investigate their association with inflammatory markers, insulin resistance and abdominal obesity. In 205 patients with dementia [89 with Alzheimer's disease (AD), 47 with vascular dementia (VaD), 69 with mixed dementia (MD)], 113 persons with mild cognitive impairment and in 107 controls serum adiponectin, leptin and resistin levels, pro-inflammatory [interleukin-6 (IL-6), C-reactive protein (hsCRP) and chitotriosidase] and anti-inflammatory (25-OH vitamin D, HDL-cholesterol and paraoxonase 1) markers, as well as glucose metabolism parameters (glucose, insulin and HOMA-IR) were determined. In all-cause dementia adiponectin and resistin levels were significantly higher as compared to the controls; leptin levels did not show differences. Higher adiponectin levels concerned AD and MD, whereas higher resistin-VaD and MD. After stratification by abdominal obesity the differences in adiponectin levels remained significant in subjects without obesity. In all-cause dementia negative correlation of adiponectin with obesity, glucose metabolism parameters, IL-6 and hsCRP and positive correlation with HDL-cholesterol were found. Positive correlation of resistin with age, IL-6, hsCRP and chitotriosidase and negative correlation with HDL-cholesterol and paraoxonase 1 were stated. We conclude that dementia of neurodegenerative origin is characterized by elevated adiponectin levels, whereas dementia with vascular changes by increase of resistin. Association with inflammatory indicators may suggest the pro-inflammatory role of resistin in the development of dementia, especially in dementia of vascular mechanism. Identification of this novel biomarker may be important in preventing dementia.

An unusual presentation of Listeria monocytogenes rhombencephalitis.

We describe a case of 52-year-old woman with a medical history of Crohn's disease presented abrupt fever, asymmetrical multiple cranial nerve palsies and focal neurological symptoms localized to the brainstem. The patient was initially diagnosed with ischaemic stroke, because of acute clinical course and results of neuroimaging. Cerebrospinal fluid analysis revealed mild infection with negative Gram staining and culture. Final diagnosis of Listeria monocytogenes brainstem infection (rhombencephalitis) was set up on the basis of further clinical course and positive blood cultures. Listerial rhombencephalitis should be kept in mind in immunocompromised adult patients who develop fever, asymmetrical multiple cranial nerve palsies and focal neurological symptoms localized to the brainstem even without typical neuroimaging, cerebrospinal fluid findings and negative cultures. Early diagnosis and adequate antibiotic treatment is of crucial importance.

Global and regional effects of potentially modifiable risk factors associated with acute stroke in 32 countries (INTERSTROKE): a case-control study.

BACKGROUND: Stroke is a leading cause of death and disability, especially in low-income and middle-income countries. We sought to quantify the importance of potentially modifiable risk factors for stroke in different regions of the world, and in key populations and primary pathological subtypes of stroke. METHODS: We completed a standardised international case-control study in 32 countries in Asia, America, Europe, Australia, the Middle East, and Africa. Cases were patients with acute first stroke (within 5 days of symptom onset and 72 h of hospital admission). Controls were hospital-based or community-based individuals with no history of stroke, and were matched with cases, recruited in a 1:1 ratio, for age and sex. All participants completed a clinical assessment and were requested to provide blood and urine samples. Odds ratios (OR) and their population attributable risks (PARs) were calculated, with 99% confidence intervals. FINDINGS: Between Jan 11, 2007, and Aug 8, 2015, 26 919 participants were recruited from 32 countries (13 447 cases [10 388 with ischaemic stroke and 3059 intracerebral haemorrhage] and 13 472 controls). Previous history of hypertension or blood pressure of 140/90 mm Hg or higher (OR 2·98, 99% CI 2·72-3·28; PAR 47·9%, 99% CI 45·1-50·6), regular physical activity (0·60, 0·52-0·70; 35·8%, 27·7-44·7), apolipoprotein (Apo)B/ApoA1 ratio (1·84, 1·65-2·06 for highest vs lowest tertile; 26·8%, 22·2-31·9 for top two tertiles vs lowest tertile), diet (0·60, 0·53-0·67 for highest vs lowest tertile of modified Alternative Healthy Eating Index [mAHEI]; 23·2%, 18·2-28·9 for lowest two tertiles vs highest tertile of mAHEI), waist-to-hip ratio (1·44, 1·27-1·64 for highest vs lowest tertile; 18·6%, 13·3-25·3 for top two tertiles vs lowest), psychosocial factors (2·20, 1·78-2·72; 17·4%, 13·1-22·6), current smoking (1·67, 1·49-1·87; 12·4%, 10·2-14·9), cardiac causes (3·17, 2·68-3·75; 9·1%, 8·0-10·2), alcohol consumption (2·09, 1·64-2·67 for high or heavy episodic intake vs never or former drinker; 5·8%, 3·4-9·7 for current alcohol drinker vs never or former drinker), and diabetes mellitus (1·16, 1·05-1·30; 3·9%, 1·9-7·6) were associated with all stroke. Collectively, these risk factors accounted for 90·7% of the PAR for all stroke worldwide (91·5% for ischaemic stroke, 87·1% for intracerebral haemorrhage), and were consistent across regions (ranging from 82·7% in Africa to 97·4% in southeast Asia), sex (90·6% in men and in women), and age groups (92·2% in patients aged ≤55 years, 90·0% in patients aged >55 years). We observed regional variations in the importance of individual risk factors, which were related to variations in the magnitude of ORs (rather than direction, which we observed for diet) and differences in prevalence of risk factors among regions. Hypertension was more associated with intracerebral haemorrhage than with ischaemic stroke, whereas current smoking, diabetes, apolipoproteins, and cardiac causes were more associated with ischaemic stroke (p<0·0001). INTERPRETATION: Ten potentially modifiable risk factors are collectively associated with about 90% of the PAR of stroke in each major region of the world, among ethnic groups, in men and women, and in all ages. However, we found important regional variations in the relative importance of most individual risk factors for stroke, which could contribute to worldwide variations in frequency and case-mix of stroke. Our findings support developing both global and region-specific programmes to prevent stroke. FUNDING: Canadian Institutes of Health Research, Heart and Stroke Foundation of Canada, Canadian Stroke Network, Health Research Board Ireland, Swedish Research Council, Swedish Heart and Lung Foundation, The Health & Medical Care Committee of the Regional Executive Board, Region Västra Götaland (Sweden), AstraZeneca, Boehringer Ingelheim (Canada), Pfizer (Canada), MSD, Chest, Heart and Stroke Scotland, and The Stroke Association, with support from The UK Stroke Research Network.

Midbrain and bilateral paramedian thalamic stroke due to artery of Percheron occlusion.

INTRODUCTION: Bilateral thalamic strokes are rare manifestations of posterior circulation infarcts. Usually the etiology is cardioembolic or small vessel disease combined with individual anatomical predisposition. The symptoms include a variety of neurological deficits depending on thalamic structure involvement, such as paresthesias or numbness, hemiparesis with increased reflexes and Babinski sign, third cranial nerve palsy, speech and cognition disturbance, memory impairment and stupor. Neuroimaging usually reveals ischemic loci in adequate thalamic nuclei. CASE PRESENTATION: We report a case of 61-year-old man, active smoker (25/per day, 50 pack-years) with untreated hypertension who presented at admission consciousness impairment (Glasgow Coma Scale score 9 points), left pupil dilatation without reaction to light, left eye deviation downwards and outwards, vertical gaze paralysis and left-sided hemiplegia. Initial brain computed tomography (CT) was normal. Brain magnetic resonance with diffusion weighted imaging and fluid attenuation inversion recovery sequences (MR DWI/FLAIR) performed on admission showed ischemic changes in bilateral thalami, which were confirmed in routine MRI. Thrombosis of basilar artery and cerebral venous was excluded in CT angiography. Further diagnostic assessment revealed hyperlipidemia, paroxysmal atrial fibrillation and renal cancer with hepatic metastases. CONCLUSION: Bilateral thalamic stroke due to artery of Percheron occlusion is a rare presentation of stroke, which can be overlooked in routine CT scan. If diagnosed, it requires further evaluation for stroke risk factors, especially cardiovascular disorders associated with increased embolic risk.

Readiness Visual Analog Scale: A Simple Way to Predict Post-Stroke Smoking Behavior.

BACKGROUND AND PURPOSE: The aim of the present study was to assess a relationship between readiness to quit and post-stroke smoking behavior. METHODS: Eighty-six active smokers with first-ever ischemic stroke were recruited in a tertiary-care stroke unit. The question "Are you ready to quit smoking within the next month?" with yes/no responses and the 10-cm readiness visual analog scale (VAS) was administered during the anti-smoking intervention. Smoking status was verified at the 3- and 12-month follow-up. RESULTS: The readiness VAS score at hospitalization was significantly lower in patients classified as smokers as compared to patients classified as non-smokers. The readiness score <5 cm was a significant predictor of smoking at the 3-month (OR, 7.3) and 12-month follow-up (OR, 4.9). CONCLUSIONS: The present results suggest that the readiness VAS can be used as a simple and inexpensive instrument for early identification of patients who continue to smoke after stroke.

Morphological changes of skeletal muscle in spinal and bulbar muscular atrophy (SBMA), Kennedy's disease: a case report.

Spinal and bulbar muscular atrophy (SBMA, Kennedy's disease) is an X-linked recessive disease affecting lower motor neurons. In the present case report, we describe morphological changes in a muscle biopsy obtained from a 62-year-old patient with gynecomastia and with the following neurological symptoms: dysphagia, dysarthria, wasting and fasciculation of the tongue, proximal weakness, fasciculations in the limb muscles, and an absence of all tendon reflexes. Neurogenic alternations were predominantly observed using light and electron microscopy. The angulated atrophic muscle fibers formed bundles. The numerous nuclei were pyknotic or pale, some of them were also ubiquitin positive; they were grouped inside so-called "nuclear sacks". At the ultrastructural level, atrophic muscle fibers revealed disruption and loss of sarcomeres, duplication of Z-line, and rod-like structures. The nuclei, often with irregular shapes, revealed varying degrees of chromatin condensation, from dispersed to highly condensed, like pyknotic nuclei. Occasionally electron-dense inclusions in the nuclei were found. Some myogenic features like hypertrophic muscle fibers and proliferation of connective tissue were also visible. The neurogenic and myogenic pathological changes suggested SBMA, which was confirmed with genetic analysis (trinucleotide CAG (glutamie)-repeat expansion in the androgen-receptor gene).

Paraoxonase 1 activity and level of antibodies directed against oxidized low density lipoproteins in a group of an elderly population in Poland - PolSenior study.

UNLABELLED: The aim of this study was to assess two factors influencing the amount of oxidized LDL-paraoxonase 1 (PON1) activity and the level of anti-oxidized LDL antibodies (anti-ox LDL) in a large group of elderly individuals in Poland. The effects of cognitive status, hypertension and metabolic syndrome and of selected serum lipids and inflammation indicators on PON1 activity and anti-ox LDL level were also examined. The investigated population consisted of 3154 individuals aged 65 and more - participants of the population-based PolSenior project. PON1 arylesterase activity was determined spectrophotometrically, anti-ox-LDL antibodies using ELISA method. PON1 activity significantly decreased with advancing age, was lower in males than in females and decreased in persons with impaired cognition. Individuals with hypertension and high lipid levels showed higher PON1 activity. Lower PON1 activity was related to higher level of inflammation indicators - hsCRP and IL-6. The significant association of PON1 activity with age, HDL-C, LDL-C, sex and IL-6 was confirmed in multivariate analysis. Anti-ox LDL antibodies level was significantly higher in the two oldest subgroups of males. It was significantly lower in males than in females. It was decreased in persons with higher serum triglycerides. No relationship of anti-ox LDL level with cognition, hypertension, metabolic syndrome, inflammation indicators and serum lipid levels was observed. In some persons very high levels of anti-ox LDL were stated, most frequently in the oldest persons, particularly in men. CONCLUSION: Both investigated antioxidant factors - PON1 activity and anti-ox LDL level, could play an important role in aging.

Reasons for quitting smoking in patients with first-ever ischemic stroke.

Ninety-eight cigarette smokers with ischemic stroke were recruited between December 2006 and December 2008 in an urban hospital. Smoking status and reasons for quit attempts after stroke were assessed at 3-month follow-up. 73% of patients (72/98) made at least one quit attempt between stroke onset and the follow-up visit. 47% of quit attempters (34/72) declared that stroke was the major reason for quitting. The patients reporting stroke as the major reason for quitting were more likely to be abstinent at the follow-up as compared to the patients who did not (61.8 vs. 36.8%). The study suggests that some motives for quitting smoking are associated with a higher chance for short-term abstinence in stroke patients.

Risk factors for ischaemic and intracerebral haemorrhagic stroke in 22 countries (the INTERSTROKE study): a case-control study.

BACKGROUND: The contribution of various risk factors to the burden of stroke worldwide is unknown, particularly in countries of low and middle income. We aimed to establish the association of known and emerging risk factors with stroke and its primary subtypes, assess the contribution of these risk factors to the burden of stroke, and explore the differences between risk factors for stroke and myocardial infarction. METHODS: We undertook a standardised case-control study in 22 countries worldwide between March 1, 2007, and April 23, 2010. Cases were patients with acute first stroke (within 5 days of symptoms onset and 72 h of hospital admission). Controls had no history of stroke, and were matched with cases for age and sex. All participants completed a structured questionnaire and a physical examination, and most provided blood and urine samples. We calculated odds ratios (ORs) and population-attributable risks (PARs) for the association of all stroke, ischaemic stroke, and intracerebral haemorrhagic stroke with selected risk factors. FINDINGS: In the first 3000 cases (n=2337, 78%, with ischaemic stroke; n=663, 22%, with intracerebral haemorrhagic stroke) and 3000 controls, significant risk factors for all stroke were: history of hypertension (OR 2.64, 99% CI 2.26-3.08; PAR 34.6%, 99% CI 30.4-39.1); current smoking (2.09, 1.75-2.51; 18.9%, 15.3-23.1); waist-to-hip ratio (1.65, 1.36-1.99 for highest vs lowest tertile; 26.5%, 18.8-36.0); diet risk score (1.35, 1.11-1.64 for highest vs lowest tertile; 18.8%, 11.2-29.7); regular physical activity (0.69, 0.53-0.90; 28.5%, 14.5-48.5); diabetes mellitus (1.36, 1.10-1.68; 5.0%, 2.6-9.5); alcohol intake (1.51, 1.18-1.92 for more than 30 drinks per month or binge drinking; 3.8%, 0.9-14.4); psychosocial stress (1.30, 1.06-1.60; 4.6%, 2.1-9.6) and depression (1.35, 1.10-1.66; 5.2%, 2.7-9.8); cardiac causes (2.38, 1.77-3.20; 6.7%, 4.8-9.1); and ratio of apolipoproteins B to A1 (1.89, 1.49-2.40 for highest vs lowest tertile; 24.9%, 15.7-37.1). Collectively, these risk factors accounted for 88.1% (99% CI 82.3-92.2) of the PAR for all stroke. When an alternate definition of hypertension was used (history of hypertension or blood pressure >160/90 mm Hg), the combined PAR was 90.3% (85.3-93.7) for all stroke. These risk factors were all significant for ischaemic stroke, whereas hypertension, smoking, waist-to-hip ratio, diet, and alcohol intake were significant risk factors for intracerebral haemorrhagic stroke. INTERPRETATION: Our findings suggest that ten risk factors are associated with 90% of the risk of stroke. Targeted interventions that reduce blood pressure and smoking, and promote physical activity and a healthy diet, could substantially reduce the burden of stroke. FUNDING: Canadian Institutes of Health Research, Heart and Stroke Foundation of Canada, Canadian Stroke Network, Pfizer Cardiovascular Award, Merck, AstraZeneca, and Boehringer Ingelheim.

Insular lesions and smoking cessation after first-ever ischemic stroke: a 3-month follow-up.

Little is known about biological factors involved in post-stroke smoking cessation. A recent retrospective study has indicated a possible association between unilateral insular lesions of various origin and a rapid disruption of nicotine addiction. Given the above, the aim of the present prospective study was to assess a relationship between post-stroke smoking cessation and lesion localization. Eighty-seven patients with first-ever, unilateral, ischemic stroke who had been active smokers at stroke onset were examined during hospitalization and at 3-month follow-up. No association was found between any specific lesion localization (the insula, operculum, striatum, thalamus, internal capsule, brainstem) and smoking status at the 3-month follow-up visit. Patients with lacunar circulation strokes (LACS) were more likely to be non-smokers at the follow-up examination. No sudden disruption of nicotine addition was observed in patients with insular or other stroke locations. Concluding, post-stroke smoking cessation may not be associated with insular lesions.

[Predictors of smoking cessation after stroke]. / Predyktory rzucania palenia po udarze mózgu.

Cigarette smoking is a major modifiable risk factor for stroke. Smoking dose dependently increases the risk of stroke, especially in patients below 75 years of age. Although smoking cessation is considered as one of the most effective methods of secondary stroke prevention, little is known about nicotine dependence and predictors of smoking cessation after stroke. Identification of such predictors could facilitate the development of anti-smoking interventions in post-stroke patients. Results of previous studies showed that smoking cessation is determined by the interplay of multiple factors, including sociodemographic (gender, age, race, living conditions, employment), clinical (functional status), psychobiological (nicotine dependence, depressed mood) and environmental (smoking household members) factors. Limitations of most studies were relatively small sample sizes and lack of verification of smoking status with a biochemical marker (e.g. expired CO). The aim of this article is to summarize current knowledge about predictors of smoking cessation after stroke.

Predictors of smoking abstinence after first-ever ischemic stroke: a 3-month follow-up.

BACKGROUND AND PURPOSE: Predictors of smoking abstinence in stroke survivors remain largely unexplored. The present study addressed the relationship between degrees of nicotine dependence and smoking abstinence 3 months after ischemic stroke. METHODS: One hundred smokers with first-ever ischemic stroke were prospectively enrolled to the study. Correlates of nicotine dependence as well as sociodemographic and clinical characteristics were assessed during hospitalization. Smoking status was determined at 3-month follow-up. RESULTS: Significant predictors of smoking abstinence at follow-up included: the Fagerström Test for Nicotine Dependence score, the Barthel Index, the number of smoking household members, and the Geriatric Depression Scale score. CONCLUSIONS: Our results suggest that smoking cessation after ischemic stroke can be determined by the interplay of psychobiological and environmental factors.

Adult glycogenosis type II (Pompe's disease): morphological abnormalities in muscle and skin biopsies compared with acid alpha-glucosidase activity.

Glycogen storage disease type II (GSD II) is an autosomal recessive deficiency of acidalpha-1,4-glucosidase(GAA) caused by mutations in the GAA gene located on human chromosome 17 (17q 25.2-q 25.3). Although its pathophysiology is partially understood, it has not yet been elucidated whether the level of GAA deficiency is directly proportional to the level of glycogen storage, vacuolar degeneration and/or GSD II severity. Muscle and skin biopsies were taken from three female patients with symptoms of progressive muscle weakness and respiratory failure: patient 1 aged 19, as well as patients 2 and 3 (two sisters) aged 31 and 29, respectively. Initial clinical manifestations, respiratory failure and muscle weakness, were similar in all the examined patients, while their character and intensity differed. For each examined patient, the activity of lysosomal GAA (at pH 3.8) was measured fluorometrically in isolated blood leukocytes (L) and dried blood spots (DBS). Biopsy samples were studied histologically, immunohistologically and ultrastructurally. Each of them displayed similar morphological features, although with different intensity. Muscle fibres were irregular in size with smaller non-rounded fibres and vacuolar degeneration. Invacuoles,we observed glycogen and intense positive ubiquitin reaction. Myofibrils were almost completely destroyed by the accumulation of glycogen granules in lysosomes and those free, without limiting membranes as well as by vacuoles of various size. Autophagic vacuoles were visible occasionally. Excess glycogen was also present in the walls of muscle and skin capillaries. All three patients showed reduced GAA activity ratios measured at pH 3.8 with and without acarbose (patient 1 - 0.12 in DBS and 0.07 in L; patient 2 - 0.05 in DBS and 0.07 in L; and patient 3 - 0.12 in DBS and 0.09 in L). Based on the study results, we concluded that GAA deficiency in vitro in late-onset type II glycogenosis was not directly proportional to the amount of glycogen storage, vacuolar degeneration and disease severity.

Acute ischemic stroke care and outcome in centers participating in the Polish National Stroke Prevention and Treatment Registry.

BACKGROUND AND PURPOSE: Significant intercenter variability in quality of care and stroke outcomes was found in many countries. The aim of the study was to compare the acute ischemic stroke care and outcomes in centers participating in the Polish National Stroke Prevention and Treatment Registry. METHODS: The World Health Organization Stepwise Approach to Stroke Surveillance-based questionnaire was used to collect data on patients admitted to participating centers between December 1, 2001, and July 31, 2002. To ensure data quality, only centers reporting representative sample of patients were analyzed. Ischemic stroke patient characteristics, in-hospital care, and early outcomes (adjusted for case mix) were compared for participating centers. RESULTS: There were 26 of 48 centers that met inclusion criteria, with a total of 8736 patients (52% women; mean age 71 years, with a range among institutions from 68 to 75 years). Significant differences between centers were observed for distribution of risk factors and in-hospital care. The rates for death and poor outcome (defined as a Rankin score > or =3 or death) ranged from 8.0% to 31.8% and from 44.2% to 74.7%, respectively. After adjusting for case mix, the death or poor outcome prognoses remained significantly different between centers. CONCLUSIONS: The observed significant differences between Polish stroke centers indicate the need for improvement of patient education, effective stroke risk factor control, and standardized in-hospital care.

The polish experience in early stroke care.

Stroke units are being organised in Poland since 1997. To ensure easy access to stroke care, 120 specialised units need to be created. There were 23 stroke units in Poland in the year 2000. Only 12 of them fulfilled the criteria of a standard stroke unit. Significant differences in stroke fatality were noted between well- and as yet insufficiently organised units. These results indicate a need for monitoring the implementation of the basic standards in stroke units.