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Introduction: There is uncertainty regarding the optimal timing for initiation of oral anticoagulation in patients with acute ischemic stroke (AIS) due to atrial fibrillation (AF). Methods: We performed a systematic review and meta-analysis of randomized-controlled clinical trials (RCTs) and prospective observational studies to assess the efficacy and safety of early anticoagulation in AF-related AIS (within 1 week versus 2 weeks). A second comparison was performed assessing the efficacy and safety of direct oral anticoagulants (DOACs) versus vitamin-K antagonists (VKAs) in the two early time windows. The outcomes of interest were IS recurrence, all-cause mortality, symptomatic intracerebral haemorrhage (sICH) and any ICH. Results: Eight eligible studies (6 observational, 2 RCTs) were identified, including 5616 patients with AF-related AIS who received early anticoagulation. Patients that received anticoagulants within the first week after index stroke had similar rate of recurrent IS, sICH and all-cause mortality compared to patients that received anticoagulation within two weeks (test for subgroup differences p = 0.1677; p = 0.8941; and p = 0.7786, respectively). When DOACs were compared to VKAs, there was a significant decline of IS recurrence in DOAC-treated patients compared to VKAs (RR: 0.65; 95%CI: 0.52-0.82), which was evident in both time windows of treatment initiation. DOACs were also associated with lower likelihood of sICH and all-cause mortality. Conclusions: Early initiation of anticoagulation within the first week may have a similar efficacy and safety profile compared to later anticoagulation (within two weeks), while DOACs seem more effective in terms of IS recurrence and survival compared to VKAs. info:eu-repo/semantics/publishedVersion

The aim of this study was to present the descriptive characteristics of the Stroke Units Necessity for Patients (SUN4P) registry.The study population derived from the web-based SUN4P registry included 823 patients with first-ever acute stroke. Descriptive statistics were used to present patients' characteristics.The vast majority of patients (80.4%) had an ischemic stroke, whereas 15.4% had a hemorrhagic stroke. Hypertension was the leading risk factor in both patients. The patients with ischemic stroke had higher prevalence of traditional cardiovascular risk factors such as diabetes mellitus, dyslipidemia and smoking and most commonly cryptogenic stroke (39%). National Institutes of Health Stroke Scale (NIHSS) was higher among patients with hemorrhagic in comparison to those with ischemic stroke (10.5 vs 6 respectively). Moreover, all patients had similar rate of disability prior to stroke, as shown by Modified Rankin Scale (mRS=0).These data are in accordance with current evidence and should be thoroughly assessed in order to ensure optimal therapeutic management of stroke patients.

The aim of this study was to calculate the average operational cost per sub-type of stroke patient and to investigate cost drivers (e.g. ALoS, NIHSS score, age) correlated to cost.Direct medical costs (diagnostic imaging and clinical laboratory exams, overheads/bed cost, pharmaceuticals, ringers and other non-durables and inpatient rehabilitation) per patient were calculated from the providers' (hospitals') perspective. Resource use data derived from the "SUN4P" web-based registry and unit costs were retrieved from publically available sources and were assigned to resource use.The sample comprised 6,282 inpatient days of 750 patients (mean age: 75.5±13.3 years) admitted from July 2019 to July 2021, in nine public hospitals. Mean length of stay was 8.4±7.6 days and mean total operational cost was calculated to €1,239.4 (from which 45% and 35% related to diagnostic exams and overheads/bed cost respectively). Mean cost related to hemorrhagic stroke patients that were discharged alive was calculated significantly higher compared to mean cost related to ischemic stroke patients who didn't undertake thrombolysis and were also discharged alive from the hospital (€2,155.2 vs. €945.2, p0.001). Linear regression analysis revealed that length of stay was significantly correlated with cost (coefficient beta=232, 95% CI confidence interval = 220-243, p0.001).These findings are in accordance with current evidence and should be thoroughly assessed to rationalize inpatient reimbursement rates in order to achieve improved value of care.

To assess stroke patient-reported experiences and hospital staff experiences, during hospital stay.Stroke patient-reported experiences (n=387) were recorded using the translated and culturally adapted NHS-Stroke Questionnaire into Greek and staff experiences (n=236) were investigated using the Compassion Satisfaction and Burnout subscales of the ProQOL questionnaire.Staff's mean compassion satisfaction score was 39.2 (SD=6.3) and mean burnout score was 24.3 (SD=5.6). Only 38.5% of the staff stated that there is smooth cooperation with healthcare professionals of other specialties/disciplines. Personnel working in an NHS Hospital was more satisfied and less burned-out when compared to personnel working at a University Hospital (p=0.02 and p0.001, respectively). Mean total patient-reported experiences score was 81.9 (SD=9.5). Bivariate analysis revealed statistically significant differences for total patient-reported experiences among the eight study hospitals (p0.001).Health policy planners and decision-makers must take into consideration the results of such self-reported measures to establish innovative techniques to accomplish goals such as staff-specialization, continuous training and applying formal frameworks for efficient cooperation amongst different disciplines.

To assess stroke patient-reported experiences and hospital staff experiences, during hospital stay. Methods: Stroke patient-reported experiences (n=387) were recorded using the translated and culturally adapted NHS-Stroke Questionnaire into Greek and staff experiences (n=236) were investigated using the Compassion Satisfaction and Burnout subscales of the ProQOL questionnaire. Results: Staff’s mean compassion satisfaction score was 39.2 (SD=6.3) and mean burnout score was 24.3 (SD=5.6). Only 38.5% of the staff stated that there is smooth cooperation with healthcare professionals of other specialties/disciplines. Personnel working in an NHS Hospital was more satisfied and less burned-out when compared to personnel working at a University Hospital (p=0.02 and p0.001). Conclusions: Health policy planners and decision-makers must take into consideration the results of such self-reported measures to establish innovative techniques to accomplish goals such as staff-specialization, continuous training and applying formal frameworks for efficient cooperation amongst different disciplines.

The aim of this study was to present the descriptive characteristics of the Stroke Units Necessity for Patients (SUN4P) registry. Methods: The study population derived from the web-based SUN4P registry included 823 patients with first-ever acute stroke. Descriptive statistics were used to present patients’ characteristics. Results: The vast majority of patients (80.4%) had an ischemic stroke, whereas 15.4% had a hemorrhagic stroke. Hypertension was the leading risk factor in both patients. The patients with ischemic stroke had higher prevalence of traditional cardiovascular risk factors such as diabetes mellitus, dyslipidemia and smoking and most commonly cryptogenic stroke (39%). National Institutes of Health Stroke Scale (NIHSS) was higher among patients with hemorrhagic in comparison to those with ischemic stroke (10.5 vs 6 respectively). Moreover, all patients had similar rate of disability prior to stroke, as shown by Modified Rankin Scale (mRS=0). Conclusions: These data are in accordance with current evidence and should be thoroughly assessed in order to ensure optimal therapeutic management of stroke patients.

The aim of this study was to calculate the average operational cost per sub-type of stroke patient and to investigate cost drivers (e.g. ALoS, NIHSS score, age) correlated to cost. Methods: Direct medical costs (diagnostic imaging and clinical laboratory exams, overheads/bed cost, pharmaceuticals, ringers and other non-durables and inpatient rehabilitation) per patient were calculated from the providers’ (hospitals’) perspective. Resource use data derived from the “SUN4P” web-based registry and unit costs were retrieved from publically available sources and were assigned to resource use. Results: The sample comprised 6,282 inpatient days of 750 patients (mean age: 75.5±13.3 years) admitted from July 2019 to July 2021, in nine public hospitals. Mean length of stay was 8.4±7.6 days and mean total operational cost was calculated to €1,239.4 (from which 45% and 35% related to diagnostic exams and overheads/bed cost respectively). Mean cost related to hemorrhagic stroke patients that were discharged alive was calculated significantly higher compared to mean cost related to ischemic stroke patients who didn’t undertake thrombolysis and were also discharged alive from the hospital (€2,155.2 vs. €945.2, p

Introduction: High-quality evidence regarding the use of endovascular treatment (EVT) in patients with acute basilar artery occlusion (BAO) has been provided by recently completed randomized controlled clinical trials (RCTs). Methods: We conducted a systematic review and meta-analysis including all available RCTs that investigated efficacy and safety of EVT in addition to best medical treatment (BMT) versus BMT alone for BAO. The random-effects model was used, while the fragility index (FI) was calculated for dichotomous outcomes of interest. Results: Four RCTs were included comprising a total of 988 patients with acute BAO (mean age: 65.6 years, 70% men, median NIHSS: 24, 39% pretreatment with intravenous thrombolysis). EVT was related to higher likelihood of good functional outcome (RR: 1.54; 95% CI: 1.16–2.05; I2 = 60%), functional independence (RR: 1.83; 95% CI: 1.08–3.08; I2 = 79%) and reduced disability at 3 months (adjusted common OR: 1.96; 95% CI: 1.26–3.05; I2 = 59%) compared to BMT alone. Despite that EVT was associated with a higher risk for symptomatic intracranial hemorrhage (RR: 7.78; 95% CI: 2.36–25.61; I2 = 0%) and any intracranial hemorrhage (RR: 2.85; 95% CI: 1.50–5.44; I2 = 16%), mortality at 3 months was lower among patients that received EVT plus BMT versus BMT alone (RR: 0.76; 95% CI: 0.65–0.89; I2 = 0%). However, sufficient robustness was not evident in any of the reported associations (FI < 10) including the overall effect regarding the primary outcome. The former associations were predominantly driven by RCTs with recruitment limited in China. Conclusions: EVT combined with BMT is associated with a higher likelihood of achieving good functional outcomes and a lower risk of death at 3 months compared to BMT alone, despite the higher risk of sICH. An individual-patient data meta-analysis is warranted to uncover and adjust for potential sources of heterogeneity and to provide further insight.

BackgroundThe accurate knowledge of secular trends in prevalence, characteristics and outcomes of patients with ischemic stroke and atrial fibrillation allows better projections into the future.AimWe aimed to report the overall, age- and sex-specific secular trends of characteristics and outcomes of patients with acute ischemic stroke (AIS) and atrial fibrillation between 1993 and 2012 in the Athens Stroke Registry.MethodsWe used Joinpoint regression analysis to calculate the average annual percent changes and 95% confidence intervals.ResultsAmong 3314 stroke patients, 1044 (31.5%) had atrial fibrillation. Between 1993 and 2012, there was an average annual reduction of 0.8% (95% CI: −1.5%; 0.0%) in the proportion of atrial fibrillation patients among all AIS patients, whereas the proportion of newly diagnosed atrial fibrillation patients among all atrial fibrillation patients increased annually by an average of 7.1% (95% CI: 5.4%;8.9%). Among all atrial fibrillation patients, there was an average annual reduction of 2.9% (95% CI: −2.7; −3.2%) in the proportion of previously known atrial fibrillation patients, followed by an annual average reduction of 2.4% (95% CI: −1.2; −3.6%) in the proportion of previously known atrial fibrillation patients not receiving any antithrombotic treatment at admission. During that period, there was an increase in the average annual proportion of previously known atrial fibrillation patients treated with anticoagulants (6.4%, 95% CI: 1.2;11.9%) and aspirin (2.3%, 95% CI: −0.4;5.0%) at admission; an average annual increase in the proportion of atrial fibrillation patients who were prescribed anticoagulant was apparent both for patients with mRSConclusionsBetween 1993 and 2012, the proportion of atrial fibrillation patients on proper antithrombotic treatment and the rate of newly diagnosed atrial fibrillation increased significantly. Rates of stroke recurrence, cardiovascular events, and mortality reduced significantly.

We report a 57 year- old man with lateral abdominal wall bulging. MRI showed thoracic disk herniation at the T11-T12 level. Needle electomyogram disclosed acute denervation in paraspinal and abdominal muscles innervated from T11 root. Eight months later the swelling was reduced significantly. Thoracic disc herniations are rare and three similar cases have been described previously.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-ragiological syndrome presenting with neurological symptoms and characteristic radiologic findings. PRES occurs in the setting of various clinical conditions and requires prompt management of the causative factor for a full recovery. This is a case report of a Crohn's disease patient who developed PRES syndrome during a complicated post-operative course. In the presence of multiple causative factors, sepsis was considered as the predominant one. After prompt management, the patient recovered with no permanent neurological damage.

Raising interest towards genes implicates the effect of estrogen receptor-alpha (ESR1) gene on cerebrovascular disease, but data are lacking regarding the effect of estrogen receptor-beta (ESR2) gene. We assessed the hypothesis that AluI (G/A) polymorphism of the ESR2 gene (rs 4986938) is associated with ischemic stroke in a Caucasian population. Four hundred twenty four consecutive stroke patients and 430 age and gender-matched controls were enrolled in three stroke centers in Greece over one-year period. Patients and controls were compared in regard to the prevalence of the aforementioned polymorphism. No association was found between variations in the ESR2 gene and risk of stroke or stroke subtype in men and women. Of note, a gender-specific association of G allele with the onset of stroke at a younger age in male patients was found (63.68 ± 12.687 years in G allele (GG + AG) carriers vs. 68.95 ± 10.757 years in non-carriers (AA), p = 0.008). Further population independent studies are needed to establish the role of ESR2 gene polymorphisms in relation to ischemic stroke in both genders. Such studies could lead to ERβ agonists being validated in individuals with certain genotypes and/or alleles towards the development of efficient strategies to preventing ischemic stroke in both men and women.

Background and Purpose— We compared among young patients with ischemic stroke the distribution of vascular risk factors among sex, age groups, and 3 distinct geographic regions in Europe. Methods— We included patients with first-ever ischemic stroke aged 15 to 49 years from existing hospital- or population-based prospective or consecutive young stroke registries involving 15 cities in 12 countries. Geographic regions were defined as northern (Finland, Norway), central (Austria, Belgium, France, Germany, Hungary, The Netherlands, Switzerland), and southern (Greece, Italy, Turkey) Europe. Hierarchical regression models were used for comparisons. Results— In the study cohort (n=3944), the 3 most frequent risk factors were current smoking (48.7%), dyslipidemia (45.8%), and hypertension (35.9%). Compared with central (n=1868; median age, 43 years) and northern (n=1330; median age, 44 years) European patients, southern Europeans (n=746; median age, 41 years) were younger. No sex difference emerged between the regions, male:female ratio being 0.7 in those aged Conclusions— Primary preventive strategies for ischemic stroke in young adults—having high rate of modifiable risk factors—should be targeted according to sex and age at continental level.

The authors sought to determine the relative efficacy and tolerability of duloxetine versus citalopram and sertraline in the treatment of poststroke depression (PSD), anxiety, and fatigue. A group of 60 patients with PSD were assigned to receive duloxetine, citalopram, or sertraline and were assessed over a 3-month period for depression, anxiety, and fatigue. Improvement of depression and anxiety, but not fatigue, was observed in all study groups. Duloxetine was well tolerated and significantly more effective than citalopram and sertraline for the treatment of anxiety symptoms in PSD patients. None of the antidepressants used was effective for reducing symptoms of fatigue.

A 39-year-old man presented pronounced asthenia and diffuse myalgias 8 months after a vaccination against the tetanus toxoid. Muscle biopsy disclosed infiltration of the perimysium and endomysium by densely packed, PAS-positive macrophages. Tropheryma whippelii detection in blood and faeces allowed the initial assumption of atypical Whipple's disease with isolated muscle involvement. However, the histological detection of aluminium-containing macrophages by means of Morin stain immunofluorescence established the diagnosis of macrophagic myofascitis in an obviously asymptomatic T. whippelii carrier.

Cor triatriatum (CT) is a rare congenital heart disease characterized by a fibromuscular membrane that divides the left atrium into two distinct chambers and mostly manifests during infancy mimicking mitral valve stenosis. We present the uncommon case of cardioembolic ischemic stroke in an adult man on the grounds of an otherwise asymptomatic CT with intra-atrial thrombus formation, which was established by means of transesophageal echocardiography and cardiac magnetic resonance imaging. Because exact diagnosis and surgical treatment is feasible, we suggest CT as a rare, but potentially treatable source of cerebral embolism in adult patients with stroke and dilatated left atrium.

Fibromuscular dysplasia is a rare cause of stroke affecting mostly young females. It is characterized by the typical "string of beads" sign located mostly bilaterally in the midcervical portion of the carotid or vertebral arteries. We present the uncommon case of borderzone hemispheric infarction in a man with isolated unilateral fibromuscular dysplasia affecting continuously the distal extracranial and proximal intracranial portion of the left internal carotid artery leading to distal hypoperfusion and ischemia.

Sirs: Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant disorder characterised by progressive retinal and cerebellar degeneration [1]. Stargardt’s disease (STD) represents the most common hereditary (autosomal recessive) macular dystrophy manifesting with juvenile central visual impairment and progressive bilateral atrophy of the macula and the retinal pigmental epithelium (RPE) [2]. We report a case of juvenile SCA7 caused by de novo expansion of polyglutamine repeats, whose clinical presentation (isolated progressive visual impairment) simulated STD. A 12-year-old girl, with no family history of neurological or retinal disease, presented for ophthalmologic evaluation due to over six months of progressing bilateral visual loss. Based on the findings of the ophthalmologic examination (visual acuity of 0.2 was homozygote for a normal allele (< 19 repeats). The length of the SCA7 CAG repeat is inversely correlated with age of onset and dictates the initial symptom presentation. Longer CAG repeats lead to earlier onset of the disease, more rapid progression and increased chance of visual failure preceding cerebellar ataxia [1]. Interestingly, Johansson et al., after investigating the effect of repeat length on clinical manifestation in four Swedish SCA7 families, reported that in 80% of SCA7 cases with 59 repeats or more the disease started with visual impairment and visual symptoms preceded ataxia over a period of 1–6 years [4]. The retinal degeneration in SCA7 consists of a progressive cone-rod dystrophy causing substantial impairment of central visual acuity [1, 5]. The same phenotypic features may be shared by STD, which represents one of the most common causes of macular degeneration during the first two decades of life and is caused by homozygous mutations in the gene ABCA4 that encodes the photoreceptor-specific, ATP-binding transporter ABCA4 [6, 7]. The same gene, which has been mapped to the short arm of chromosome 1, is also associated with the phenotypic manifestation of fundus flavimaculatus (FFM) (Fig. 1D) [6, 7]. Recent, genotypephenotype correlation studies have highlighted the wide allelic heterogeneity of this gene. The spectrum of disease severity, associated with specific mutations in the ABCA4 gene, may vary from a mild phenotypic manifestation of STD (with absent dark choroid and normal electroretinographic amplitudes) to a severe and diffuse atrophy of the retinal pigmental epithelium and the choriocapillaris (present dark choroid) [6, 7]. Thus, in the absence of neurological manifestations and positive family history the differential diagnosis between bilaterally, impairment of color vision in the blue-yellow axis and subtle pigment mottling of the RPE; Fig. 1A), the visual field testing (central scotoma bilaterally), the unremarkable Ganzfeld electroretinography and the findings of fluorescein angiography (perifoveal flecks and absence of dark choroid), STD was diagnosed. Neurological examination and brain MRI were normal. Three years later she developed progressive truncal ataxia and poor motor coordination. The patient was admitted for further evaluation at our institution. Neurological examination revealed severe brainstem (bilateral pyramidal symptoms, dysphagia, tongue atrophy, markedly slowed ocular saccades) and cerebellar involvement (truncal ataxia, dysmetria, dysdiadochokinesia, gaze-evoked nystagmus and dysarthria). Repeat ophthalmologic examination disclosed pigmentary degeneration and accumulation, fundus atrophy with “bull’s eye configuration” (Fig. 1B). Substantial brainstem and cerebellar atrophy was noted on repeat brain MRI (Fig. 1C). Other systemic disorders with retinal and cerebellar degeneration (juvenile neuronal ceroid lipofuscinosis, abetalipoproteinemia, mitochondriopathies) were excluded on the basis of diagnostic work-up (negative skin punch biopsy, unremarkable muscle biopsy, negative DNA testing for Leber hereditary optic neuropathy, normal serum lipid electrophoresis). Molecular genetic testing identified one allele with abnormal CAG expansion (70 repeats, normal < 36) in the ataxin 7 gene and confirmed the diagnosis of SCA7. The patient’s father (asymptomatic with unremarkable neurological and ophthalmologic evaluations) was heterozygote for a mutable normal allele (intermediate allele, 34 repeats) [3]. The patient’s mother G. Tsivgoulis, MD ( ) · S. Vassilopoulou, MD · K. Spengos, MD Dept. of Neurology University of Athens School of Medicine Iras 39, Gerakas 15344 Athens, Greece Tel.: +306937178635 Fax: +302106742604 E-Mail: tsivgoulisgiorg@yahoo.gr

Current evidence suggests cardiac involvement and electrocardiographic changes of increasing frequency with age in patients with myotonic dystrophy type 2 (DM2). Myocyte hypertrophy with concurrent fibrosis seems to be the anatomical correlate. Moreover, morphological and functional changes indicative of subclinical cardiomyopathy have been demonstrated by means of cardiac magnetic resonance imaging (CMRI) and spectroscopy in patients with no overt cardiac disease. We present a 68-year-old woman with genetically established DM2 and no clinical, electrocardiographic or echocardiographic signs indicative of cardiac involvement. CMRI revealed delayed contrast enhancement of the anterior portion of the interventricular septum, indicating myocardial involvement. Contrast-enhanced CMRI might be a useful diagnostic tool in assessing cardiac involvement in cases of DM2. The role of delayed contrast enhancement should be further investigated in order to elucidate the cardiac features of this fascinating multisystem disease.

One day after a minor accident, an 85-year-old man developed headache and proximal left leg weakness. He was on warfarin due to atrial fibrillation. On hospital admission, a right posterior parasagittal subdural haematoma that was visualised on CT was considered as cause of all symptoms. Although no surgical intervention was performed he stayed for 2 weeks in a neurosurgical ward. Clinical deterioration occurred within the first days as he was not able to stand on his left leg or rise up from the sitting position. Headache had ceased, but the patient reported low abdominal pain. This condition remained stable for over 2 months although haematoma size decreased significantly. He was then examined for the first time by a neurologist who documented typical clinical features of femoral nerve palsy. Pelvic CT established the diagnosis of traumatic iliacus- and iliopsoas-muscle haematoma under anticoagulants as cause of subacute femoral mononeuropathy.

Background: The involvement of different brain areas in spontaneous eyeblink rate control is largely unknown. Methods: Spontaneous eyeblink rate was assessed in 211 consecutive acute stroke patients within two days after symptom onset and was correlated with infarction location as well as with other parameters such as the presence of hemineglect or significant small vessel disease in MRI or CT scans. Blink rates were measured in two conditions: (1) during a trivial conversation requiring no cognitive effort and (2) during mental arithmetic. Results: Patients and healthy controls displayed similar average blink rates at rest (19.1 and 17.3 blinks/min, respectively) and during mental arithmetic (22.6 and 20.2 blinks/min, respectively). The latter condition reliably increased blinking frequency in both patients and control subjects. The subgroups of patients with frontal, frontoparietal and frontotemporal infarctions exhibited a significantly higher blink rate modulation, expressed as an increased relative difference of blinking at rest compared with blinking during mental arithmetic. Conclusion: As a whole, patients with acute cerebrovascular disease do not show changes in blink rate. Subjects with frontal infarcts, however, systematically increase their blink rates during cognitive effort, most probably due to a lack of frontal inhibitory control on blinking modulation.

Headache related to airplane flights is rare. We describe a 37-year-old female patient with multiple intense, jabbing headache episodes over the last 3 years that occur exclusively during airplane flights. The pain manifests during take-off and landing, and is located always in the left retro-orbital and frontotemporal area. It is occasionally accompanied by dizziness, but no additional symptoms occur. Pain intensity diminishes and disappears after 15–20 min. Apart from occasional dizziness, no other symptoms occur. The patient has a history of tension-type headache and polycystic ovaries. Blood tests and imaging revealed no abnormalities. Here, we present the first case in Greece. We review the current literature on this rare syndrome and discuss on possible pathophysiology and the investigation of possible co-factors such as anxiety and depression.

Pompe disease is an inherited metabolic disorder caused by α-glycosidase deficiency. The adult onset form is mainly characterized by progressive proximal muscle weakness and respiratory dysfunction. The aim of the present study is to evaluate body composition in adult patients before and after enzyme replacement therapy (ERT). Body composition was examined at baseline by means of dual x-ray absorptiometry (DXA) in nine adult patients and after different time periods in six of them who received ERT. Total BMD (bone mineral density) was initially mildly decreased in two patients, while femoral neck BMD was decreased in five patients. On the other hand fat mass was increased in the majority of patients, while body mass index (BMI) was high in four. ERT administration did not seem to induce obvious BMD changes in any patient. Conclusively, the greater femoral neck BMD involvement may be attributed to the lower mechanical load applied by the selectively weakened muscles, whereas the increased fat mass may be the result of metabolic and nutritional derangement.

Objective Pompe disease is an autosomal recessive lysosomal disorder caused by α-glucosidase deficiency. A specific treatment for the disease with enzyme replacement therapy is currently available. The aim of the present study is to describe the clinical manifestations and the effect of treatment in the first Greek patients with the adult form of the disease. Methods Five Greek patients with adult onset Pompe disease aged between 40 and 73 years received 20 mg/kg Myozyme intravenously at two weekly intervals over a different period. Clinical and functional parameters were longitudinally registered. Results Proximal muscle weakness and respiratory insufficiency were the most common manifestations of the disease, but their severity was different even among patients with similar genotype. The effect of treatment varied with most patients experiencing some improvement in muscle strength and fatigability, while the most severely affected patient did not benefit and stopped therapy. Conclusion No clear genotype–phenotype correlation emerges from our study. The different effect of treatment on our patients seems to be mainly related to their pre-treatment condition and can be reliably assessed only on a long term basis.

Whereas the coexistence of different autoimmune or rheumatologic diseases with myasthenia gravis is well documented, the combination of myasthenia with sarcoidosis is extremely rare. There very few case reports of patients suffering from these two immune-mediated diseases. Nearly all had acetylcholine receptor antibodies. As far as we know myasthenia gravis with antibodies to muscle-specific tyrosine kinase-MuSK--has not been associated with any form of sarcoidosis. We present probably the first case of MuSK-positive myasthenia gravis with concurrent, asymptomatic pulmonary sarcoidosis.

To the Editor: We read with great interest the recent study by Andersen and Olsen regarding the usefulness of anticoagulation treatment in reducing poststroke mortality in patients with ischemic stroke (IS) and atrial fibrillation (AF). The authors, after evaluating data from a prospective nationwide registry, concluded that IS patients with AF and no contraindication to oral anticoagulants (OA) had an almost 50% reduction in the hazard of death when secondary prevention with anticoagulation treatment was instituted. This effect was independent of age, stroke severity, and stroke risk factors.1 The former findings raise certain potential clinical implications regarding the optimal …

MuSK-positive Myasthenia Gravis is in most cases clinically characterized by a progressive course with severe oculobulbar involvement or prominent neck, shoulder and respiratory muscle weakness. It is also distinguished from other forms of myastehnia through its lack of germinal centers or lymphocytic infiltrates in the thymic tissue. We present the case of a MuSK-positive female myasthenic patient with over four years slowly progressive weakness of the neck extensor muscles in the presence of thymus hyperplasia and discuss its uncommon and markedly focal clinical and electrophysiological features, as well as the excellent course under medication with pyridostigmine and prednisone, especially after thymectomy.

Superficial siderosis (SS), as a result of chronic subarachnoid haemorrhage and haemosiderin deposition on the leptomeninges and subpial layers of the brain, cerebellum and spinal cord, can cause ataxia, pyramidal tract lesions and hearing deficits. In cases with not pronounced hearing impairment adult-onset spinocereballar ataxia can be considered as a differential diagnostic alternative. We report a similar case where the diagnosis of SS was established by means of gradient echo MRI sequences 5 years after symptom onset. A bleeding lumbar ependymoma was identified as a source of haemorrhage. Surgical tumor resection stopped any further disease progression. Our report underlines that clinicians should be aware of the clinical features and diagnostic pitfalls of SS. Time of diagnosis and neurosurgical intervention can essentially influence the patients’ prognosis.

BACKGROUND AND OBJECTIVES Almost every fifth stroke occurs during sleep. Data about characteristics and etiology of stroke during sleep are conflicting. We investigated the association of the activity at stroke onset (onset during night sleep vs. onset while awake) with stroke subtypes of different etiology. METHODS A total of 1448 patients with first-ever stroke with known time of symptom presentation were prospectively evaluated. Statistical comparisons were performed between patients with stroke during sleep and stroke while awake in terms of demographic features, known risk factors, vascular comorbidities, and stroke subtypes. Multiple variable logistic regression analyses were performed to identify predictor variables (including stroke risk factors and stroke subtypes) for stroke during sleep. RESULTS Stroke during sleep was documented in 264 cases (18.2%). In subjects with stroke during sleep, lacunar infarction was the most prevalent stroke subtype (39%), while in patients with stroke while awake, small-vessel disease was the underlying mechanism significantly (P < .001) less often (13.8%). In contrast, patients with stroke while awake suffered significantly (P < .001) more frequently from intracerebral hemorrhage (18.2%) and cardioembolic stroke (34.9%) when compared with subjects with stroke during sleep (6.4% and 18.9%, respectively). The multiple variable logistic regression model identified the following factors as independent predictors of stroke during sleep: atrial fibrillation (odds ratio: 0.346, 95% confidence interval: 0.237-0.505, P < .001) and intracerebral hemorrhage versus ischemic stroke (odds ratio: 0.238, 95% confidence interval: 0.138-0.410, P < .001). Lacunar infarction was the only ischemic stroke subgroup that was positively associated with stroke during sleep (odds ratio: 2.568, 95% confidence interval: 1.447-4.560, P < .001). CONCLUSIONS There are significant differences between stroke during sleep and stroke while awake concerning vascular risk profile and stroke etiologic subtypes.

Fibrocartilaginous embolism of the intervertebral disc represents an uncommon cause of spinal cord infarction. We present the case of a previously healthy 30-year old ballet dancer who noted acute severe neck pain shortly after an intensive training session and developed weakness and numbness of both arms, as well as difficulties in emptying the bladder and bowel. Her clinical presentation and neuroimaging studies including diffusion weighted imaging were consistent with a spinal cord infarction in the anterior spinal territory at the C3-C6 spinal cord level. Although no histological confirmation was obtained, lack of evidence of other plausible diagnoses in the setting of the patient's clinical presentation and neuroimaging findings made fibrocartilaginous embolism the most likely etiopathogenetic mechanism of spinal stroke.

Background Anticoagulation therapy remains largely underused in older people with atrial fibrillation (AF). The aim of this pilot trial was to investigate the effects of low-intensity adjusted-dose and fixed-dose coumadin on the incidence of arterial thromboembolism and bleeding, as well as the efficacy of anticoagulation monitoring in elderly AF patients. Methods In this open-label, randomized trial we recruited patients over 75 years of age without previous stroke or systemic embolism. Patients were randomized into three groups, with group A receiving aspirin 100 mg/day, group B fixed-dose coumadin 1 mg/day; and group C adjusted-dose coumadin with a target range of international normalized ratios (INR) between 1.6 and 2.5. Primary endpoints (ischemic strokes and systemic embolisms) and secondary endpoints (deaths, myocardial infarctions, and major bleeding events) were prospectively documented. Results The study was discontinued 6 months after the enrollment of the first patient for safety reasons. During this period, 45 patients were recruited (15 patients in group A, 14 in group B, and 16 in group C). Over a mean follow-up period of 3.7 months (range: 1–6 months), two patients from group B developed a dangerous prolongation of the INR (7.0 and 4.2), which led to the discontinuation of fixed-dose coumadin. Another patient from the same group experienced a major bleeding event 1 month after enrollment in the study (INR: 5.5). The percentage of INR measurements within the target range was significantly ( p Conclusions Older people receiving fixed-dose oral anticoagulants may be at risk of developing a dangerous prolongation of their INR.

An insufficiently treated hypertensive woman developed severe neck pain, followed by a partial Brown-Sequard syndrome, due to a spontaneous spinal epidural hematoma compressing the cervical spinal cord at the levels C4–C7. We discuss the diagnostic difficulties in the acute setting and underline the importance of a careful and detailed neurological evaluation in all patients with any kind of acute neurological deficit. Application of modern imaging techniques in such selected acute cases is helpful in order to establish a diagnosis and initiate therapy. Although rare, clinicians should keep spinal epidural hematoma in mind and consider it as a highly uncommon, yet possible, complication of untreated hypertension.

OBJECTIVES: Blood pressure (BP) management in acute stroke remains a matter of little consensus. Data on BP changes during the first hours of ictus are lacking. We aimed to evaluate the early spontaneous time course of BP in different ischaemic stroke (IS) subtypes. METHODS: Twenty-four h BP monitoring was performed in 200 first-ever hyper-acute IS patients. The recording was initiated and terminated at 3 h and 27 h of ictus respectively. All IS patients were classified on admission into the following subgroups of different etiology: large artery atherosclerotic stroke (LAA), cardio-embolic stroke (CE), lacunar stroke (LAC) and infarct of undetermined cause (IUC). Statistical comparisons between stroke subgroups were performed using one-way ANOVA and linear regression analyses were used to evaluate the influence of different factors in BP course. RESULTS: Although there were no significant differences in 24 h systolic (SBP) and diastolic (DBP) BP values between IS subgroups, a distinctly different SBP course was observed. The SBP dropped sharply in the LAA and LAC subgroups, while a more gradual decrease was monitored in the CE subgroup. Throughout the BP-recording, a SBP decrease of 10.1% (95% CI: 8.6-11.5) and 10.4% (95% CI: 9.0-11.8) was documented in patients with LAA and LAC respectively, while a milder drop was recorded in CE (3.7%, 95% CI: 2.4-5.0) and IUC (5.5%, 95% CI: 4.1-6.8). Increasing stroke severity (p

a Department of Neurology, University of Athens Medical School, Eginition Hospital, and b Department of Clinical Therapeutics, University of Athens Medical School, Alexandra Hospital, Athens , Greece Case Description A 60-year-old man, with a history of hypercholesterolaemia and smoking, awakened with numbness on the right part of the inner mouth and the right arm and leg. Symptoms persisted over 3 days, when he consulted the family physician, who suspected stroke and referred him to our hospital for further evaluation. On examination the patient was alert, with normal and symmetrical tendon refl exes, while both plantar responses were fl exor. A right hemihypaesthesia involving the face, trunk, arm and leg was manifest. No ataxia, dysarthria, limb weakness, or ocular motor manifestations were present. MRI of the brain was than performed on the fourth day after ictus. A hyperintense lesion of ischaemic origin located in the left dorsal portion of the midbrain was demonstrated on fl uid attenuated inversion recovery (FLAIR, fi g. 1 a), T 2 -, as well as diffusionweighted images. The infarct was obviously affecting structures localised in the dorsal (superior colliculus, periaqueductal grey matter) and the dorsolateral (medial lemsiscus and lateral spinothalamic tract) midbrain territory, supplied by the superior cerebellar artery. MR angiographic images in the time-of-fl ight technique and ultrasound of the carotid and vertebral arteries were normal. Transthoracic echocardiography, 24-hour blood pressure monitoring and Holter electrocardiographic monitoring revealed no abnormalities. Visualand auditory-evoked potentials as well as extensive blood coagulation studies were unremarkable. The patient was diagnosed as having a brainstem lacunar stroke and was discharged on aspirin and lipid-lowering medication. A slight improvement of his sensory symptoms was reported at the follow-up evaluation 6 months later, while a new MRI revealed a substantial decrease in the extent of the hyperintense lesion on the FLAIR images ( fi g. 1 b). Introduction Occlusion of branches of the thalamogeniculate arteries supplying the thalamic somatosensory nuclei is responsible for the vast majority of strokes presenting with the clinical syndrome termed by Fisher as ‘pure sensory stroke’ (isolated hemihypaesthesia or paraesthesia involving the face, trunk, arm and leg) [1] . Occasionally, pure sensory stroke can be caused by a lateral tegmental pontine or medullary infarct [2] . Brain ischaemia limited to the mesencephalon is uncommon, accounting for 0.2–2.3% of total admitted ischaemic strokes [2–5] . The anteromedial territory, supplied by the direct perforators of the basilar artery, is most frequently affected in patients with isolated midbrain infarct, whose neurological picture is dominated by oculomotor disturbances [2–5] . We report the uncommon case of a patient presenting with the clinical syndrome of pure sensory stroke due to an infarction limited to the dorsal midbrain territory.

Viral infection is a rare cause of painful ophthalmoplegia. We report on a 67-year-old patient who developed painful double vision after a vesicular skin rash on the left forehead. MRI disclosed simultaneous inflammatory lesions in all extraocular muscles, the second and third cranial nerve, as well as pathological signal intensity along the spinal trigeminal tract and nucleus within the medulla oblongata and the pons. Cerebrospinal fluid and serum tests for varicella zoster were positive. The patient was treated effectively with intravenous acyclovir and methylprednisolone. Simultaneous lesions in various neighbouring neural structures may be characteristic for the highly neurotropic behaviour of the herpesviridae and should be considered as a cause of painful ophthalmoplegia that can be depicted by appropriate imaging.