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Background: Basilar artery occlusion usually causes severe disability or death. Until the recent developments in local intra-arterial or systemic intravenous fibrinolysis, interest in early diagnosis was low because there was no satisfactory treatment. Thus there is little information about the initial phase of the disease.Objective: To report on the early clinical features and patterns of evolution of severe symptomatic basilar artery occlusion.Methods: 24 patients with established basilar artery occlusion (confirmed by angiography or at necropsy) were reviewed retrospectively, focusing on the early clinical aspects and time course of the disease.Results: The most common initial symptoms were motor deficits (16/24, including facial palsies), articulatory speech difficulties (15/24), vertigo, nausea or vomiting (13/24), and headaches (10/24). The most frequent objective initial findings were motor deficits (22/24), facial palsies (19/24), eye movement abnormalities (15/24), lower cranial nerve deficits (15/24), altered level of consciousness (12/24), and bilateral extensor plantar responses (9/24). Onset of the disease was gradual in nearly all patients and in half the warning signs were present for up to two months before the final stage. Headaches and visual disturbances were early signs, while speech difficulties and motor deficits were late signs. Once permanent neurological deficits were present, the final illness was reached within six hours in 41%, between six and 24 hours in 32%, and in two to three days in 27%.Conclusions: All the patients reviewed presented some symptoms and signs pointing to brain stem involvement. Only 8% (2/24) had an acute course with no adequate warning signs. [ABSTRACT FROM AUTHOR]

A list of ad hoc reviewers for the journal is presented including Craig Anderson, Angelo Antonini, and Peter Appelros.

ASCO phenotyping (A: atherosclerosis; S: small-vessel disease; C: cardiac pathology; O: other causes) assigns a degree of likelihood of causal relationship to every potential disease (1 for potentially causal, 2 for causality is uncertain, 3 for unlikely causal but the disease is present, 0 for absence of disease, and 9 for insufficient workup to rule out the disease) commonly encountered in ischemic stroke describing all underlying diseases in every patient. In this new evolution of ASCO called ASCOD, we have added a 'D' for dissection, recognizing that dissection is a very frequent disease in young stroke patients. We have also simplified the system by leaving out the 'levels of diagnostic evidence', which has been integrated into grades 9 and 0. Moreover, we have also changed the cutoff for significant carotid or intracranial stenosis from 70% to more commonly used 50% luminal stenosis, and added a cardiogenic stroke pattern using neuroimaging. ASCOD captures and weights the overlap between all underlying diseases present in ischemic stroke patients. © 2013 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background Intravenous recombinant tissular plasminogen activator (rt-PA) is the only approved pharmacological treatment for acute ischaemic stroke. The authors aimed to analyse potential causes of the variable effect on early course and late outcome. Methods and results 136 patients (42% women, 58% men) treated with intravenous rt-PA within 3 h of stroke onset in an acute stroke unit over a 3-year period, were included. Early clinical profiles of evolution at 48 h were divided into clinical improvement (CI) (decrease >4 points in the National Institute of Health Stroke Scale (NIHSS)); clinical worsening (CW) (increase >4 points NIHSS); clinical worsening after initial improvement (CWFI) (variations of >4 points in the NIHSS). Patients with clinical stability (no NIHSS modification or <4 points) were excluded. The patients showed in 66.9% CI, 13.2% CW 8.1 % CWFI and 11.8% remained stable. Female sex, no hyperlipaemia and peripheral arterial disease were associated with CW. Male sex and smoking were associated with CI. Absence of arterial occlusion on admission (28.4%) and arterial recanalisation at 24 h were associated with CI. Main causes of clinical deterioration included symptomatic intracranial haemorrhage (sICH), persistent occlusion and cerebral oedema. 23.5% developed ICH, 6.6% of which had sICH. At 3 months, 15.5% had died. Mortality was increased in CW, mainly related to sICH and cerebral oedema. The outcome of CWFI was intermediate between CW and CI. Conclusions Early clinical profiles of evolution in thrombolysed patients vary considerably. Even with CI, it is critical to maintain vessel permeability to avoid subsequent CW. [ABSTRACT FROM AUTHOR]

Background In some cases of hemiplegia, the initiation of yawning is associated with involuntary raising of the paralysed arm. Reports are scarce in the literature, probably because the phenomenon has largely been overlooked. Methods We studied six patients from two neurologic units, and compared them with published cases from the last 200 years. Brain imaging typically shows a small vascular lesion most often located in the internal capsule. Results After comparison with experimental models in cats, we suggest that damage to the cortico-neocerebellar tract of the extrapyramidal system disinhibits the spino-archeocerebellar tract, enabling a motor stimulation of the arm by the lateral reticular nucleus, which harmonises both central respiratory and locomotor rhythms. Conclusions When phylogenetically primitive structures are disinhibited, they regain autonomy in the homeostatic process associating the massive inspiration of yawning – a form of behaviour that stimulates vigilance – with a motor control mechanism that is active during locomotion. For this phenomenon, we coined the term ‘parakinesia brachialis oscitans’. [ABSTRACT FROM AUTHOR]

This article reviews published stroke subtype classification systems and offers rules and a basis for a new way to subtype stroke patients. Stroke subtyping can have different purposes, e.g. describing patients’ characteristics in a clinical trial, grouping patients in an epidemiological study, careful phenotyping of patients in a genetic study, and classifying patients for therapeutic decision-making in daily practice. The classification should distinguish between ischemic and hemorrhagic stroke, subarachnoid hemorrhage, cerebral venous thrombosis, and spinal cord stroke. Regarding the 4 main categories of etiologies of ischemic stroke (i.e. atherothrombotic, small vessel disease, cardioembolic, and other causes), the classification should reflect the most likely etiology without neglecting the vascular conditions that are also found (e.g. evidence of small vessel disease in the presence of severe large vessel obstructions). Phenotypes of large cohorts can also be characterized by surrogate markers or intermediate phenotypes (e.g. presence of internal carotid artery plaque, intima-media thickness of the common carotid artery, leukoaraiosis, microbleeds, or multiple lacunae). Parallel classifications (i.e. surrogate markers) may serve as within-study abnormalities to support research findings. Copyright © 2009 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

We now propose a new approach to stroke subtyping. The concept is to introduce a complete ‘stroke phenotyping’ classification (i.e. stroke etiology and the presence of all underlying diseases, divided by grade of severity) as distinguished from past classifications that subtype strokes by characterizing only the most likely cause(s) of stroke. In this phenotype-based classification, every patient is characterized by A-S-C-O: A for atherosclerosis, S for small vessel disease, C for cardiac source, O for other cause. Each of the 4 phenotypes is graded 1, 2, or 3. One for ‘definitely a potential cause of the index stroke’, 2 for ‘causality uncertain’, 3 for ‘unlikely a direct cause of the index stroke (but disease is present)’. When the disease is completely absent, the grade is 0; when grading is not possible due to insufficient work-up, the grade is 9. For example, a patient with a 70% ipsilateral symptomatic stenosis, leukoaraiosis, atrial fibrillation, and platelet count of 700,000/mm3 would be classified as A1-S3-C1-O3. The same patient with a 70% ipsilateral stenosis, no brain imaging, normal ECG, and normal cardiac imaging would be identified as A1-S9-C0-O3. By introducing the ‘level of diagnostic evidence’, this classification recognizes the completeness, the quality, and the timing of the evaluation to grade the underlying diseases. Diagnostic evidence is graded in levels A, B, or C: A for direct demonstration by gold-standard diagnostic tests or criteria, B for indirect evidence or less sensitive or specific tests or criteria, and C for weak evidence in the absence of specific tests or criteria. With this new way of classifying patients, no information is neglected when the diagnosis is made, treatment can be adapted to the observed phenotypes and the most likely etiology (e.g. grade 1 in 1 of the 4 A-S-C-O phenotypes), and analyses in clinical research can be based on 1 of the 4 phenotypes (e.g. for genetic analysis purpose), while clinical trials can focus on 1 or several of these 4 phenotypes (e.g. focus on patients A1-A2-A3). Copyright © 2009 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

The famous Swiss writer Charles-Ferdinand Ramuz suffered a stroke at 65 years, which he called ‘the adventure’ or ‘the accident’. He developed language disturbances suggesting crossed aphasia in a right hander with left hemiparesis. This uncommon pattern allowed him to continue to write his diary and to report his disturbances, with a unique depth and precision, especially for cognitive-emotional changes. Language and motor dysfunction recovered within a few weeks, but Ramuz complained of persisting emotional flattening alternating with irritability, fatigue, depression, anxiety, and concentration difficulty, which gave him the feeling to have become another person and to be inhabited by a stranger, whom he compared with devils. Ramuz fought several months to resume his literary activity, having the impression to have lost inspiration and creativity. However, the novels he wrote less than 6 months after stroke show no stylistic changes and have been found to be of the same quality as his previous production. Ramuz even ‘used’ his stroke experience in his work, in particular in a novel depicting an old man who has a stroke and dies of it. Ramuz’s diary, with his own daily description of stroke features and consequences during acute and recovery phases, is a unique document in a writer of his importance, and provides invaluable information on subjective emotional and cognitive experience of stroke. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Neuroimaging techniques are necessary for the evaluation of stroke, one of the leading causes of death and neurological impairment in developed countries. The multiplicity of techniques available has increased the complexity of decision making for physicians. We performed a comprehensive review of the literature in English for the period 1965–2005 and critically assessed the relevant publications. The members of the panel reviewed and corrected an initial draft, until a consensus was reached on recommendations stratified according to the European Federation of Neurological Societies (EFNS) criteria. Non-contrast computed tomography (CT) scan is the established imaging procedure for the initial evaluation of stroke patients. However, magnetic resonance imaging (MRI) has a higher sensitivity than CT for the demonstration of infarcted or ischemic areas and depicts well acute and chronic intracerebral hemorrhage. Perfusion and diffusion MRI together with MR angiography (MRA) are very helpful for the acute evaluation of patients with ischemic stroke. MRI and MRA are the recommended techniques for screening cerebral aneurysms and for the diagnosis of cerebral venous thrombosis and arterial dissection. For the non-invasive study of extracranial vessels, MRA is less portable and more expensive than ultrasonography but it has higher sensitivity and specificity for carotid stenosis. Transcranial Doppler is very useful for monitoring arterial reperfusion after thrombolysis, for the diagnosis of intracranial stenosis and of right-to-left shunts, and for monitoring vasospasm after subarachnoid hemorrhage. Currently, single photon emission computed tomography and positron emission tomography have a more limited role in the evaluation of the acute stroke patient. [ABSTRACT FROM AUTHOR]

Complex painful reflex syndrome is sometimes described in the chronic phase of stroke. Acute autonomic dysfunction (AAD), which is occasionally present in cases of acute stroke, has not been studied prospectively. The aim of the study was to investigate AAD on the hemibody contralateral to the lesion in the acute phase of stroke. One hundred consecutive patients (median age ± interquartile range, 74 ± 21; range 19–93; 51 women: 80 ± 17 and 49 men: 70 ± 17 years) in the acute phase of stroke were studied prospectively. Changes in skin temperature or coloration, diaphoresis, pain, or edema were noted in the first 3 days post-stroke. Associations between AAD and topography (cortical pre- and/or post-central, insular, corona radiata, basal ganglia, internal capsule, thalamus, and brainstem), age, gender, ischemic or hemorrhagic etiology, or the presence of sensorimotor deficits or ataxia were examined using the chi-squared or Fisher's exact test and logistic regression analysis. AAD was found in 71% of the patients and showed a significant positive association with the presence of a lesion in the post-central cortex ( P = 0.037), internal capsule ( P = 0.005), basal ganglia ( P = 0.002), or insula ( P = 0.011) and a negative association with the presence of a lesion in the brainstem ( P = 0.004). Multivariate logistic regression analysis including all studied topographic variables showed that only brainstem lesions were significantly associated with a decreased risk of developing AAD (odds ratio = 0.08, 95% confidence interval: 0.01–0.69, P = 0.022). AAD was not associated with age, gender, the ischemic or hemorrhagic nature of the lesion, the side of lesion, hypertonic or hypotonic paresis, or hyperreflexia or hyporeflexia. AAD was found in association with sensory deficits ( P = 0.001) and contralateral hyperkinesia ( P = 0.004). Acute AAD is significantly more likely to occur in the presence of hemispheric lesions involving sensory pathways from the cortex to the internal capsule and insula and is significantly less prevalent in the presence of brainstem lesions. [ABSTRACT FROM AUTHOR]

Progressive multifocal leucoencephalopathy (PML), a demyelinating disease caused by the JC virus (JCY), occurs in immunosuppressed patients and carries a poor prognosis. A favourable outcome is reported in two patients with PML and dermatomyositis. Immunosuppressive drugs were stopped in patient 1 but could only be partially tapered in patient 2. The JCV-specific CD8+ T cell response was strong in patient 1 and weak in patient 2. Both were treated with cytosine-arabinoside, and patient 2 was also treated with mirtazapine, a 5HT2A receptor antagonist. Combination of these drugs might be helpful to treat HIV-negative patients with PML. [ABSTRACT FROM AUTHOR]

Migraine is one of the most common neurological disorders and one of the most frequent primary headaches. It imposes a significant burden on the affected individuals, society and health care system. As the etiology and pathophysiology of migraine are not well understood, treatment is largely symptomatic. Patent foramen ovale is a remnant of a fetal circulation and is highly prevalent in the general population. Its presence was linked to several disorders including migraine. The aim of this review was to search in the available data the answer to the question whether the link between migraine and patent foramen ovale is coincidental or whether they represent a pathophysiological entity. Copyright © 2006 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Reliable data on stroke incidence and prevalence are essential for calculating the burden of stroke and the planning of prevention and treatment of stroke patients. In the current study we have reviewed the published data from EU countries, Iceland, Norway, and Switzerland, and provide WHO estimates for stroke incidence and prevalence in these countries. Studies on stroke epidemiology published in peer-reviewed journals during the past 10 years were identified using Medline/PubMed searches, and reviewed using the structure of WHO's stroke component of the WHO InfoBase. WHO estimates for stroke incidence and prevalence for each country were calculated from routine mortality statistics. Rates from studies that met the ‘ideal’ criteria were compared with WHO's estimates. Forty-four incidence studies and 12 prevalence studies were identified. There were several methodological differences that hampered comparisons of data. WHO stroke estimates were in good agreement with results from ‘ideal’ stroke population studies. According to the WHO estimates the number of stroke events in these selected countries is likely to increase from 1.1 million per year in 2000 to more than 1.5 million per year in 2025 solely because of the demographic changes. Until better and more stroke studies are available, the WHO stroke estimates may provide the best data for understanding the stroke burden in countries where no stroke data currently exists. A standardized protocol for stroke surveillance is recommended. [ABSTRACT FROM AUTHOR]

Background: Two professional painters experienced significant changes in their art as the main consequence of minor stroke located in the left occipital lobe or thalamus.Methods: The features of this artistic conversion were analysed on the basis of extensive neurological, neuropsychological, and psychiatric evaluations.Results: Both painters, initially unaware of the artistic changes, exhibited mild signs of executive dysfunction, but no general cognitive decline. The first painter, who showed mild visual-perceptive difficulties (dyschromatopsia and scotoma in his right upper visual field after left occipital stroke), together with increased anxiety and difficulty in emotional control, switched to a more stylised and symbolic art. The second painter, who also presented features of emotionalism related to his left latero-thalamic stroke, switched from an impressionist style to a more joyous and geometric, but more simplistic, abstract art.Conclusions: These findings show that mild cognitive and affective modifications due to focal posterior brain lesions can have significant repercussions on artistic expression. [ABSTRACT FROM AUTHOR]

Cerebral aneurysms and arteriovenous malformations (AVMs) are well-known sources of intracranial hemorrhage, but can also manifest as other clinical symptoms or remain clinically asymptomatic. The aim was to document and analyze cases of aneurysm or AVM with brain infarction. Survey on 4804 stroke patients treated at the Department of Neurology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland between 1978 and 2000 using the Lausanne Stroke Registry. Twenty patients presented with cerebral aneurysm and 21 with cerebral AVM. Hemorrhage was present in 100% of the AVM and in 75% of the aneurysm patients; in one (5%) of the remaining aneurysm patients, aneurysm and infarction were located in different territories. Infarction associated with Sylvian artery aneurysm was found in three (15%), vertebrobasilar ischemia because of fusiform left vertebral artery aneurysm in one (5%), and dural fistula draining to the distal transversal and left sigmoid sinus associated with a stroke in the territory of the left anterior inferior cerebellar artery in one patient. Ischemic stroke is infrequent, but important, complication in unruptured intracranial aneurysms and AVMs. The early recognition and therapy of these vascular malformations in selected patients can avoid a major neurological deficit or death caused by their rupture. [ABSTRACT FROM AUTHOR]

Discusses key issues concerning making accurate medical diagnoses in neurology. Importance of correct diagnoses on the work and reputation of neurologists; Sample cases on the issue; Implications on neurology.

Presents a case study of dissociated preservation of automatic-voluntary jaw movements in a patient with biopercular and unilateral pontine infarcts. Information on apraxia; Clinical history of the patient; Details of Foix-Chavany-Marie syndrome.

Presents information on hypertension. Role of hypertension in stroke; Primary prevention of stroke in hypertension; Hypertension as a risk factor in secondary prevention of stroke; Relationship between blood pressure variations and neurological deficits.