A beautiful stroke? A side note on the 75th anniversary of the spectacular death of the French organist and composer Louis Vierne (1870-1937)

The great French organist and composer Louis Vierne (1870-1937) died while performing an organ recital at Notre Dame cathedral in Paris - right in front of the console. This historical article provides insights into the biography of a highly talented musician who was challenged by disability and diseases throughout his career. A special focus is placed on the circumstances of Vierne’s remarkable death. Until now, both a primary cerebrovascular event and a "heart attack" are discussed in reference books and encyclopedias as the immanent causes of death. From the perspective of a stroke neurologist, a reappraisal of Vierne’s medical history and the events that happened during his last concert is presented. Copyright (C) 2012 S. Karger AG, Base

Chronic small vessel disease affects clinical outcome in patients with acute striatocapsular stroke

Background: Recovery from stroke is presumed to be a function of a cerebral network. Chronic small vessel disease (SVD) has been shown to disrupt this network’s integrity and has been proposed as a predictor of poor outcome after stroke. We studied this hypothesis in patients with acute ischemic stroke of the striatocapsular region, an area of pronounced cortical and subcortical connectivity. Methods: We identified 62 patients with isolated striatocapsular stroke from our stroke registry. The standardized workup included clinical rating according to the modified Rankin Scale (mRS) and MRI, rated according to the Fazekas scale for the extent of SVD, ranging from grade 0 to III. MRS at admission, at discharge, and a short-term recovery parameter (the difference between mRS at admission and discharge) were correlated with the extent of SVD. Comorbidity was assessed with the Charlson comorbidity index (CCI). Results: SVD was graded 0 in 7%, I in 60%, II in 18%, and III in 16% of patients. The median mRS at discharge for the groups was 2, 1, 2 and 4, and the median recovery parameter was 2, 1, 1 and 0.5, respectively. The extent of SVD significantly correlated with both the mRS at discharge and the recovery parameter. While age was also a significant predictor of these outcome parameters, SVD severity was a significant predictor even after correction for age or CCI. Conclusions: SVD is a predictor of poor outcome and recovery in striatocapsular stroke, independent of age or comorbidity. Severe SVD disturbs the integrity of the cerebral network leading to aggravation of and poor recovery from neurological deficits. Copyright (c) 2011 S. Karger AG, Base

Thrombolytic Stroke Treatment of a 12-Year-Old Girl with Intracranial Fibromuscular Dysplasia

Fibromuscular dysplasia, predominantly found in adult women, is a rare disease of small and middle-sized arteries of the kidney and brain. We present a case of a 12-year-old girl with acute ischemic stroke, due to fibromuscular dysplasia of the distal internal carotid artery and the proximal middle cerebral artery, which was successfully treated with t-PA

Diffusion-weighted imaging for the differential diagnosis of disorders affecting the hippocampus

Background: The human hippocampus can be affected in a large variety of very different neurological diseases, of which acute ischemic stroke, transient global amnesia, epilepsy, and limbic encephalitis are the most common. Less frequent etiologies include various infections and encephalopathy of different origins. Clinical presentation notably comprises confusional state, altered vigilance, memory deficits of various extent and seizures. While in hypoxic or hypoglycemic encephalopathy, clinical presentation and surrounding circumstances provide some clues to reach the correct diagnosis, in the above-listed more common disorders, signs and symptoms might overlap, making the differential diagnosis difficult. This review presents recent studies using the diffusion-weighted imaging (DWI) technique in diseases involving the hippocampus. Methods: References for the review were identified through searches of PubMed from 1965 to January 2011. Only papers published in English were reviewed. Full articles were obtained and references were checked for additional material where appropriate. Results: All pathologies affecting the hippocampus are associated with distinct lesion patterns on magnetic resonance imaging, and especially DWI has the ability to demonstrate even minute and transient hippocampal lesions. In acute ischemic stroke in the posterior cerebral artery territory, involvement of the hippocampal formation occurs in four distinct patterns on DWI that can be easily differentiated and correspond to the known vascular anatomy of the hippocampus. In the subacute phase after transient global amnesia (TGA), dot-like hyperintense lesions are regularly found in the lateral aspect of the hippocampus on DWI. The DWI lesions described after prolonged seizures or status epilepticus include unilateral or bilateral hippocampal, thalamic, and cortical lesions of various extent, not restricted to vascular territories. In limbic encephalitis, DWI lesions are only infrequently found and usually affect the hippocampus, uncus and amygdala. Furthermore, in some rare cases DWI lesions of different etiology may coexist. Conclusion: In patients with diseases affecting the hippocampus, DWI appears to be useful in differentiating between underlying pathologies and may facilitate a definite diagnosis conducive to an optimal treatment. With a careful clinical examination, experience with the interpretation of DWI findings and knowledge of associated phenomena, it is indeed possible to differentiate between ischemic, ictal, metabolic, and TGA-associated findings. Copyright (C) 2011 S. Karger AG, Base

Coexisting large and small vessel disease in patients with ischemic stroke of undetermined cause

Background and Purpose: Large artery atherosclerosis (LAA) and small vessel disease (SVD) share common risk factors for stroke. We aimed at investigating the association of SVD with cerebral LAA as well as with atherosclerosis in patients with stroke likely to originate from aortic plaques. Methods: We investigated 71 consecutive patients (48 men, mean age 64.2 +/- 13 years) with ischemic stroke of undetermined cause according to the ASCO classification, who received ECG-triggered CT angiography for best available atherosclerotic plaque detection in the aorta. Results: Aortic atherosclerotic plaques were detected in 54 patients (76.1%). The presence of SVD significantly correlated with the presence of aortic plaques (p < 0.001), as well as LAA (p < 0.001) and risk factors such as arterial hypertension (p = 0.032) and diabetes mellitus (p = 0.017). Conclusions: Aortic plaques are common in patients with stroke of undetermined cause. If so, SVD and LAA are often coexisting, which demonstrates the close link of macro- and microangiopathy, at least in cases of severe risk factors of atherosclerosis. Copyright © 2012 S. Karger AG, Base

End-of-life decisions in acute stroke patients: an observational cohort study

Background: Crucial issues of modern stroke care include best practice end-of-life-decision (EOLD)-making procedures and the provision of high-quality palliative care for dying stroke patients. Methods: We retrospectively analyzed records of those patients who died over a 4-year period (2011–2014) on our Stroke Unit concerning EOLD, focusing on the factors that most probably guided decisions to induce limitation of life-sustaining therapy and subsequently end-of-life-care procedures thereafter. Results: Of all patients treated at our Stroke Unit, 120 (2.71 %) died. In 101 (86.3 %), a do-not-resuscitate-order (DNRO) was made during early treatment. A decision to withdraw/withhold further life supportive therapy was made in 40 patients (34.2 %) after a mean of 5.0 days (range 0–29). Overall patient death occurred after a mean time of 7.0 days (range 1–30) and 2.6 days after therapy restrictions. Disturbance of consciousness at presentation, dysphagia on day 1 and large supratentorial stroke were possible indicators of decisions to therapeutic withdrawing/withholding. Proceedings of EOL care in these patients were heterogeneous; in most cases monitoring (95 %), medical procedures (90 %), oral medication (88 %), parenteral nutrition (98 %) and antibiotic therapy (86 %) were either not ordered or withdrawn, however IV fluids were continued in all patients. Conclusions: A high percentage of stroke patients were rated as terminally ill and died in the course of caregiving. Disturbance of consciousness at presentation, dysphagia on day 1 and large supratentorial stroke facilitated decisions to change therapeutic goals thus initiating end-of-life-care. However, there is further need to foster research on this field in order to ameliorate outcome prognostication, to understand the dynamics of EOLD-making procedures and to educate staff to provide high-quality patient-centred palliative care in stroke medicine

Brain imaging in patients with transient ischemic attack: a comparison of computed tomography and magnetic resonance imaging

Background: Brain imaging in stroke aims at the detection of the relevant ischemic tissue pathology. Cranial computed tomography (CT) is frequently used in patients with transient ischemic attack (TIA) but no data is available on how it directly compares to magnetic resonance imaging (MRI). Methods: We compared detection of acute ischemic lesions on CT and MRI in 215 consecutive TIA patients who underwent brain imaging with either CT (n = 161) or MRI (n = 54). An MRI was performed within 24 h in all patients who had CT initially. Results: An initial assessment with CT revealed no acute pathology in 154 (95.7%) and possible acute infarction in 7 (4.3%) patients. The acute infarct on CT was confirmed by diffusion-weighted imaging (DWI) in only 2 cases (28.6%). DWI detected an acute infarct in 50 of the 154 patients with normal baseline CT (32.5%). Among 54 patients without baseline CT, DWI showed acute ischemic lesions in 19 (35.2%). The ischemic lesions had a median volume of 0.87 cm 3 (range: 0.08–15.61), and the lesion pattern provided clues to the underlying etiology in 13.7%. Conclusion: Acute MRI is advantageous over CT to confirm the probable ischemic nature and to identify the etiology in TIA patients

CT angiography of the aorta is superior to transesophageal echocardiography for determining stroke subtypes in patients with cryptogenic ischemic stroke

Background: The etiology of ischemic strokes remains cryptogenic in about one third of patients, even after extensive workup in specialized centers. Atherosclerotic plaques in the aorta can cause thromboembolic events but are often overlooked. They can elude standard identification by transesophageal echocardiography (TEE), which is invasive or at best uncomfortable for many patients. CT angiography (CTA) can be used as an alternative or in addition to TEE if this technique fails to visualize every part of the aorta and in particular the aortic arch. Methods: We prospectively studied 64 patients (47 men, age 60 8 13 years) classified as having cryptogenic stroke after standard and full workup [including brain MRI and 24-hour electrocardiogram (ECG)] with ECG-triggered CTA of the aorta in search of plaques and compared the results with those of TEE. Investigators were blinded to the results of both techniques

Focal delivery of AAV2/1-transgenes into the rat brain by localized ultrasound-induced BBB opening

Delivery of drugs and macromolecules to the central nervous system (CNS) is hindered by the blood-brain barrier (BBB). Several approaches have been used to overcome this hindrance to facilitate the treatment of various CNS diseases. We now present results showing that chimeric adeno-associated virus 2/1 (AAV2/1) particles containing the coding region for the LacZ gene are efficiently delivered into the rat brain upon intravenous (IV) administration after BBB opening by focused ultrasound in the presence of vascular acoustic resonators. We show that the transgene is correctly and efficiently expressed in cells located in the neighborhood of the insonated focus, especially in the vicinity of small vessels and capillaries. Histochemical LacZ staining allows the identification of large amounts of cells expressing the enzymatically active protein. Using double immunofluorescence (IF) with antibodies against tubulinIII and bacterial LacZ, we identified these cells to be mostly neurons. A small proportion of the transduced cells was recognized as glial cells, reacting positive in the IF with antibodies against astrocytic markers. These results demonstrate that our approach allows a very specific, localized, and efficient expression of intravenously administered transgenes in the brain of rats upon ultrasound-induced BBB opening