Undetermined stroke genesis and hidden cardiomyopathies determined by cardiac magnetic resonance.

OBJECTIVE: To determine whether cardiac magnetic resonance imaging (CMR) could be useful in identifying previously undiagnosed cardiomyopathies in a cohort of patients with ischemic stroke who underwent standard etiologic investigation and to describe the type and frequency of these cardiomyopathies. METHODS: We performed a subanalysis of a previously collected prospective cohort of patients with ischemic stroke. Patients with structural changes on echocardiography that are considered causal for stroke in the Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification were excluded. A 3T CMR was performed. We compared the frequency of the cardiomyopathies that we found with reference values for the general population. RESULTS: One hundred thirty-two patients with a mean age of 68.4 years were included. In 7 patients (5.3%, 95% confidence interval 2.59%-10.54%) CMR identified cardiomyopathy. Four patients had hypertrophic cardiomyopathy, 2 had restrictive cardiomyopathy, and 1 had noncompaction cardiomyopathy. Six of these patients had been classified after standard evaluation as having undetermined stroke and 1 patient as having cardioembolic stroke (atrial fibrillation). We found a higher frequency of hypertrophic cardiomyopathy in the entire cohort and in the undetermined cause group compared to the general population (3.03% and 5.81% vs 0.2%, respectively, p = 0.001 and p < 0.001). The frequency of noncompaction cardiomyopathy was also higher in our cohort (0.76% vs 0.05%, respectively, p < 0.001). CONCLUSIONS: Although rare, cardiomyopathies should be considered as a possible cause of ischemic stroke classified as of undetermined etiology after standard evaluation.

Vascular White Matter Lesions in Young Adults: A Neurology Outpatient Clinic Registry.

INTRODUCTION: Although frequently assumed to be age-related changes, vascular white matter lesions (WML) are sometimes found in young adults. Etiology is usually attributed to sporadic small vessel disease; nevertheless, genetic disorders may also be implicated. We aimed to characterize the population of young adults with vascular WML in Neurology outpatient clinics. METHODS: Neurologists from 12 Portuguese hospitals were invited to include patients aged 18-55 years evaluated in consultation, with vascular WML on MRI, scoring II or III in the Fazekas scale. Central imaging validation was performed by 2 independent, blinded, Neuroradiologists. Demographic and clinical data were collected as well as results of investigations performed. RESULTS: During 2 years, 77 patients were included (mean age 47.7 years). Vascular risk factors were present in 88.3% patients (hypertension in 53.2%) and previous history of stroke in 36.4%. Patients without history of stroke were younger (46.6 ± 7.2 vs. 49.6 ± 3.9 years, p = 0.045) and had fewer vascular risk factors (p < 0.001). They were more frequently females (87.8 vs. 46.4%, p < 0.001), and headache (30.6 vs. 3.6%, p = 0.007), contrary to focal symptoms (16.3 vs. 53.6%, p = 0.001), was the most frequent reason of referral. Etiological investigations performed differed between Neurologists. A genetic disorder was identified in 6 out of 58 patients (CADASIL n = 5; COL4A1 n = 1). CONCLUSION: Young adults with vascular WML evaluated in Neurology outpatient clinics concentrate in the oldest age groups. Vascular risk factors should be screened carefully in this population. Among patients without history of stroke, females largely outweigh males. Diagnostic investigations performed do not follow a standardized protocol.

Skiing Associated Stroke: Causes, Treatment, and Outcome.

BACKGROUND: Previous studies have described ischemic stroke temporally related to specific triggers, but only 1 series collected patients with acute ischemic stroke (AIS) following downhill skiing and all caused by cervical artery dissections. Here we describe our series of AIS temporally associated to ski practice, focusing on the frequency, pathogenesis, clinical presentation, and prognosis. METHODS: We maintained a prospective list of Skiing Associated Strokes (SASs) from 2003 to 2017. From all AIS patients included in our stroke registry Acute Stroke Registry and Analysis of Lausanne (ASTRAL) over the same period, we identified a comparison group of non-SAS patients, matched for age and gender. RESULTS: In the 12-year observation period, we identified 17 SASs (4 females, median age 51 years) and 51 matched control patients with nonski-associated strokes. Vascular risk factors, stroke features, and outcome were similar between the 2 groups. Stroke mechanism was arterial dissection in 11 of 17 SASs (65%) and in 7 of 51 control patients (14%, chi-square test: P < .05). In the other 6 cases of ski-associated stroke, etiology was cardiac embolism from atrial fibrillation in 2 patients, large vessel atherosclerosis with stenosis >50% in 1 patient, and undetermined in 3. Among the 11 patients with SAS caused by dissection, 8 reported minor falls while skiing, 1 had a major head trauma without loss of consciousness, and 2 had no traumatism (compared to preceding trauma in 29 of 147 [20%] of all other AIS caused by arterial dissection in ASTRAL, P < .01). CONCLUSIONS: Arterial dissection was a significantly more frequent stroke mechanism in SAS compared to matched controls, but other mechanisms occurred as well. Minor or moderate skiing-related trauma preceded most SAS with dissections.

Women Who Have Ischemic Strokes Have a Higher Burden of Left Atrial Fibrosis Than Men.

Background and Purpose- Women with atrial fibrillation (AF) have a higher risk of stroke than men who have AF. Atrial fibrosis is a marker of atrial disease that precedes the appearance of AF increasing the risk of ischemic stroke. We aimed to determine whether female sex is independently associated with left atrial fibrosis in stroke patients. Methods- We prospectively included a consecutive sample of ischemic stroke patients aged over 50 years of age. Late gadolinium enhancement cardiac magnetic resonance imaging was performed to quantify the severity of left atrial fibrosis and the wall pattern of its distribution. A multivariable linear regression analysis was performed to determine whether female sex was independently associated with left atrial fibrosis after adjusting for potential confounders namely AF and age. Results- One hundred twenty-four patients were deemed eligible; 117 patients were included (7 were excluded because of cardiomyopathy identified by cardiac magnetic resonance imaging). All had usable cardiac magnetic resonance imaging data. Fifty-three patients (45.3%) were women. Women were older and were less frequently treated with angiotensin-converting-enzyme inhibitors. Ninety-one patients had any degree of atrial fibrosis. Women had a higher percentage of atrial fibrosis than men-median (interquartile range)-18% (17) versus 10% (20). In a multivariable linear regression model adjusted for demographics, medications, AF, comorbidities, and cardiac parameters, female sex was found to be independently associated with left atrial fibrosis. Women were found to have more 4.70% of left atrial fibrosis than men (95% CI, 0.70-8.71%; P=0.02) after controlling for confounders. Conclusions- Female sex was found to be independently associated with left atrial fibrosis after controlling for confounders such as AF and age. Further studies are needed to understand if this contributes to the increased stroke risk related to AF in women compared with men.

Patients With Undetermined Stroke Have Increased Atrial Fibrosis: A Cardiac Magnetic Resonance Imaging Study.

BACKGROUND AND PURPOSE: Some patients with ischemic strokes that are currently classified as having an undetermined cause may have structural or functional changes of the left atrium (LA) and left atrial appendage, which increase their risk of thromboembolism. We compared the LA and left atrial appendage of patients with different ischemic stroke causes using cardiac magnetic resonance imaging. METHODS: We prospectively included a consecutive sample of ischemic stroke patients. Patients with structural changes on echocardiography currently considered as causal for stroke in the Trial of ORG 10172 in Acute Stroke Treatment (TOAST) classification were excluded. A 3-T cardiac magnetic resonance imaging was performed. RESULTS: One hundred and eleven patients were evaluated. Patients with an undetermined cause had a higher percentage of LA fibrosis (P=0.03) than patients with other stroke causes and lower, although not statistically significant, values of LA ejection fraction. Patients with atrial fibrillation and undetermined stroke cause showed a similar value of atrial fibrosis. CONCLUSIONS: The LA phenotype that was found in patients with undetermined cause supports the hypothesis that an atrial disease may be associated with stroke.

The electroclinical spectrum, etiologies, treatment and outcome of nonconvulsive status epilepticus in the elderly.

BACKGROUND: Nonconvulsive status epilepticus (NCSE) in the elderly is particularly difficult to diagnose, mainly due to subtle clinical manifestations and associated comorbidities. The recently validated electroencephalography (EEG) diagnostic criteria for NCSE and the proposed operational classification of status epilepticus provide tools that can allow an earlier diagnosis and better management of NCSE in this age group, possibly contributing to reduce its high mortality. MATERIAL AND METHODS: we used these tools to identify and characterize a cohort of elderly (>60year-old) patients admitted at our institution in a 3-year period; the video-EEG and clinical files of the patients fulfilling EEG diagnostic criteria for NCSE were reviewed, being in this study described their electroclinical spectrum, etiologies, treatment, inhospital mortality, and status epilepticus severity score (STESS). RESULTS: Fourty patients (23 women; mean age 76.6years) were identified. Although dyscognitive NCSE associated with >2.5Hz of epileptiform discharges (ED) was the most frequent electroclinical phenotype, this was quite heterogeneous, ranging from patients with aura continua to patients in coma, associated with frequent ED or rhythmic slow activities. Acute symptomatic (45%) and multifactorial (27.5%) etiologies were the most common, and associated with the worst prognosis. There was a trend to use newer antiepileptic drugs in the early steps of NCSE treatment. The inhospital mortality was high (22.5%) and predicted by STESS scores ≥3. CONCLUSION: In the elderly, NCSE has heterogeneous electroclinical phenotypes and etiologies. In spite of the treatment limitations conditioned by the comorbidities, more aggressive treatments could be justified to reduce mortality in patients with high STESS scores.

Headache as the sole presentation of cerebral venous thrombosis: a prospective study.

Headache is the most frequent presenting symptom of cerebral venous thrombosis (CVT), most commonly associated with other manifestations. It has been described as its only clinical presentation in 15 % of patients. There is no typical pattern of headache in CVT. The objective of this study was to study the characteristics of headache as the sole manifestation of CVT. From a prospective study of 30 consecutive patients diagnosed with CVT over 18 months, we selected those who presented with headache only: they had a normal neurological examination, no papilloedema and no blood or any parenchymal lesion on CT scan. All were submitted to a systematic etiological workup and a structured questionnaire about the characteristics of headache was provided. Headache was the sole manifestation of CVT in 12 patients; it was diffuse or bilateral in the majority. Seven patients referred worsening with sleep/lying down, Valsalva maneuvers or straining. There was no association between the characteristics of headache and extension of CVT. Time from onset to diagnosis was significantly delayed in these patients presenting only with headache. In our series, 40 % of patients presented only with headache. There was no uniform pattern of headache apart from being bilateral. There was a significant delay of diagnosis in these patients. Some characteristics of headache should raise the suspicion of CVT: recent persistent headache, thunderclap headache or pain worsening with straining, sleep/lying down or Valsalva maneuvers even in the absence of papilloedema or focal signs.