Maximiliano A. Hawkes

Autosomal dominant cerebellar ataxias: a systematic review of clinical features.

To assess, through systematic review, distinctive or common clinical signs of autosomal dominant cerebellar ataxias (ADCAs), also referred to as spinocerebellar ataxias (SCAs) in genetic nomenclature.

Risk Stratification for Recurrence and Mortality in Embolic Stroke of Undetermined Source

Background and Purpose— The risk of stroke recurrence in patients with Embolic Stroke of Undetermined Source (ESUS) is high, and the optimal antithrombotic strategy for secondary prevention is unclear. We investigated whether congestive heart failure, hypertension, age ≥75 years, diabetes mellitus, and stroke or transient ischemic attack (TIA; CHADS2) and CHA2DS2-VASc scores can stratify the long-term risk of ischemic stroke/TIA recurrence and death in ESUS. Methods— We pooled data sets of 11 stroke registries from Europe and America. ESUS was defined according to the Cryptogenic Stroke/ESUS International Working Group. Cox regression analyses were performed to investigate if prestroke CHADS2 and congestive heart failure, hypertension, age ≥75 years, diabetes mellitus, stroke or TIA, vascular disease, age 65–74 years, sex category (CHA2DS2-VASc) scores were independently associated with the risk of ischemic stroke/TIA recurrence or death. The Kaplan–Meier product limit method was used to estimate the cumulative probability of ischemic stroke/TIA recurrence and death in different strata of the CHADS2 and CHA2DS2-VASc scores. Results— One hundred fifty-nine (5.6% per year) ischemic stroke/TIA recurrences and 148 (5.2% per year) deaths occurred in 1095 patients (median age, 68 years) followed-up for a median of 31 months. Compared with CHADS2 score 0, patients with CHADS2 score 1 and CHADS2 score >1 had higher risk of ischemic stroke/TIA recurrence (hazard ratio [HR], 2.38; 95% confidence interval [CI], 1.41–4.00 and HR, 2.72; 95% CI, 1.68–4.40, respectively) and death (HR, 3.58; 95% CI, 1.80–7.12, and HR, 5.45; 95% CI, 2.86–10.40, respectively). Compared with low-risk CHA2DS2-VASc score, patients with high-risk CHA2DS2-VASc score had higher risk of ischemic stroke/TIA recurrence (HR, 3.35; 95% CI, 1.94–5.80) and death (HR, 13.0; 95% CI, 4.7–35.4). Conclusions— The risk of recurrent ischemic stroke/TIA and death in ESUS is reliably stratified by CHADS2 and CHA2DS2-VASc scores. Compared with the low-risk group, patients in the high-risk CHA2DS2-VASc group have much higher risk of ischemic stroke recurrence/TIA and death, approximately 3-fold and 13-fold, respectively.

Age- and sex-specific analysis of patients with embolic stroke of undetermined source

Objective: To investigate whether the correlation of age and sex with the risk of recurrence and death seen in patients with previous ischemic stroke is also evident in patients with embolic stroke of undetermined source (ESUS). Methods: We pooled datasets of 11 stroke registries from Europe and America. ESUS was defined according to the Cryptogenic Stroke/ESUS International Working Group. We performed Cox regression and Kaplan-Meier product limit analyses to investigate whether age (<60, 60–80, >80 years) and sex were independently associated with the risk for ischemic stroke/TIA recurrence or death. Results: Ischemic stroke/TIA recurrences and deaths per 100 patient-years were 2.46 and 1.01 in patients <60 years old, 5.76 and 5.23 in patients 60 to 80 years old, 7.88 and 11.58 in those >80 years old, 3.53 and 3.48 in women, and 4.49 and 3.98 in men, respectively. Female sex was not associated with increased risk for recurrent ischemic stroke/TIA (hazard ratio [HR] 1.15, 95% confidence interval [CI] 0.84–1.58) or death (HR 1.35, 95% CI 0.97–1.86). Compared with the group <60 years old, the 60- to 80- and >80-year groups had higher 10-year cumulative probability of recurrent ischemic stroke/TIA (14.0%, 47.9%, and 37.0%, respectively, p < 0.001) and death (6.4%, 40.6%, and 100%, respectively, p < 0.001) and higher risk for recurrent ischemic stroke/TIA (HR 1.90, 95% CI 1.21–2.98 and HR 2.71, 95% CI 1.57–4.70, respectively) and death (HR 4.43, 95% CI 2.32–8.44 and HR 8.01, 95% CI 3.98–16.10, respectively). Conclusions: Age, but not sex, is a strong predictor of stroke recurrence and death in ESUS. The risk is ≈3- and 8-fold higher in patients >80 years compared with those <60 years of age, respectively. The age distribution in the ongoing ESUS trials may potentially influence their power to detect a significant treatment association.

Bilateral Paramedian Thalamic Infarction.

Introduction:Rarely, both paramedian thalami receive arterial blood flow from a single unilateral vessel arising from the first segment of 1 posterior cerebral artery. This artery has received the name of artery of Percheron (AP). There is no consensus regarding the true prevalence of this anatomical variant. Bilateral paramedian thalamic infarcts are uncommon (0.1% to 2% of ischemic strokes). The main cause is the occlusion of the AP due to cardioembolism. Diffusion-weighted magnetic resonance imaging demonstrates the lesion in the acute setting. Materials and Methods:From September 2004 to October 2011, we identified 5 patients who had bilateral paramedian thalamic infarcts. We describe clinical findings and diagnostic imaging patterns observed in these cases and review the literature. Results:Three men and 2 women with bilateral paramedian thalamic infarction probably due to occlusion of AP are described. Mean age at presentation was 58±24 years. Magnetic resonance imaging showed the lesion in all patients. Four patients presented loss of consciousness as initial symptom. Only 1 patient evidenced mesencephalic extension of the infarct on magnetic resonance imaging, although 4 presented abnormal ocular signs. No patients received intravenous thrombolisis because of delayed diagnosis. All patients were discharged home. A 90-year-old woman recovered completely and the other 4 subjects persisted with cognitive symptoms and gaze abnormalities. Conclusions:Clinical presentation and imaging patterns described in this group of patients were similar to published data. High level of suspicion based on clinical and imaging findings is essential for early diagnosis of this rare condition. None of our patients had an early diagnosis of acute ischemic stroke and received proper thrombolytic treatment.

Differential characteristics, stroke recurrence, and predictors of covert atrial fibrillation of embolic strokes of undetermined source

Background and purpose Identifying embolic strokes of undetermined source (ESUS) patients likely to harbor atrial fibrillation may have diagnostic and therapeutic implications. Our aim was to examine differences between ESUS and cardioembolic strokes, to evaluate stroke recurrence rate among ESUS and to identify baseline characteristics of ESUS patients who were later diagnosed with atrial fibrillation. Materials and methods We assessed all ischemic stroke patients admitted between June 2012 and November 2013. ESUS were compared to cardioembolic strokes at discharge. After at least 12-month follow-up, ESUS patients diagnosed with atrial fibrillation were compared to those who remained as ESUS. Results There were 236 ischemic strokes, 32.6% were ESUS. Compared to cardioembolic strokes, ESUS were younger (p < 0.0001), had milder strokes (p < 0.05), less prevalence of hypertension (p < 0.05), peripheral vascular disease (p < 0.05), and previous ischemic stroke (p < 0.05). After follow-up, 15% of ESUS patients had stroke recurrences and 12% evidenced paroxysmal atrial fibrillation. ESUS patients diagnosed with atrial fibrillation in the follow-up were older (p < 0.0001), had higher erythrocyte sedimentation rate (p < 0.05), and were more likely to have ≥2 infarcts in the same arterial territory in the initial magnetic resonance imaging (p < 0.05). Conclusions Older age, small-scattered infarcts on initial magnetic resonance imaging and high erythrocyte sedimentation rate levels appear to identify ESUS patients more likely to be diagnosed of atrial fibrillation during follow-up.

Door-to-Needle Time in Acute Stroke Treatment and the "July Effect".

Intravenous thrombolysis improves outcomes in acute ischemic stroke in a time-dependent fashion. As in teaching hospitals, clinical outcomes may worsen due to the arrival of new inexperienced house staff early in the academic year (July effect, JE), we evaluated the impact of the “JE” on the door-to-needle time for intravenous thrombolysis and other stroke outcomes. In this retrospective cohort study, we assessed all acute ischemic strokes treated with intravenous thrombolysis between July 2003 and June 2016. Among 101 patients, there was no detrimental July effect on the door-to-needle time, rate of thrombolysis within 60 minutes of arrival, thrombolysis of stroke mimics, post-thrombolysis intracranial hemorrhages, National Institutes of Health Stroke Scale, and modified Rankin Scale outcomes.

Systemic Complications Following Status Epilepticus

Purpose of ReviewStatus epilepticus (SE) is a multisystem disorder. Initially, complications of a massive catecholamine release followed by the side effects of medical therapies, impact patients’ outcomes. The aim of this article is to provide an updated summary of the systemic complications following SE.Recent FindingsIn recent years, the importance of the multifaceted nature of SE and its relationship with clinical outcomes has been increasingly recognized. The cumulative systemic effects of prolonged seizures and their treatment contribute to morbidity and mortality in this condition.SummaryMost systemic complications after SE are predictable. Anticipating their occurrence and respecting a number of simple guidelines may improve the prognosis of these patients.

Left atrial appendage occlusion with Amplatzer Cardio Plug is an acceptable therapeutic option for prevention of stroke recurrence in patients with non-valvular atrial fibrillation and contraindication or failure of oral anticoagulation with acenocumarol

UNLABELLED Left atrial appendage occlusion (LAAO) appears as a therapeutic option for some atrial fibrillation patients not suitable for oral anticoagulation because an increased hemorrhagic risk or recurrent ischemic events despite anticoagulant treatment. METHODS Report of consecutive atrial fibrillation patients treated with LAAO with Amplatzer Cardio Plug because contraindication or failure of oral anticoagulation with acenocumarol. CHA2DS2VASC, HAS-BLED, NIHSS, mRS, procedural complications and outcome were assessed. Seven patients (73 ± 6 year-old) were treated after intracerebral (n = 5) and gastrointestinal (n = 1) hemorrhages or ischemic stroke recurrence while on acenocumarol (n = 1). RESULTS Mean follow up was 18 months. Baseline CHA2DS2Vasc y HAS-BLED scores were 5.6 ± 0.7 and 4.1 ± 0.3 respectively. There were no strokes or deaths. There was only one non-serious adverse event. CONCLUSION LAAO with ACP appears as a feasible therapeutic option for stroke prevention in patients with atrial fibrillation and failure or contraindication to acenocumarol.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) in Argentina.

CADASIL is the most common cause of hereditary stroke and vascular dementia. Published information about this disease in South America is scant. We describe clinical and demographic characteristics of 13 patients (10 families) with CADASIL from Argentina.Methods Medical records, diagnostic tests and family history of patients with CADASIL were reviewed.Results Thirteen patients with CADASIL (10 families) were included. All patients had European ancestry. Initial presentation was stroke in most patients (n = 11). Stroke patients later developed cognitive complaints (n = 9), migraine with aura (n = 1), apathy (n = 4) and depression (n = 6). External capsule and temporal lobe involvement on MRI were characteristic imaging findings. Two patients died after intracerebral hemorrhage.Conclusion This is the first report of non-related patients with CADASIL in South America addressing ancestry. Since European ancestry is not highly prevalent in all South American countries, there may be variable incidence of CADASIL within this region.

Cervical spine disease: A possible cause of neuromuscular respiratory failure

Identifying the cause of neuromuscular respiratory failure is essential for guiding therapy and has major prognostic implications.1 The most frequent causes can be easily recognized but diagnosing less common etiologies can be challenging.