An algorithm for the National Institute of Health Stroke Scale assessment: A multicenter, two-arm and cluster randomized study.

BACKGROUND: The NIH Stroke Scale (NIHSS) is a validated tool for assessing stroke severity, increasingly used by general practitioners in telemedicine services. Mobile apps may enhance its reliability. We aim to validate a digital platform (SPOKES) for NIHSS assessment in telemedicine and healthcare settings. METHODS: Hospitals using a telemedicine service were randomly allocated to control or SPOKES-user groups. The discrepancy between the NIHSS scores reported and those confirmed by experts was evaluated. Healthcare providers from comprehensive stroke centers were invited for interrater validation. Participants were randomized to assess the NIHSS using videos of real patients. Weighted Kappa (wk) statistics analyzed the agreement, and logistic regression determined the correlation with the congruency. RESULTS: A total of 299 telemedicine consultations from 12 hospitals were included. The difference between the NIHSS scores reported and double-checked was lower in the SPOKES group (p = 0.03), with a significantly higher level of complete agreement (72.5 % vs. 50.4 %, p = 0.005). Adoption of SPOKES was associated with complete congruency (OR 4.01, 95 %CI 1.42-11.35, p = 0.009). For interrater validation, 20 participants were considered. In the SPOKES group, almost-perfect and strong agreement occurred in 13.3 %(n = 6/45) and 84.4 %(n = 38/45) of ratings, respectively; in the control group, 6.7 %(n = 3/45) were almost-perfect, 28.9 %(n = 13/45) strong and 51 %(n = 23/45) were minimal. CONCLUSION: A free and reliable mobile application for NIHSS assessment can significantly improve interrater agreement between healthcare professionals, and between NIHSS-certified neurologists and general practitioners. Our results underscore the importance of ongoing training and education in enhancing the consistency and reliability of NIHSS scores.

A Brazilian series utilizing the SMASH-U system for etiologic classification of intracerebral hemorrhage.

BACKGROUND: Understanding the causes of intracerebral hemorrhage (ICH) is crucial for effective treatment and preventing recurrences. The SMASH-U scale is a suggested method for classifying and predicting the outcomes of ICH. OBJECTIVE: To describe the SMASH-U classification and outcomes by etiology in patients admitted to a comprehensive stroke center in São Paulo, Brazil. METHODS: A retrospective analysis was conducted on patients admitted to the hospital or outpatient clinic between April 2015 and January 2018. Two stroke neurologists evaluated the SMASH-U classification, and patients with incomplete medical records were excluded. RESULTS: Out of the 2000 patients with a stroke diagnosis evaluated, 140 were included in the final analysis. The mean age was 57.9 (± 15.5) years, and 54.3% were male. Hypertension was the most frequent etiology, accounting for 41.4% of cases, followed by amyloid angiopathy (18.5%) and structural lesions (14.1%). Structural lesions were more common among women and patients under 45 years old. Favorable outcomes were observed in 61% of patients with structural lesions, compared to 10% of patients with medication-related etiologies. CONCLUSION: This study provides important evidence regarding the etiological classification of Brazilian patients with ICH. Hypertension and amyloid angiopathy were the most frequent causes, while structural lesions and systemic diseases were more common in younger patients.

ANTECEDENTES: Compreender as causas da hemorragia intracerebral (HIC) é crucial para o tratamento eficaz e prevenção de recorrências. A escala SMASH-U é um método sugerido para classificar e prever os resultados da HIC. OBJETIVO: Descrever a classificação SMASH-U e os resultados por etiologia em pacientes admitidos em um centro de acidente vascular cerebral (AVC) em São Paulo, Brasil. MéTODOS: Foi realizada uma análise retrospectiva de pacientes admitidos no hospital ou ambulatório entre abril de 2015 e janeiro de 2018. Dois neurologistas especializados em doenças cerebrovasculares avaliaram a classificação SMASH-U e pacientes com prontuários incompletos foram excluídos. RESULTADOS: Dos 2000 pacientes com diagnóstico de AVC avaliados, 140 foram incluídos na análise final. A idade média foi de 57,9 (±15,5) anos e 54,3% eram do sexo masculino. A hipertensão foi a etiologia mais frequente, correspondendo a 41,4% dos casos, seguida pela angiopatia amiloide (18,5%) e lesões estruturais (14,1%). As lesões estruturais foram mais comuns em mulheres e pacientes com menos de 45 anos. Resultados favoráveis foram observados em 61% dos pacientes com lesões estruturais, em comparação com 10% dos pacientes com etiologias relacionadas a medicamentos. CONCLUSãO: Este estudo fornece evidências importantes sobre a classificação etiológica de pacientes brasileiros com HIC. A hipertensão e a angiopatia amiloide foram as causas mais frequentes, enquanto lesões estruturais e doenças sistêmicas foram mais comuns em pacientes mais jovens.

Optic Disc and Retinal Architecture Changes in Patients with Spinocerebellar Ataxia Type 2.

BACKGROUND: ATXN2 is the causative gene of spinocerebellar ataxia type 2 (SCA2) and has been implicated in glaucoma pathogenesis. Therefore, studying ocular changes in SCA2 could uncover clinically relevant changes. OBJECTIVE: The aim was to investigate optic disc and retinal architecture in SCA2. METHODS: We evaluated 14 patients with SCA2 and 26 controls who underwent intraocular pressure measurement, fundoscopy, and macular and peripapillary spectral domain optical coherence tomography (SD-OCT). We compared SD-OCT measurements in SCA2 and controls, and the frequency of glaucomatous changes among SCA2, controls, and 76 patients with other SCAs (types 1, 3, 6, and 7). RESULTS: The macula, peripapillary retinal nerve fiber and inner plexiform layers were thinner in SCA2 than in controls. Increased cup-to-disc ratio was more frequent in SCA2 than in controls and other SCAs. CONCLUSIONS: Ocular changes are part of SCA2 phenotype. Future studies should further investigate retinal and optic nerve architecture in this disorder.

A Brazilian series utilizing the SMASH-U system for etiologic classification of intracerebral hemorrhage / Classificação etiológica de pacientes admitidos com hemorragia intracerebral utilizando a escala SMASH-U: uma série brasileira

Abstract Background Understanding the causes of intracerebral hemorrhage (ICH) is crucial for effective treatment and preventing recurrences. The SMASH-U scale is a suggested method for classifying and predicting the outcomes of ICH. Objective To describe the SMASH-U classification and outcomes by etiology in patients admitted to a comprehensive stroke center in São Paulo, Brazil. Methods A retrospective analysis was conducted on patients admitted to the hospital or outpatient clinic between April 2015 and January 2018. Two stroke neurologists evaluated the SMASH-U classification, and patients with incomplete medical records were excluded. Results Out of the 2000 patients with a stroke diagnosis evaluated, 140 were included in the final analysis. The mean age was 57.9 (± 15.5) years, and 54.3% were male. Hypertension was the most frequent etiology, accounting for 41.4% of cases, followed by amyloid angiopathy (18.5%) and structural lesions (14.1%). Structural lesions were more common among women and patients under 45 years old. Favorable outcomes were observed in 61% of patients with structural lesions, compared to 10% of patients with medication-related etiologies. Conclusion This study provides important evidence regarding the etiological classification of Brazilian patients with ICH. Hypertension and amyloid angiopathy were the most frequent causes, while structural lesions and systemic diseases were more common in younger patients.

Resumo Antecedentes Compreender as causas da hemorragia intracerebral (HIC) é crucial para o tratamento eficaz e prevenção de recorrências. A escala SMASH-U é um método sugerido para classificar e prever os resultados da HIC. Objetivo Descrever a classificação SMASH-U e os resultados por etiologia em pacientes admitidos em um centro de acidente vascular cerebral (AVC) em São Paulo, Brasil. Métodos Foi realizada uma análise retrospectiva de pacientes admitidos no hospital ou ambulatório entre abril de 2015 e janeiro de 2018. Dois neurologistas especializados em doenças cerebrovasculares avaliaram a classificação SMASH-U e pacientes com prontuários incompletos foram excluídos. Resultados Dos 2000 pacientes com diagnóstico de AVC avaliados, 140 foram incluídos na análise final. A idade média foi de 57,9 (±15,5) anos e 54,3% eram do sexo masculino. A hipertensão foi a etiologia mais frequente, correspondendo a 41,4% dos casos, seguida pela angiopatia amiloide (18,5%) e lesões estruturais (14,1%). As lesões estruturais foram mais comuns em mulheres e pacientes com menos de 45 anos. Resultados favoráveis foram observados em 61% dos pacientes com lesões estruturais, em comparação com 10% dos pacientes com etiologias relacionadas a medicamentos. Conclusão Este estudo fornece evidências importantes sobre a classificação etiológica de pacientes brasileiros com HIC. A hipertensão e a angiopatia amiloide foram as causas mais frequentes, enquanto lesões estruturais e doenças sistêmicas foram mais comuns em pacientes mais jovens.

Reperfusion therapy for acute ischemic stroke: where are we in 2023?

Over the last three decades, stroke care has undergone significant transformations mainly driven by the introduction of reperfusion therapy and the organization of systems of care. Patients receiving treatment through a well-structured stroke service have a much higher chance of favorable outcomes, thereby decreasing both disability and mortality. In this article, we reviewed the scientific evidence for stroke reperfusion therapy, including thrombolysis and thrombectomy, and its implementation in the public health system in Brazil.

Nas últimas três décadas, o tratamento do AVC sofreu transformações significativas, impulsionadas principalmente pela introdução das terapias de reperfusão e pela organização dos serviços de AVC. Os pacientes que recebem tratamento em um serviço de AVC bem estruturado têm uma probabilidade muito maior de resultados favoráveis, diminuindo assim a incapacidade funcional e a mortalidade. Neste artigo, revisamos as evidências científicas para as terapias de reperfusão do AVC, incluindo trombólise e trombectomia e sua implementação no sistema público de saúde no Brasil.

Reperfusion therapy for acute ischemic stroke: where are we in 2023? / Terapia de reperfusão para o acidente vascular isquêmico agudo: onde estamos em 2023?

Abstract Over the last three decades, stroke care has undergone significant transformations mainly driven by the introduction of reperfusion therapy and the organization of systems of care. Patients receiving treatment through a well-structured stroke service have a much higher chance of favorable outcomes, thereby decreasing both disability and mortality. In this article, we reviewed the scientific evidence for stroke reperfusion therapy, including thrombolysis and thrombectomy, and its implementation in the public health system in Brazil.

Resumo Nas últimas três décadas, o tratamento do AVC sofreu transformações significativas, impulsionadas principalmente pela introdução das terapias de reperfusão e pela organização dos serviços de AVC. Os pacientes que recebem tratamento em um serviço de AVC bem estruturado têm uma probabilidade muito maior de resultados favoráveis, diminuindo assim a incapacidade funcional e a mortalidade. Neste artigo, revisamos as evidências científicas para as terapias de reperfusão do AVC, incluindo trombólise e trombectomia e sua implementação no sistema público de saúde no Brasil.

Multimodal monitoring intracranial pressure by invasive and noninvasive means.

Although the placement of an intraventricular catheter remains the gold standard method for the diagnosis of intracranial hypertension (ICH), the technique has several limitations including but not limited to its invasiveness. Current noninvasive methods, however, still lack robust evidence to support their clinical use. We aimed to estimate, as an exploratory hypothesis generating analysis, the discriminative power of four noninvasive methods to diagnose ICH. We prospectively collected data from adult intensive care unit (ICU) patients with subarachnoid hemorrhage (SAH), intraparenchymal hemorrhage (IPH), and ischemic stroke (IS) in whom invasive intracranial pressure (ICP) monitoring had been placed. Measures were simultaneously collected from the following noninvasive methods: optic nerve sheath diameter (ONSD), pulsatility index (PI) using transcranial Doppler (TCD), a 5-point visual scale designed for brain Computed Tomography (CT), and two parameters (time-to-peak [TTP] and P2/P1 ratio) of a noninvasive ICP wave morphology monitor (Brain4Care[B4c]). ICH was defined as a sustained ICP > 20 mmHg for at least 5 min. We studied 18 patients (SAH = 14; ICH = 3; IS = 1) on 60 occasions with a mean age of 52 ± 14.3 years. All methods were recorded simultaneously, except for the CT, which was performed within 24 h of the other methods. The median ICP was 13 [9.8-16.2] mmHg, and intracranial hypertension was present on 18 occasions (30%). Median values from the noninvasive techniques were ONSD 4.9 [4.40-5.41] mm, PI 1.22 [1.04-1.43], CT scale 3 points [IQR: 3.0], P2/P1 ratio 1.16 [1.09-1.23], and TTP 0.215 [0.193-0.237]. There was a significant statistical correlation between all the noninvasive techniques and invasive ICP (ONSD, r = 0.29; PI, r = 0.62; CT, r = 0.21; P2/P1 ratio, r = 0.35; TTP, r = 0.35, p < 0.001 for all comparisons). The area under the curve (AUC) to estimate intracranial hypertension was 0.69 [CIs = 0.62-0.78] for the ONSD, 0.75 [95% CIs 0.69-0.83] for the PI, 0.64 [95%Cis 0.59-069] for CT, 0.79 [95% CIs 0.72-0.93] for P2/P1 ratio, and 0.69 [95% CIs 0.60-0.74] for TTP. When the various techniques were combined, an AUC of 0.86 [0.76-0.93]) was obtained. The best pair of methods was the TCD and B4cth an AUC of 0.80 (0.72-0.88). Noninvasive technique measurements correlate with ICP and have an acceptable discrimination ability in diagnosing ICH. The multimodal combination of PI (TCD) and wave morphology monitor may improve the ability of the noninvasive methods to diagnose ICH. The observed variability in non-invasive ICP estimations underscores the need for comprehensive investigations to elucidate the optimal method-application alignment across distinct clinical scenarios.

Predictors of congruency between clinical and radiographic Oxfordshire Community Stroke Project Classification subtypes.

BACKGROUND: The Oxfordshire Community Stroke Project (OCSP) classification has been widely used to assess ischemic stroke patients based on clinical characteristics alone. However, the correlation between the clinical presentation evaluated using OCSP and imaging findings is yet to be determined. Our study aimed to describe the baseline characteristics of the OCSP subtypes of patients admitted with ischemic stroke and evaluate the predictors of the relationship between clinical and neuroimaging findings. METHODS: Patients with a confirmed diagnosis of ischemic stroke admitted to a comprehensive stroke center in Brazil between February 2015 and October 2017 were eligible for the study. All participants underwent computed tomography (CT) at admission and follow-up neuroimaging within seven days, per the institutional protocol. Trained staff classified patients according to the OCSP at hospital admission. The radiographic OCSP classification was retrospectively assessed based on the last follow-up neuroimaging by investigators unaware of the clinical classification. RESULTS: The overall agreement rates ranged from 65.5% to 88.7%. Lower NIHSS scores, absent hyperdense MCA sign, higher ASPECTS, and absent brainstem symptoms were related to a higher risk of misclassification. Treatment with intravenous tPA was associated with reclassification in patients with total anterior circulation syndrome. For predicting radiographic posterior circulation involvement, vertigo (OR 2.9, 95% CI 1.7-5.1, p < 0.001) and brainstem symptoms (OR 35, 95% CI 20.5-60.2, p < 0.001) were directly associated with correct classification, but motor and higher cerebral function were not correlated. CONCLUSION: The clinical OCSP classification presented good congruency rates with the neuroimaging findings. However, patients with lacunar syndromes are often misclassified when radiological criteria are considered.

Predicting hemorrhagic transformation in posterior circulation stroke patients not treated with reperfusion therapies.

INTRODUCTION: Posterior Circulation (PC) stroke represents one-fifth of all ischemic strokes, with peculiar physiological characteristics. Hemorrhagic Transformation (HT) is a dreaded complication among stroke patients. Many predictive scores of this complication have been proposed, but none is designed specifically for PC stroke patients - therefore, patients who are not eligible for reperfusion therapies (RT) represent about 80% of hospitalized cases. We propose a scoring system to assess the HT risk in PC stroke patients not submitted to RT. METHODS: We retrospectively evaluated data of patients diagnosed with PC stroke not treated with RT from 5 Comprehensive Stroke Centers (four in Brazil, 1 in the US) from 2015 to 2018. All patients underwent CT scan or MRI at admission and a follow-up neuroimaging within seven days. Independent variables identified in a logistic regression analysis were used to produce a predictive grading score. RESULTS: We included 952 patients in the final analysis. The overall incidence of HT was 8.7%. Male gender (1 point), NIH Stroke Scale at admission ≥ 5 points (1), blood glucose at admission ≥ 160 mg/dL (1), and cardioembolism (2) were independently associated with HT. The AUC of the grading score (0 to 5 points) was 0.713 (95% CI 0.65-0.78). Subjects with a score ≥ 3 points had an OR of 4.8 (95% CI 2.9-7.9, p < 0.001) for HT. CONCLUSIONS: Our score has good accuracy in identifying patients at higher risk of HT. This score may be useful for evaluating secondary prevention and stratifying patients in the context of even clinical trials.

Accuracy of predictive scores of hemorrhagic transformation in patients with acute ischemic stroke / Acurácia de escores preditivos de transformação hemorrágica em pacientes com isquemia cerebral aguda

ABSTRACT Background: Hemorrhagic transformation (HT) is a complication in ischemic strokes, regardless of use of reperfusion therapy (RT). There are many predictive scores for estimating the risk of HT. However, most of them include patients also treated with RT. Therefore, this may lead to a misinterpretation of the risk of HT in patients who did not undergo RT. Objective: We aimed to review published predictive scores and analyze their accuracy in our dataset. Methods: We analyzed the accuracy of seven scales. Our dataset was derived from a cohort of 1,565 consecutive patients from 2015 to 2017 who were admitted to a comprehensive stroke center. All patients were evaluated with follow-up neuroimaging within seven days. Comparison of area under the curve (AUC) was performed on each scale, to analyze differences between patients treated with recombinant tissue plasminogen activator (tPA) and those without this treatment. Results: Our dataset provided enough data to assess seven scales, among which six were used among patients with and without tPA treatment. HAT (AUC 0.76), HTI (0.73) and SEDAN (0.70) were the most accurate scores for patients not treated with tPA. SPAN-100 (0.55) had the worst accuracy in both groups. Three of these scores had different cutoffs between study groups. Conclusions: The predictive scores had moderate to fair accuracy for predicting HT in patients treated with tPA. Three scales were more accurate for predicting HT in patients not treated with tPA. Through standardizing these characteristics and including more patients not treated with RT in a large multicenter series, accurate predictive scores may be created.

RESUMO Background: Transformação hemorrágica (TH) é uma complicação frequente no acidente vascular cerebral (AVC) isquêmico independente do uso de terapia de reperfusão (TR). Diversos escores preditivos de TH foram elaborados. Entretanto, a maioria desses escores incluíram pacientes submetidos a TR — o que pode levar à má interpretação do risco de TH nos pacientes não submetidos a TR. Objetivo: Nosso objetivo é revisar escores preditivos já publicados e analisar a sua acurácia em nossa amostra. Métodos: Analisamos a acurácia de sete escores. Nosso banco foi criado de uma coorte de 1.565 pacientes consecutivos, admitidos entre 2015 e 2017 em um centro avançado de AVC. Os pacientes realizaram neuroimagem de controle em até sete dias. Uma comparação entre áreas abaixo da curva/característica de operação do receptor (AUC) foi realizada, analisando-se as diferenças entre grupos de pacientes tratados ou não com ativador de plasminogênio tecidual recombinante (tPA). Resultados: Nosso banco de dados proporcionou informação suficiente para avaliar sete escores, dos quais seis foram aplicados em pacientes tratados ou não com tPA. HAT (AUC 0,76), HTI (0,73) e SEDAN (0,70) foram os escores mais acurados em pacientes não tratados com tPA. SPAN-100 (0,55) teve a pior acurácia nos grupos. Três desses escores apresentaram diferentes valores de corte entre os grupos. Conclusões: Os escores apresentaram de boa a moderada acurácia na predição de TH em pacientes tratados com tPA. Três escores foram mais acurados em pacientes não tratados com tPA. A parametrização dessas características e a inclusão de mais pacientes não tratados com TR em um estudo multicêntrico poderia levar a escores mais acurados.

Waveform Morphology as a Surrogate for ICP Monitoring: A Comparison Between an Invasive and a Noninvasive Method.

BACKGROUND: Although the placement of an intraventricular catheter remains the gold standard technique for measuring intracranial pressure (ICP), the method has several limitations. Therefore, noninvasive alternatives to ICP (ICPni) measurement are of great interest. The main objective of this study was to compare the correlation and agreement of wave morphology between ICP (standard intraventricular ICP monitoring) and a new ICPni monitor in patients admitted with stroke. The second objective was to estimate the discrimination of the noninvasive method to detect intracranial hypertension. METHODS: We prospectively collected data of adults admitted to an intensive care unit with subarachnoid hemorrhage, intracerebral hemorrhage, or ischemic stroke in whom an invasive ICP monitor was placed. Measurements were simultaneously collected from two parameters [time-to-peak (TTP) and the ratio regarding the second and first peak of the ICP wave (P2/P1 ratio)] of ICP and ICPni wave morphology monitors (Brain4care). Intracranial hypertension was defined as an invasively measured sustained ICP > 20 mm Hg for at least 5 min. RESULTS: We studied 18 patients (subarachnoid hemorrhage = 14; intracerebral hemorrhage = 3; ischemic stroke = 1) on 60 occasions with a median age of 52 ± 14.3 years. A total of 197,400 waves (2495 min) from both ICP (standard ICP monitoring) and the ICPni monitor were sliced into 1-min-long segments, and we determined TTP and the P2/P1 ratio from the mean pulse. The median invasively measured ICP was 13 (9.8-16.2) mm Hg, and intracranial hypertension was present on 18 occasions (30%). The correlation and agreement between invasive and noninvasive methods for wave morphology were strong for the P2/P1 ratio and moderate for TTP using categoric (κ agreement 88.1% and 71.3%, respectively) and continuous (intraclass correlation coefficient 0.831 and 0.584, respectively) measures. There was a moderate but significant correlation with the mean ICP value (P2/P1 ratio r = 0.427; TTP r = 0.353; p < 0.001 for all) between noninvasive and invasive techniques. The areas under the curve to estimate intracranial hypertension were 0.786 [95% confidence interval (CI) 0.72-0.93] for the P2/P1 ratio and 0.694 (95% CI 0.60-0.74) for TTP. CONCLUSIONS: The new ICPni wave morphology monitor showed a good agreement with the standard invasive method and an acceptable discriminatory power to detect intracranial hypertension. Clinical trial registration Trial registration: NCT05121155.

Accuracy of predictive scores of hemorrhagic transformation in patients with acute ischemic stroke.

BACKGROUND: Hemorrhagic transformation (HT) is a complication in ischemic strokes, regardless of use of reperfusion therapy (RT). There are many predictive scores for estimating the risk of HT. However, most of them include patients also treated with RT. Therefore, this may lead to a misinterpretation of the risk of HT in patients who did not undergo RT. OBJECTIVE: We aimed to review published predictive scores and analyze their accuracy in our dataset. METHODS: We analyzed the accuracy of seven scales. Our dataset was derived from a cohort of 1,565 consecutive patients from 2015 to 2017 who were admitted to a comprehensive stroke center. All patients were evaluated with follow-up neuroimaging within seven days. Comparison of area under the curve (AUC) was performed on each scale, to analyze differences between patients treated with recombinant tissue plasminogen activator (tPA) and those without this treatment. RESULTS: Our dataset provided enough data to assess seven scales, among which six were used among patients with and without tPA treatment. HAT (AUC 0.76), HTI (0.73) and SEDAN (0.70) were the most accurate scores for patients not treated with tPA. SPAN-100 (0.55) had the worst accuracy in both groups. Three of these scores had different cutoffs between study groups. CONCLUSIONS: The predictive scores had moderate to fair accuracy for predicting HT in patients treated with tPA. Three scales were more accurate for predicting HT in patients not treated with tPA. Through standardizing these characteristics and including more patients not treated with RT in a large multicenter series, accurate predictive scores may be created.

Characterization of Retinal Architecture in Spinocerebellar Ataxia Type 3 and Correlation with Disease Severity.

BACKGROUND: Neurodegeneration affects the brain and peripheral nervous system in spinocerebellar ataxia type 3 (SCA3). As the retina is also involved, studying the retinal architecture in a cohort of patients could reveal clinically relevant biomarkers. OBJECTIVE: The aim is to investigate retinal architecture in SCA3 to identify potential biomarkers. METHODS: We evaluated 38 patients with SCA3 and 25 healthy age-matched controls, who underwent visual acuity assessment, intraocular pressure measurement, and fundoscopy and macular and peripapillary spectral domain optical coherence tomography (SD-OCT). We measured the peripapillary retinal nerve fiber layer (pRNFL) thickness in each quadrant of the temporal-superior-nasal-inferior-temporal chart and the macular layer thicknesses in each sector of the inner circle of the Early Treatment Diabetic Retinopathy Study (IC-ETDRS) grid. Linear regression analysis was employed to test the associations between retinal parameters and age, disease duration, CAG repeats, and SARA (Scale of the Assessment and Rating of Ataxia) and ICARS (International Cooperative Ataxia Rating Scale) scores in SCA3. RESULTS: In all sectors, except for the temporal quadrant, pRNFL was significantly thinner in SCA3 patients than in controls. Average total macular, ganglion cell layer (GCL), and inner plexiform layer (IPL) thicknesses were significantly decreased in SCA3 patients in comparison to controls. The average total macular thickness and the average thicknesses of RNFL, GCL, and IPL negatively correlated with ICARS scores, whereas average GCL and IPL thicknesses negatively correlated with SARA scores. CONCLUSIONS: The retinal ganglion cells, their dendrites, and axons are selectively affected in SCA3 patients. The RNFL, GCL, and IPL thicknesses in SD-OCT correlate with the clinical phenotype and represent potential biomarkers for future clinical trials and natural history studies. © 2021 International Parkinson and Movement Disorder Society.

Oxfordshire Community Stroke Project Classification: A proposed automated algorithm.

INTRODUCTION: The Oxfordshire Community Stroke Project (OCSP) proposed a clinical classification for Stroke patients. This classification has proved helpful to predict the risk of neurological complications. However, the OCSP was initially based on findings on the neurological assesment, which can pose difficulties for classifying patients. We aimed to describe the development and the validation step of a computer-based algorithm based on the OCSP classification. MATERIALS AND METHODS: A flow-chart was created which was reviewed by five board-certified vascular neurologists from which a computer-based algorithm (COMPACT) was developed. Neurology residents from 12 centers were invited to participate in a randomized trial to assess the effect of using COMPACT. They answered a 20-item questionnaire for classifying the vignettes according to the OCSP classification. Each correct answer has been attributed to 1-point for calculating the final score. RESULTS: Six-two participants agreed to participate and answered the questionnaire. Thirty-two were randomly allocated to use our algorithm, and thirty were allocated to adopt a list of symptoms alone. The group who adopted our algorithm had a median score of correct answers of 16.5[14.5, 17]/20 versus 15[13, 16]/20 points, p = 0.014. The use of our algorithm was associated with the overall rate of correct scores (p = 0.03). DISCUSSION: Our algorithm seemed a useful tool for any postgraduate year Neurology resident. A computer-based algorithm may save time and improve the accuracy to classify these patients. CONCLUSION: An easy-to-use computer-based algorithm improved the accuracy of the OCSP classification, with the possible benefit of further improvement of the prediction of neurological complications and prognostication.

Clinical and Epidemiological Characterization of Neurological Consults: When a Neurological Evaluation Is Requested.

BACKGROUND AND OBJECTIVES: Medical consultation by a specialist physician consists of an evaluation to review diagnosis and management of patients with some neurological conditions referred from other specialty wards. This mode of care delivery has gained relevance in the field of neurology and adequate training on it is valuable, allowing neurologists to provide state-of-the-art management to patients with neurological manifestations. The present study aimed to characterize neurology consults and to discuss the roles of the neurologist within a hospital setting. METHODS: A prospective analysis of neurological consultations provided to inpatients of a university hospital in São Paulo, Brazil, was performed from September 2016 to September 2017. These patients were followed by the principal investigator, who was not involved in their care. RESULTS: We evaluated data from 117 female and 106 male inpatients with a mean age of 53.8 ± 2.4. The medical specialties that most frequently requested neurological consultations were Internal Medicine (17%), Cardiology (11.2%) and Pulmonology (9.4%). The main reasons for a neurology consultation request were seizures (15.6%); decreased level of consciousness (8.9%) and confusion (7.1%). The most frequent diagnosis in patients receiving a neurology consult were stroke (10.2%); hypoxic-ischemic encephalopathy (5.3%) and sepsis (2.2%). CONCLUSION: Our findings show the growing importance of the role of neurologists within hospital settings as many medical conditions present with neurological manifestations and the significance of the neurohospitalist model of care.

Retinal Architecture in Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS): Insights into Disease Pathogenesis and Biomarkers.

BACKGROUND: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) causes unique retinal abnormalities, which have not been systematically investigated. OBJECTIVE: To deeply phenotype the retina in ARSACS in order to better understand its pathogenesis and identify potential biomarkers. METHODS: We evaluated 29 patients with ARSACS, 66 with spinocerebellar ataxia (SCA), 38 with autosomal recessive cerebellar ataxia (ATX), 22 with hereditary spastic paraplegia (SPG), 21 cases of papilledema, and 20 healthy controls (total n = 196 subjects). Participants underwent visual acuity assessment, intraocular pressure measurement, fundoscopy, and macular and peripapillary optical coherence tomography (OCT). Macular layers thicknesses in ARSACS were compared with those of age-matched healthy controls. Ophthalmologists analyzed the scans for abnormal signs in the different patient groups. Linear regression analysis was conducted to look for associations between retinal changes and age, age at onset, disease duration, and Scale for the Assessment and Rating of Ataxia (SARA) scores in ARSACS. RESULTS: Only patients with ARSACS exhibited peripapillary retinal striations (82%) on fundoscopy, and their OCT scans revealed foveal hypoplasia (100%), sawtooth appearance (89%), papillomacular fold (86%), and macular microcysts (18%). Average peripapillary retinal nerve fiber layer (pRNFL) was thicker in ARSACS than in SCA, ATX, SPG, and controls; a cut-off of 121 µm was 100% accurate in diagnosing ARSACS. All macular layers were thicker in ARSACS when compared to healthy controls. RNFL thickness in the inferior sector of the macula positively correlated with SARA scores. CONCLUSIONS: Retinal abnormalities are highly specific for ARSACS, and suggest retinal hyperplasia due to abnormal retinal development. OCT may provide potential biomarkers for future clinical trials. © 2021 International Parkinson and Movement Disorder Society.

A Proposal for Classification of Retinal Degeneration in Spinocerebellar Ataxia Type 7.

The aim of this study is to propose a classification system for the spinocerebellar ataxia type 7 retinal degeneration (SCA7-RD). Twenty patients with molecularly confirmed SCA7 underwent slit lamp examination, fundus photography, and optical coherence tomography (Spectralis®). Scale for the Assessment and Rating of Ataxia (SARA) and International Cooperative Ataxia Rating Scale (ICARS) were applied, and age, sex, age at symptom onset, and number of CAG expansions were recorded. After analyzing the ophthalmological findings in each participant, a panel of retinal disease experts created a qualitative classification system for SCA7-RD comprising four stages. We assessed the correlations of retinal degeneration severity with SARA and ICARS scores, number of CAG repeats in ATXN7 allele, and age at symptom onset. We graded retinal degeneration as stage 1 in nine participants, as stage 2 in five, and as stage 3 in six. No differences in age and visual symptoms duration were found between groups. SARA and ICARS scores correlated with the severity of SCA7-RD on the classification system (p = 0.024 and p = 0.014, respectively). After adjusting for disease duration, retinal disease stage association with SARA and ICARS scores remained significant (ANCOVA, p < 0.05). The classification system for SCA7-RD was able to characterize different disease stages representing the landmarks in the cone-rod dystrophy natural history. Neurodegeneration appears to occur in parallel in the cerebellum and in the visual pathway. We conclude that retinal degeneration in SCA7 is a potential biomarker of the neurological phenotype severity.

Predicting hemorrhagic transformation in patients not submitted to reperfusion therapies.

BACKGROUND: Well studied in patients with ischemic stroke after reperfusion therapies (RT), hemorrhagic transformation (HT) is also common in patients not treated with RT and can lead to disability even in initially asymptomatic cases. The best predictors of HT in patients not treated with RT are not well established. Therefore, we aimed to identify predictors of HT in patients not submitted to RT and create a user-friendly predictive score (PROpHET). MATERIAL AND METHODS: Patients admitted to a Comprehensive Stroke Center from 2015 to 2017 were prospectively evaluated and randomly selected to the derivation cohort. A multivariable logistic regression modeling was built to produce a predictive grading score for HT. The external validation was assessed using datasets from 7 Comprehensive Stroke Centers using the area under the receiver operating characteristic curve (AUROC). RESULTS: In the derivation group, 448 patients were included in the final analysis. The validation group included 2,683 patients. The score derived from significant predictors of HT in the multivariate logistic regression analysis was male sex (1 point), ASPECTS ≤ 7 (2 points), presence of leukoaraiosis (1 point), hyperdense cerebral middle artery sign (1 point), glycemia at admission ≥180 mg/dL (1 point), cardioembolism (1 point) and lacunar syndrome (-3 points) as a protective factor. The grading score ranges from -3 to 7. A Score ≥3 had 78.2% sensitivity and 75% specificity, and AUROC of 0.82 for all cases of HT. In the validation cohort, our score had an AUROC of 0.83. CONCLUSIONS: The PROpHET is a simple, quick, cost-free, and easy-to-perform tool that allows risk stratification of HT in patients not submitted to RT. A cost-free computerized version of our score is available online with a user-friendly interface.

Predictors of hemorrhagic transformation after acute ischemic stroke based on the experts opinion / Preditores de transformação hemorrágica após acidente vascular cerebral isquêmico agudo a partir da opinião de especialistas

ABSTRACT Background: Hemorrhagic transformation (HT) is a common complication after ischemic stroke. It may be associated to poor outcomes. Some predictors of HT have been previously identified, but there remain controversies. Objective: To describe the risk factors for HT more frequently reported by a panel of experts surveyed for this project. Methods: We sent a standard questionnaire by e-mail to specialists in Vascular Neurology from 2014 to 2018. Forty-five specialists were contacted and 20 of them responded to the invitation. Predictors cited by three or more specialists were included in a table and ranked by the frequency in which they appeared. A review of the literature looking for published predictive scores of HT was performed, comparing to the answers received. Results: The 20 responding specialists cited 23 different risk factors for HT. The most frequent factors in the order of citation were the volume of ischemia, previous use of antithrombotic medication, neurological severity, age, hyperglycemia at presentation, hypertension on admission, and cardioembolism. Most variables were also found in previously published predictive scores, but they were reported by the authors with divergences of frequency. Conclusion: Although many studies have evaluated HT in patients with acute ischemic stroke, the published risk factors were neither uniform nor in agreement with those cited by the stroke specialists. These findings may be helpful to build a score that can be tested with the goal of improving the prediction of HT.

RESUMO Introdução: A transformação hemorrágica (TH) é uma complicação comum após a isquemia cerebral e pode estar associada a desfechos desfavoráveis. Alguns fatores de risco para TH têm sido identificados, mas ainda há controvérsias. Objetivo: Descrever os fatores de risco para TH mais frequentemente reportados por um painel de especialistas consultados para esse projeto. Métodos: Enviamos um questionário padronizado por e-mail para 45 especialistas em Neurologia Vascular no período de 2014 a 2018. Vinte dos 45 especialistas responderam ao convite. Preditores citados por três ou mais especialistas foram incluídos em uma tabela e classificados pela frequência em que foram reportados. Uma revisão de literatura foi realizada em busca de escores preditivos de TH publicados anteriormente, comparando-os com as respostas recebidas. Resultados: Os 20 especialistas citaram 23 diferentes fatores de risco para TH. Os fatores mais frequentemente citados foram, pela ordem, volume da isquemia, uso prévio de medicação antitrombótica, gravidade neurológica, idade, hiperglicemia na apresentação, hipertensão na admissão e cardioembolismo. A maioria das variáveis também foi incluída em escores preditivos de TH já publicados, mas sem a mesma frequência e com divergências entre os especialistas consultados. Conclusão: Embora muitos estudos tenham avaliado a TH em pacientes com isquemia cerebral, os fatores de risco já publicados não foram uniformes na concordância com aqueles reportados pelos neurologistas vasculares consultados. Esses achados podem ser úteis para elaborar um escore que possa ser testado para aperfeiçoar a predição de transformação hemorrágica.

The Role of Hemorrhagic Transformation in Acute Ischemic Stroke Upon Clinical Complications and Outcomes.

BACKGROUND AND PURPOSE: Hemorrhagic transformation (HT) is a common neurological complication after ischemic stroke. The influence of symptomatic HT upon clinical outcomes post-stroke is well established, however, the role of asymptomatic HT upon prognosis is still unclear. We aimed to analyze the relationship between HT, clinical complications and outcomes in patients not submitted to reperfusion therapies (RT). METHODS: We included 448 randomly selected patients admitted with acute ischemic stroke to a tertiary hospital stroke unit from 2015 to 2017. Patients submitted to RT were excluded. All patients were evaluated with computed tomography (CT) at admission and within 7 days from the initial CT. Patients with HT were divided into two groups: symptomatic and asymptomatic cases based on the ECASS II definition. A good clinical outcome was defined as a modified Rankin Scale (mRS) 0-2 at discharge. RESULTS: A total of 95 patients (21.2%) had HT (51 asymptomatic and 44 symptomatic). Age, NIHSS at admission and symptomatic HT were associated with a higher risk of developing pneumonia and seizures during hospitalization. Symptomatic HT was also associated with a prolonged length of hospitalization and death and inversely associated with good clinical outcomes at discharge (OR 0.96, 95% CI 0.94-0.98, p<0.001). In an adjusted analysis, even asymptomatic HT was independently associated with worse clinical outcomes at discharge (mRS 4-6) (OR 5.99, 95% CI 1.83-19.58, p = 0.003). CONCLUSIONS: Symptomatic HT is associated with a higher risk of clinical complications, prolonged hospitalization, death and worse clinical outcome at discharge. Furthermore, even patients with asymptomatic HT had a higher chance of worse clinical outcomes at discharge.