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AIM: To review the current data on cognitive and psychological characteristics of patients with CAA and on the instruments used for their evaluation. METHODS: A systematic search was performed in Embase, Scopus and PubMed with terms related to "cerebral amyloid angiopathy", "neuropsychological measures" and "patient-reported outcome measures" from January 2001 to December 2021. RESULTS: Out of 2851 records, 18 articles were selected. The cognitive evaluation was present in all of which, while the psychological one only in five articles. The MMSE (Mini Mental State Examination), TMT (Trail Making Test), fluency test, verbal learning test, digit span, digit symbol and Rey figure tests were the most used cognitive tests, while executive function, memory, processing speed, visuospatial function, attention and language were the most frequent impaired cognitive functions. Depression was the most considered psychological factor usually measured with BDI (Beck Depression Inventory) and GDS (Geriatric Depression Scale). CONCLUSIONS: The results of this study might be used in clinical practice as a guide to choose cognitive and psychological instruments and integrate them in the clinical evaluation. The results might also be used in the research field for studies investigating the impact of cognitive and psychological variables on the disease course and for consensus studies aimed at define a standardized evaluation of these aspects.
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Angiopatía Amiloide Cerebral , Humanos , Angiopatía Amiloide Cerebral/psicología , Angiopatía Amiloide Cerebral/complicaciones , Disfunción Cognitiva/etiología , Disfunción Cognitiva/diagnóstico , Depresión/etiología , Depresión/psicología , Depresión/diagnóstico , Pruebas NeuropsicológicasRESUMEN
Despite the growing interest in gender medicine, the influence of sex and gender on human diseases, including stroke, continues to be underestimated and understudied. The COVID-19 pandemic has overall impacted not only the occurrence and management of stroke but has also exacerbated sex and gender disparities among both patients and healthcare providers. This paper aims to provide an updated overview on the influence of sex and gender in stroke pathophysiology and care during COVID-19 pandemic, through biological, clinical, psychosocial and research perspectives. Gender equity and awareness of the importance of sexual differences are sorely needed, especially in times of health crisis but have not yet been achieved to date. To this purpose, the sudden yet worldwide diffusion of COVID-19 represents a unique learning experience that highlights critical unmet needs also in gender medicine. The failures of this recent past should be kept as food for thought to inspire proper strategies reducing inequalities and to address women's health and wellbeing issues, particularly in case of future pandemics.
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BACKGROUND AND PURPOSE: Iatrogenic cerebral amyloid angiopathy (iCAA) is a specific type of cerebral amyloid angiopathy which is becoming increasingly diagnosed. It has been hypothesized that iCAA might arise as a late consequence of past neurosurgical interventions involving dural patch grafts. Positron emission tomography (PET) scans with amyloid tracers and the assay of beta-amyloid levels in cerebrospinal fluid (CSF) are auxiliary criteria, however, definite diagnosis remains histopathologically determined. METHODS: Case report. RESULTS: We present a 48-year-old patient who suffered multiple lobar cerebral haemorrhages from the age of 47. The patient had undergone surgery for remolval of hemangioblastoma with lyophilized dural graft at the age of 11, in 1987. Brain MRI, amiloid PET and CSF analysis led to a diagnosis of probable iCAA. CONCLUSION: It is necessary to increase the awareness of iCAA, in order to avoid overlooking the potential causal involvement of surgical procedures which took place far back in time. Moreover, the diagnostic relevance of amyloid PET and beta-amyloid levels in CSF must be emphasised.
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Angiopatía Amiloide Cerebral , Humanos , Persona de Mediana Edad , Angiopatía Amiloide Cerebral/complicaciones , Angiopatía Amiloide Cerebral/diagnóstico por imagen , Péptidos beta-Amiloides/líquido cefalorraquídeo , Hemorragia Cerebral , Imagen por Resonancia Magnética , Enfermedad IatrogénicaRESUMEN
OBJECTIVES: To investigate the relationship between N20-P25 peak-to-peak amplitude (N20p-P25p) of somatosensory evoked potentials (SEPs) and the occurrence of abnormalities of the peripheral and/or central sensory pathways and of myoclonus/epilepsy, in 308 patients with increased SEPs amplitude from upper limb stimulation. METHODS: We compared cortical response (N20p-P25p) in different groups of patients identified by demographic, clinical, and neurophysiological factors and performed a cluster analysis for classifying the natural occurrence of subgroups of patients. RESULTS: No significant differences of N20p-P25p were found among different age-dependent groups, and in patients with or without PNS/CNS abnormalities of sensory pathways, while myoclonic/epileptic patients showed higher N20p-P25p than other groups. Cluster analysis identified four clusters of patients including myoclonus/epilepsy, central sensory abnormalities, peripheral sensory abnormalities, and absence of myoclonus and sensory abnormalities. CONCLUSIONS: Increased N20p-P25p prompts different possible pathophysiological substrates: larger N20p-P25p in patients with cortical myoclonus and/or epilepsy is likely sustained by strong cortical hyperexcitability, while milder increase of N20p-P25p could be underpinned by plastic cortical changes following abnormalities of sensory pathways, or degenerative process involving the cortex. SEPs increased in amplitude cannot be considered an exclusive hallmark of myoclonus/epilepsy. Indeed, in several neurological disorders, it may represent a sign of adaptive, plastic, and/or degenerative cortical changes.
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Epilepsias Mioclónicas , Epilepsia , Mioclonía , Electroencefalografía , Potenciales Evocados Somatosensoriales/fisiología , Humanos , Nervio Mediano , Corteza Somatosensorial/fisiologíaRESUMEN
Ring Finger Protein 213 (RNF213), also known as Mysterin, is the major susceptibility factor for Moyamoya Arteriopathy (MA), a progressive cerebrovascular disorder that often leads to brain stroke in adults and children. Although several rare RNF213 polymorphisms have been reported, no major susceptibility variant has been identified to date in Caucasian patients, thus frustrating the attempts to identify putative therapeutic targets for MA treatment. For these reasons, the investigation of novel biochemical functions, substrates and unknown partners of RNF213 will help to unravel the pathogenic mechanisms of MA and will facilitate variant interpretations in a diagnostic context in the future. The aim of the present review is to discuss novel perspectives regarding emerging RNF213 roles in light of recent literature updates and dissect their relevance for understanding MA and for the design of future research studies. Since its identification, RNF213 involvement in angiogenesis and vasculogenesis has strengthened, together with its role in inflammatory signals and proliferation pathways. Most recent studies have been increasingly focused on its relevance in antimicrobial activity and lipid metabolism, highlighting new intriguing perspectives. The last area could suggest the main role of RNF213 in the proteasome pathway, thus reinforcing the hypotheses already previously formulated that depict the protein as an important regulator of the stability of client proteins involved in angiogenesis. We believe that the novel evidence reviewed here may contribute to untangling the complex and still obscure pathogenesis of MA that is reflected in the lack of therapies able to slow down or halt disease progression and severity.
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Adenosina Trifosfatasas , Enfermedad de Moyamoya , Adenosina Trifosfatasas/metabolismo , Adulto , Niño , Predisposición Genética a la Enfermedad , Humanos , Enfermedad de Moyamoya/patología , Factores de Transcripción , Ubiquitina-Proteína Ligasas/genética , Ubiquitina-Proteína Ligasas/metabolismoRESUMEN
[Figure: see text].
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Antitrombinas/uso terapéutico , Fibrilación Atrial/complicaciones , Hemorragia Cerebral/inducido químicamente , Accidente Cerebrovascular/prevención & control , Administración Oral , Anciano , Anciano de 80 o más Años , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Factores de Riesgo , Accidente Cerebrovascular/etiologíaRESUMEN
BACKGROUND: Transient global amnesia (TGA) is a clinical syndrome characterized by a temporary short-term memory loss with inability to retain new memories, usually lasting 2 to 8 h. TGA may be related to several medical procedures, including angiography, general anesthesia, gastroscopy. CASE PRESENTATION: We report a 58-year-old woman who experiencing TGA one hour after the execution of her first-time nasopharyngeal swab for COVID-19. Brain MRI showed a typical punctate Diffusion Weight Image (DWI) hippocampal lesion. CONCLUSIONS: This is the first report of TGA after the execution of nasopharyngeal swab for COVID-19. This association lengthen the list of medical procedures associated with TGA, and we discuss the possible plausible mechanisms by which a nasopharyngeal swab could trigger TGA.
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Amnesia Global Transitoria , COVID-19/diagnóstico , Manejo de Especímenes/efectos adversos , Amnesia Global Transitoria/diagnóstico , Amnesia Global Transitoria/etiología , Prueba de COVID-19 , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Nasofaringe/virologíaRESUMEN
Moyamoya arteriopathy (MA) is a rare cerebrovascular disorder characterized by ischemic/hemorrhagic strokes. The pathophysiology is unknown. A deregulation of vasculogenic/angiogenic/inflammatory pathways has been hypothesized as a possible pathophysiological mechanism. Since lipids are implicated in modulating neo-vascularization/angiogenesis and inflammation, their deregulation is potentially involved in MA. Our aim is to evaluate angiogenic/vasculogenic/inflammatory proteins and lipid profile in plasma of MA patients and control subjects (healthy donors HD or subjects with atherosclerotic cerebrovascular disease ACVD). Angiogenic and inflammatory protein levels were measured by ELISA and a complete lipidomic analysis was performed on plasma by mass spectrometry. ELISA showed a significant decrease for MMP-9 released in plasma of MA. The untargeted lipidomic analysis showed a cumulative depletion of lipid asset in plasma of MA as compared to HD. Specifically, a decrease in membrane complex glycosphingolipids peripherally circulating in MA plasma with respect to HD was observed, likely suggestive of cerebral cellular recruitment. The quantitative targeted approach demonstrated an increase in free sphingoid bases, likely associated with a deregulated angiogenesis. Our findings indicate that lipid signature could play a central role in MA and that a detailed biomarker profile may contribute to untangle the complex, and still obscure, pathogenesis of MA.
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Lípidos/sangre , Enfermedad de Moyamoya/sangre , Enfermedades Vasculares/sangre , Biomarcadores/sangre , Femenino , Humanos , Inflamación/sangre , Arteriosclerosis Intracraneal/sangre , Lipidómica/métodos , Masculino , Persona de Mediana Edad , Neovascularización Patológica/sangreRESUMEN
BACKGROUND: It has been widely reported that anticoagulants (ACs) are underused for primary and secondary prevention of ischemic stroke in patients with atrial fibrillation (AFib). Furthermore, precise evidence-based guidelines about the best timing for AC initiation after acute stroke are currently lacking. METHODS AND RESULTS: In this retrospective, observational study, we analyzed prescription trends in AFib patients with acute ischemic stroke who were hospitalized in four neurologic stroke units of our region (Lombardia, Italy). In-hospital antithrombotic prescription was performed in highly heterogeneous patterns. A prestroke treatment with AC was the leading factor enhancing AC prescription during hospitalization. The other factors promoting AC were male gender, younger age, lower prestroke disability and stroke severity, and smaller stroke volumes. AFib subtype influenced AC prescription only in AC-naïve patients. Interestingly, Congestive heart failure, Hypertension, Age higher than 75 years, Diabetes, previous Stroke or TIA or thromboembolism, Vascular disease, Age 64-75 years, female Sex (CHA2DS2-VASc) and Hypertension, Abnormal renal and liver function, Stroke, Bleeding, Labile INRs, Elderly, Drugs and alcohol (HAS-BLED) scores were not associated with AC prescription. However, patients who were treated with AC, including early treatment (<48 hours), showed a low rate of bleeding. CONCLUSIONS: Our findings potentially suggest that, although apparently neglecting the common risk stratification tools, our neurologists were able to select the more suitable candidates for prompt AC treatment. Further studies are needed to develop new scoring systems to aid ischemic and hemorrhagic risk estimation in the secondary prevention of stroke.
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Anticoagulantes/uso terapéutico , Fibrilación Atrial/complicaciones , Fibrilación Atrial/tratamiento farmacológico , Seguridad del Paciente , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/tratamiento farmacológico , Factores de Edad , Anciano , Anciano de 80 o más Años , Fibrilación Atrial/diagnóstico por imagen , Prescripciones de Medicamentos/estadística & datos numéricos , Femenino , Insuficiencia Cardíaca/complicaciones , Hemorragia/etiología , Hospitalización/estadística & datos numéricos , Humanos , Hipertensión/complicaciones , Italia , Modelos Logísticos , Masculino , Persona de Mediana Edad , Neuroimagen , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Accidente Cerebrovascular/diagnóstico por imagenRESUMEN
BACKGROUND: Statins, due to their well-established pleiotropic effects, have noteworthy benefits in stroke prevention. Despite this, a significant proportion of high-risk patients still do not receive the recommended therapeutic regimens, and many others discontinue treatment after being started on them. The causes of non-adherence to current guidelines are multifactorial, and depend on both physicians and patients. The aim of this study is to identify the factors influencing statin prescription at Stroke Unit (SU) discharge. METHODS: This study included 12,750 patients enrolled on the web-based Lombardia Stroke Registry (LRS) from July 2009 to April 2012 and discharged alive, with a diagnosis of ischemic stroke or transient ischemic attack (TIA) and without contra-indication to statin therapy. By logistic regression analysis and classification trees, we evaluated the impact of demographic data, risk factors, tPA treatment, in-hospital procedures and complications on statin prescription rate at discharge. RESULTS: We observed a slight increase in statins prescription during the study period (from 39.1 to 43.9%). Lower age, lower stroke severity and prestroke disability, the presence of atherothrombotic/lacunar risk factors, a diagnosis of non-cardioembolic stroke, tPA treatment, the absence of in-hospital complications, with the sole exception of hypertensive fits and hyperglycemia, were the patient-related predictors of adherence to guidelines by physicians. Overall, dyslipidemia appears as the leading factor, while TOAST classification does not reach statistical significance. CONCLUSIONS: In our region, Lombardia, adherence to guidelines in statin prescription at Stroke Unit discharge is very different from international goals. The presence of dyslipidemia remains the main factor influencing statin prescription, while the presence of well-defined atherosclerotic etiopathogenesis of stroke does not enhance statin prescription. Some uncertainties about the risk/benefit of statin therapy in stroke etiology subtypes (cardioembolism, other or undetermined causes) may partially justify the underuse of statin in ischemic stroke. The differences that exist between current international guidelines may prevent a more widespread use of statin and should be clarified in a consensus.
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Isquemia Encefálica/tratamiento farmacológico , Isquemia Encefálica/epidemiología , Inhibidores de Hidroximetilglutaril-CoA Reductasas/uso terapéutico , Sistema de Registros , Accidente Cerebrovascular/tratamiento farmacológico , Accidente Cerebrovascular/epidemiología , Anciano , Anciano de 80 o más Años , Isquemia Encefálica/diagnóstico , Femenino , Estudios de Seguimiento , Humanos , Italia/epidemiología , Masculino , Factores de Riesgo , Accidente Cerebrovascular/diagnósticoRESUMEN
OBJECTIVE: Moyamoya angiopathy (MA) is a rare cerebrovascular disorder characterized by recurrent ischemic/hemorrhagic strokes due to progressive occlusion of the intracranial carotid arteries. The lack of reliable disease severity biomarkers led us to investigate molecular features of a Caucasian cohort of MA patients. METHODS: The participants consisted of 30 MA patients and 40 controls. We measured cerebrospinal fluid (CSF) levels of angiogenic/inflammatory factors (ELISA). We then applied quantitative real-time PCR on cerebral artery specimens for expression analyses of angiogenic factors. By an immunoassay based on microfluidic technology, we examined the potential correlations between plasma protein expression and MA clinical progression. A RNA interference approach toward Ring Finger Protein 213 (RNF213) and a tube formation assay were applied in cellular model. RESULTS: We detected a statistically significant (p < 0.000001) up-regulation of Angiopoietin-2 (Ang-2) in CSF and stenotic middle cerebral arteries (RQ >2) of MA patients compared to controls. A high Ang-2 plasma concentration (p = 0.018) was associated with unfavorable outcome in a subset of MA patients. ROC curve analyses indicated Ang-2 as diagnostic CSF biomarker (>3741 pg/mL) and prognostic plasma biomarker (>1162 pg/mL), to distinguish stable-from-progressive MA. Consistently, MA cellular model showed a significant up-regulation (RQ >2) of Ang-2 in RNF213 silenced condition. INTERPRETATION: Our results pointed out Ang-2 as a reliable biomarker mirroring arterial steno-occlusion and vascular instability of MA in CSF and blood, providing a candidate factor for patient stratification. This pilot study may pave the way to the validation of a biomarker to identify progressive MA patients deserving a specific treatment path.
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Angiopoyetina 2 , Enfermedad de Moyamoya , Humanos , Enfermedad de Moyamoya/genética , Enfermedad de Moyamoya/líquido cefalorraquídeo , Enfermedad de Moyamoya/diagnóstico , Angiopoyetina 2/líquido cefalorraquídeo , Angiopoyetina 2/genética , Angiopoyetina 2/sangre , Masculino , Femenino , Adulto , Persona de Mediana Edad , Pronóstico , Biomarcadores/líquido cefalorraquídeo , Biomarcadores/sangre , Ubiquitina-Proteína Ligasas/genética , Adulto Joven , Adenosina TrifosfatasasRESUMEN
OBJECTIVES: Cerebral amyloid angiopathy (CAA)-related features on neuroimaging often coexist with signs of arteriolosclerosis-small vessel disease on neuroimaging in people with intracranial hemorrhage (ICH). This study aimed at defining the value of amyloid pathology detected by 18Fflutemetamol PET in reclassification and stratification of risk of bleeding in people with mixed CAA-arteriolosclerosis features. METHODS: We included consecutive patients admitted to 2 institutions (2018-2023) with spontaneous symptomatic ICH, subarachnoid hemorrhage (SAH), transient focal neurologic episodes (TFNE), or cognitive impairment and MRI showing CAA hallmarks. All patients underwent brain magnetic resonance imaging (MRI) with susceptibility weighted imaging and 18Fflutemetamol PET imaging and were followed up for at least 1 year. We compared cases with CAA and arteriolosclerosis + CAA features and defined long-term outcomes (composite outcome including death, ICH, ischemic stroke, SAH, TFNE) depending on PET status (CAA/amyloid pathology vs arteriolosclerosis-predominant groups). RESULTS: Among 47 patients, according to PET and MRI imaging, 38 patients were reclassified in the CAA/amyloid pathology group and 9 in the arteriolosclerosis-predominant group, with similar cardiovascular risk factors but a significantly higher lobar microbleed burden for the former group. The CAA/amyloid pathology group had higher rates of composite outcome (43.9 vs 11.1 events per 100 patient-year; p = 0.039) and ICH (36.5 vs 5.6 events per 100 patient-years; p = 0.04) compared with the arteriolosclerosis-predominant group. DISCUSSION: 18FFlutemetamol PET imaging can help in reclassification of mixed arteriolosclerosis + CAA into CAA/amyloid pathology and arteriolosclerosis-predominant, with implications on long-term risk of recurrent events. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that 18Fflutemetamol PET can distinguish between CAA + arteriolosclerosis and arteriolosclerosis-predominant pathology.
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Compuestos de Anilina , Angiopatía Amiloide Cerebral , Imagen por Resonancia Magnética , Tomografía de Emisión de Positrones , Humanos , Angiopatía Amiloide Cerebral/diagnóstico por imagen , Angiopatía Amiloide Cerebral/patología , Angiopatía Amiloide Cerebral/clasificación , Angiopatía Amiloide Cerebral/complicaciones , Masculino , Femenino , Anciano , Tomografía de Emisión de Positrones/métodos , Persona de Mediana Edad , Benzotiazoles , Anciano de 80 o más Años , Hemorragias Intracraneales/diagnóstico por imagenRESUMEN
BACKGROUND: Symptomatic cluster headache (CH) secondary to internal carotid artery dissection (ICAD) has been frequently reported, however, as far as we know, the coexistence of episodic CH and acute symptomatic CH secondary to ICAD has not. CASE REPORT: A 39 year-old man, affected by episodic CH since the age of 19, presented an atypical headache associated with his usual autonomic symptoms. After a series of negative tests, MRA eventually revealed dissection of the right distal internal carotid artery. DISCUSSION AND CONCLUSIONS: The coexistence of episodic CH and acute CH symptomatic of ICAD in our patient suggests that, at least in some cases, CH and ICAD may be different expressions of a common underlying cause: hidden vessel wall damage. When risk factors and the change - though partial - of clinical features suggest symptomatic cases, CH patients have to be strictly monitored over time. Given the lack of a gold standard investigation for dynamic diseases such as dissections, these patients require multimodal diagnostic investigation over time, even in cases where exams are normal at onset.
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Disección Aórtica/complicaciones , Enfermedades de las Arterias Carótidas/complicaciones , Cefalalgia Histamínica/complicaciones , Adulto , Disección Aórtica/diagnóstico por imagen , Enfermedades de las Arterias Carótidas/diagnóstico por imagen , Enfermedades de las Arterias Carótidas/patología , Arteria Carótida Interna/diagnóstico por imagen , Cefalalgia Histamínica/diagnóstico por imagen , Humanos , Angiografía por Resonancia Magnética , Masculino , RadiografíaRESUMEN
Well-being is a relevant outcome after stroke, potentially impacted by mental health difficulties. We addressed the psychological and cognitive predictors of psychological well-being in a sample of 122 stroke survivors (75 males, 97 with ischemic stroke; mean age 64.1, mean NIHSS 2.9, mean distance from the acute event 5.1 years) admitted to the 'Carlo Besta' Neurological Institute. Trait anxiety (ß = -0.257), state anxiety (ß = -0.208) and symptoms of depression (ß = -0.484) significantly predicted well-being variation (Adj. R2â =â 0.687). These potentially modifiable factors are promising targets for interventions to reduce the burden of illness and enhance the recovery process.
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Bienestar Psicológico , Accidente Cerebrovascular , Humanos , Masculino , Persona de Mediana Edad , Cognición , Estudios Transversales , Depresión/psicología , Accidente Cerebrovascular/psicología , Sobrevivientes , FemeninoRESUMEN
Thanks to a more widespread knowledge of the disease, and improved diagnostic techniques, the clinical spectrum of cerebral amyloid angiopathy (CAA) is now broad. Sporadic CAA, hereditary CAA, CAA-related inflammation (CAA-ri) and iatrogenic CAA (iCAA) create a clinical and radiological continuum which is intriguing and only partially discovered. Despite being relatively rare, CAA-ri, an aggressive subtype of CAA with vascular inflammation, has gained growing attention also because of the therapeutic efficacy of anti-inflammatory and immunomodulating drugs. More recently, diagnostic criteria have been proposed for an unusual variant of CAA, probably related to an iatrogenic origin (iCAA), toward which there is mounting scientific interest. These atypical forms of CAA are still poorly known, and their recognition can be challenging and deserve to be pursued in specialized referral centres. The aim of this brief review is to focus current developments in the field of rare forms of CAA, its pathogenesis as well as clinical and biological features in order to increase awareness of these rare forms.
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Stroke causes a significant reduction in health-related quality of life (HRQoL), and studies addressing its predictors often rely on models with few variables. This study aimed to assess the degree to which health status, health habits, and features of the environment predict HRQoL in stroke survivors with stable clinical condition. WHO Quality of Life questionnaire for old-Age subjects (WHOQOL-AGE) was used to assess HRQoL. We ran a multivariable linear regression to predict WHOQOL-AGE variation, entering measures of health state, bad habits, healthy behaviors, physical environment features, and social support. Patients were stroke survivors with a stable clinical condition, distance from acute event of more than 6 months, and National Institutes of Health Stroke Scale (NIHSS) of 10 or less. A total of 122 participants (47 females, 97 with ischemic stroke) were enrolled, the mean age was 64.1, mean NIHSS 2.9, and mean distance from the acute event was 5.1 years. State anxiety (ß = -0.202), trait anxiety (ß = -0.232), depression (ß = -0.255), social support (ß = 0.247), and functional independence (ß = -0.210) predicted WHOQOL-AGE variation (Adj. R2 = 0.549). Our results show that psychological symptoms, reduced social network, and functional dependence together have a negative impact on HRQoL. These elements, which are partly stroke-specific, should be taken into account in the recovery process to enhance patients' health outcomes.
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Rehabilitación de Accidente Cerebrovascular , Accidente Cerebrovascular , Femenino , Humanos , Persona de Mediana Edad , Calidad de Vida/psicología , Estudios Transversales , Depresión/psicología , Accidente Cerebrovascular/psicologíaRESUMEN
The European Stroke Organisation (ESO) guidelines on Moyamoya Angiopathy (MMA), developed according to ESO standard operating procedure and Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology, were compiled to assist clinicians in managing patients with MMA in their decision making. A working group involving neurologists, neurosurgeons, a geneticist and methodologists identified nine relevant clinical questions, performed systematic literature reviews and, whenever possible, meta-analyses. Quality assessment of the available evidence was made with specific recommendations. In the absence of sufficient evidence to provide recommendations, Expert Consensus Statements were formulated. Based on low quality evidence from one RCT, we recommend direct bypass surgery in adult patients with haemorrhagic presentation. For ischaemic adult patients and children, we suggest revascularization surgery using direct or combined technique rather than indirect, in the presence of haemodynamic impairment and with an interval of 6-12 weeks between the last cerebrovascular event and surgery. In the absence of robust trial, an Expert Consensus was reached recommending long-term antiplatelet therapy in non-haemorrhagic MMA, as it may reduce risk of embolic stroke. We also agreed on the utility of performing pre- and post- operative haemodynamic and posterior cerebral artery assessment. There were insufficient data to recommend systematic variant screening of RNF213 p.R4810K. Additionally, we suggest that long-term MMA neuroimaging follow up may guide therapeutic decision making by assessing the disease progression. We believe that this guideline, which is the first comprehensive European guideline on MMA management using GRADE methods will assist clinicians to choose the most effective management strategy for MMA.
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Accidente Cerebrovascular Embólico , Enfermedad de Moyamoya , Accidente Cerebrovascular , Adulto , Niño , Humanos , Accidente Cerebrovascular/diagnóstico , Enfermedad de Moyamoya/diagnóstico , Progresión de la Enfermedad , Adenosina Trifosfatasas , Ubiquitina-Proteína LigasasRESUMEN
BACKGROUND: Intracerebral hemorrhage and cognitive decline are typical clinical presentations of cerebral amyloid angiopathy (CAA). OBJECTIVE: To determine whether magnetic resonance imaging (MRI) features differ between CAA with hemorrhagic versus cognitive onset. METHODS: In this retrospective study, sixty-one patients with CAA were classified by onset presentation of the disease: hemorrhage (n = 31) or cognitive decline (n = 30). The two groups were compared for MRI markers of small vessel disease, namely cerebral microbleeds (CMBs), cortical superficial siderosis, white matter hyperintensities (WMHs), enlarged perivascular spaces, cortical microinfarcts, and visual rating scales for cortical atrophy. In the patients with cognitive onset, further exploratory analyses investigated MRI markers according to cerebrospinal fluid (CSF) and neuropsychological profiles. RESULTS: Patients with cognitive onset showed a higher prevalence of CMBs (p < 0.001), particularly in temporal (p = 0.015) and insular (p = 0.002) lobes, and a higher prevalence of WMHs (p = 0.012). Within the cognitive onset group, 12 out of 16 (75%) patients had an Alzheimer's disease (AD) CSF profile but did not differ in MRI markers from those without AD pathology. Patients with cognitive onset displayed a multidomain profile in 16 out of 23 (70%) cases; patients with this profile showed increased WMHs and CMBs in parietal lobes compared with the amnestic group (p = 0.002) and dysexecutive group (p = 0.032), respectively. CONCLUSION: Higher burdens of WMHs and CMBs, especially in temporal and insular lobes, are associated with the cognitive onset of CAA. MRI markers could help to shed light on the clinical heterogeneity of the CAA spectrum and its underlying mechanisms.
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Enfermedad de Alzheimer , Angiopatía Amiloide Cerebral , Humanos , Estudios Retrospectivos , Angiopatía Amiloide Cerebral/complicaciones , Angiopatía Amiloide Cerebral/diagnóstico por imagen , Hemorragia Cerebral/etiología , Hemorragia Cerebral/complicaciones , Neuroimagen , Imagen por Resonancia Magnética/métodos , Enfermedad de Alzheimer/complicaciones , Enfermedad de Alzheimer/diagnóstico por imagen , CogniciónRESUMEN
INTRODUCTION: The best therapeutic strategy for patients with mechanical heart valves (MHVs) having acute ischemic stroke during treatment with vitamin K antagonists (VKAs) remain unclear. Being so, we compared the outcomes for: (i) full dose heparin along with VKA (bridging therapy group) and (ii) restarting VKA without heparin (nonbridging group). PATIENTS AND METHODS: For this multicenter observational cohort study, data on consecutive acute ischemic stroke patients with MHV was retrospectively collected from prospective registries. Propensity score matching (PSM) was adopted to adjust for any treatment allocation confounders. The primary outcome was the composite of stroke, systemic embolism, symptomatic cerebral bleeding, and major extracerebral bleeding at 90 days. RESULTS: Overall, 255 out of 603 patients (41.3%) received bridging therapy: 36 (14.1%) had combined outcome, compared with 28 (8.0%) in the nonbridging group (adjusted OR 1.83; 95% CI 1.05-3.18; p = 0.03). Within the bridging group, 13 patients (5.1%) compared to 12 (3.4%) in the nonbridging group had an ischemic outcome (adjusted OR 1.71; 95% CI 0.84-3.47; p = 0.2); major bleedings were recorded in 23 (9.0%) in the bridging group and 16 (4.6%) in the nonbridging group (adjusted OR 1.88; 95% CI 0.95-3.73; p = 0.07). After PSM, 36 (14.2%) of the 254 bridging patients had combined outcome, compared with 23 (9.1%) of 254 patients in the nonbridging group (OR 1.66; 95% CI 0.95-2.85; p = 0.07). CONCLUSION: Acute ischemic stroke patients with MHV undergoing bridging therapy had a marginally higher risk of ischemic or hemorrhagic events, compared to nonbridging patients.
Asunto(s)
Fibrilación Atrial , Accidente Cerebrovascular Isquémico , Humanos , Heparina/efectos adversos , Accidente Cerebrovascular Isquémico/tratamiento farmacológico , Estudios Retrospectivos , Estudios Prospectivos , Fibrilación Atrial/inducido químicamente , Anticoagulantes/efectos adversos , Hemorragia/inducido químicamente , Válvulas CardíacasRESUMEN
BACKGROUND: The syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid (CSF) Lymphocytosis (HaNDL) is classified among secondary headaches attributed to "non-infectious, inflammatory intracranial disease". Despite its classification among secondary headaches, the current definition of HaNDL does not contemplate a causal agent. Thus, the aetiology, as well as the pathogenesis of both the headache and the transient focal deficits, remains unknown. CASE PRESENTATION: We describe a 29-year-old healthy male developing episodes of thunderclap headaches associated with recurrence of hemiparesis/hemi-paraesthesia; CSF showed lymphocytosis 200/mm3 and increased albumin; brain MRI revealed widespread leptomeningeal enhancement and a non-enhancing, circular diffusion restriction in the splenium of corpus callosum. Screening for neurotropic pathogens detected Epstein-Barr (EBV) DNA in serum and CSF, interpreted as a primary EBV infection once the seroconversion of EBV nuclear antigen (EBNA) IgM to IgG was proven on follow-up. Transcranial Doppler detected, during headache, increased flow velocity in middle cerebral arteries, possibly indicating vasospasm. Oral nimodipine was administered, with prompt clinical recovery, resolution of CSF/MRI abnormalities, and normalization of flow velocities in middle cerebral arteries. CASE-BASED REVIEW: Although the definition of HaNDL does not contemplate a viral trigger or abnormal brain imaging, we found other literature cases of HaNDL associated with direct or indirect signs of CNS infection. CONCLUSIONS: At least in a proportion of patients, a viral aetiology may have a role in HaNDL. Whatever the aetiology, we suggest that the pathogenic mechanism may rely on the (viral or other) agent ultimately triggering cerebral vasoconstriction, which would explain both focal symptoms and headache. Calcium channel blockers might be a therapeutic option.