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1.
Neurology ; 103(2): e209548, 2024 Jul 23.
Artículo en Inglés | MEDLINE | ID: mdl-38900992

RESUMEN

BACKGROUND AND OBJECTIVES: Cerebral amyloid angiopathy-related inflammation (CAA-RI) and biopsy-positive primary angiitis of the CNS (BP-PACNS) have overlapping clinicoradiologic presentations. It is unknown whether clinical and radiologic features can differentiate CAA-RI from BP-PACNS and whether both diseases have different relapse rates. The objectives of this study were to compare clinicoradiologic presentations and relapse rates in patients with CAA-RI vs BP-PACNS. METHODS: Patients with CAA-RI and BP-PACNS were enrolled from 2 retrospective multicenter cohorts. Patients with CAA-RI were biopsy-positive or met probable clinicoradiologic criteria. Patients with BP-PACNS had histopathologic confirmation of CNS angiitis, with no secondary etiology. A neuroradiologist read brain MRIs, blinded to the diagnosis of CAA-RI or BP-PACNS. Clinicoradiologic features were compared using univariable logistic regression models. Relapse rates were compared using a univariable Fine-Gray subdistribution hazard model, with death as a competing risk. RESULTS: This study enrolled 104 patients with CAA-RI (mean age 73 years, 48% female sex) and 52 patients with BP-PACNS (mean age 45 years, 48% female sex). Patients with CAA-RI more often had white matter hyperintense lesions meeting the probable CAA-RI criteria (93% vs 51%, p < 0.001), acute subarachnoid hemorrhage (15% vs 2%, p = 0.02), cortical superficial siderosis (27% vs 4%, p < 0.001), ≥1 lobar microbleed (94% vs 26%, p < 0.001), past intracerebral hemorrhage (17% vs 4%, p = 0.04), ≥21 visible centrum semiovale perivascular spaces (34% vs 4%, p < 0.01), and leptomeningeal enhancement (70% vs 27%, p < 0.001). Patients with BP-PACNS more often had headaches (56% vs 31%, p < 0.01), motor deficits (56% vs 36%, p = 0.02), and nonischemic parenchymal gadolinium enhancement (82% vs 16%, p < 0.001). The prevalence of acute ischemic lesions was 18% in CAA-RI and 22% in BP-PACNS (p = 0.57). The features with the highest specificity for CAA-RI were acute subarachnoid hemorrhage (98%), cortical superficial siderosis (96%), past intracerebral hemorrhage (96%), and ≥21 visible centrum semiovale perivascular spaces (96%). The probable CAA-RI criteria had a 71% sensitivity (95% CI 44%-90%) and 91% specificity (95% CI 79%-98%) in differentiating biopsy-positive CAA-RI from BP-PACNS. The rate of relapse in the first 2 years after remission was lower in CAA-RI than in BP-PACNS (hazard ratio 0.46, 95% CI 0.22-0.96, p = 0.04). CONCLUSION: Clinicoradiologic features differed between patients with CAA-RI and those with BP-PACNS. Specific markers for CAA-RI were hemorrhagic signs of subarachnoid involvement, past intracerebral hemorrhage, ≥21 visible centrum semiovale perivascular spaces, and the probable CAA-RI criteria. A biopsy remains necessary for diagnosis in some cases of CAA-RI. The rate of relapse in the first 2 years after disease remission was lower in CAA-RI than in BP-PACNS.


Asunto(s)
Angiopatía Amiloide Cerebral , Vasculitis del Sistema Nervioso Central , Humanos , Femenino , Masculino , Angiopatía Amiloide Cerebral/diagnóstico por imagen , Angiopatía Amiloide Cerebral/patología , Angiopatía Amiloide Cerebral/complicaciones , Anciano , Persona de Mediana Edad , Vasculitis del Sistema Nervioso Central/diagnóstico por imagen , Vasculitis del Sistema Nervioso Central/patología , Estudios Retrospectivos , Biopsia , Imagen por Resonancia Magnética , Anciano de 80 o más Años , Encéfalo/patología , Encéfalo/diagnóstico por imagen , Adulto , Recurrencia
2.
Stroke ; 55(8): 2086-2093, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38920025

RESUMEN

BACKGROUND: Periventricular white matter hyperintensities (PVWMHs) in cerebral amyloid angiopathy (CAA) have been reported posterior predominant using semiautomated segmentation method and logarithmic transformation. We aimed to compare PVWMH extent and posterior/anterior distribution between patients with CAA and patients with hypertensive arteriopathy with radiological tools available in daily practice. METHODS: We retrospectively analyzed confluent PVWMH directly adjacent to lateral ventricles on axial FLAIR (fluid-attenuated inversion recovery) from 108 patients with CAA and 99 patients with hypertensive arteriopathy presenting with hemorrhage-related symptoms consecutively recruited in our stroke database (Nîmes University Hospital, France) between January 2015 and March 2022. For each of the left (L), right (R), anterior (A), and posterior (P) horns of lateral ventricles, the maximal distance between the outer PVWMH border and ventricle border was measured. The sum of anterior left PVWMH and anterior right PVWMH, and posterior left PVWMH and posterior right PVWMH resulted in anterior and posterior extent, respectively. RESULTS: Compared with hypertensive arteriopathy, patients with CAA were older (median, 77 versus 71; P=0.0010) and less frequently male (46% versus 64%; P=0.012) and had less frequent hypertension (45% versus 63%; P=0.013) and more chronic hemorrhages (P<0.0001). CAA showed slightly more extensive anterior right PVWMH (median, 6.50 versus 5.90 mm; P=0.034), far more extensive (all P<0.0001) posterior left PVWMH (median, 13.95 versus 6.95 mm), posterior right PVWMH (median, 14.15 versus 5.45 mm), posterior (median, 27.95 versus 13.00 mm), and total (median, 39.60 versus 24.65 mm) PVWMH, and higher posterior/anterior ratios (median, 1.82 versus 1.01). Age-/sex-adjusted model predicting CAA incorporating total PVWMH extent and posterior/anterior ratios for the given score (-4.3683+0.0268×PVWMH-T+0.3749×posterior/anterior PVWMH ratio+0.0394×age+0.3046 when female) showed highest area under the curve (0.76 [0.70-0.83]), with a 72% [62.50-80.99] sensitivity and 76% [67.18-84.12] specificity. Values above the optimal threshold of 0.22 for the score showed a crude relative risk of 2.75 (2.26-2.37; P<0.0001). CONCLUSIONS: Severe posterior PVWMH and high posterior/anterior PVWMH ratio assessed by radiological tools used in daily practice are associated with probable CAA versus hypertensive arteriopathy. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT05486897.


Asunto(s)
Angiopatía Amiloide Cerebral , Hipertensión , Imagen por Resonancia Magnética , Humanos , Masculino , Femenino , Anciano , Estudios Retrospectivos , Hipertensión/complicaciones , Hipertensión/diagnóstico por imagen , Persona de Mediana Edad , Angiopatía Amiloide Cerebral/diagnóstico por imagen , Angiopatía Amiloide Cerebral/complicaciones , Anciano de 80 o más Años , Sustancia Blanca/diagnóstico por imagen , Sustancia Blanca/patología , Diagnóstico Diferencial
3.
Brain ; 2024 May 02.
Artículo en Inglés | MEDLINE | ID: mdl-38696726

RESUMEN

Congenital myasthenic syndromes (CMS) are clinically and genetically heterogeneous diseases caused by mutations affecting neuromuscular transmission. Even if the first symptoms mainly occur during childhood, adult neurologists must confront this challenging diagnosis and manage these patients throughout their adulthood. However, long-term follow-up data from large cohorts of CMS patients are lacking and the long-term prognosis of these patients is largely unknown. We report the clinical features, diagnostic difficulties, and long-term prognosis of a French nationwide cohort of 235 adult patients with genetically confirmed CMS followed in 23 specialized neuromuscular centres. Data were retrospectively analysed. Of the 235 patients, 123 were female (52.3%). The diagnosis was made in adulthood in 139 patients, 110 of whom presented their first symptoms before the age of 18. Mean follow-up time between first symptoms and last visit was 34 years (SD = 15.1). Pathogenic variants were found in 19 disease-related genes. CHRNE-low expressor variants were the most common (23.8%), followed by variants in DOK7 (18.7%) and RAPSN (14%). Genotypes were clustered into four groups according to the initial presentation: ocular group (CHRNE-LE, CHRND, FCCMS), distal group (SCCMS), limb-girdle group (RAPSN, COLQ, DOK7, GMPPB, GFPT1), and a variable-phenotype group (MUSK, AGRN). The phenotypical features of CMS did not change throughout life. Only four genotypes had a proportion of patients requiring intensive care unit (ICU) admission that exceeded 20%: RAPSN (54.8%), MUSK (50%), DOK7 (38.6%) and AGRN (25.0%). In RAPSN and MUSK patients most ICU admissions occurred before age 18 years and in DOK7 and AGRN patients at or after 18 years of age. Different patterns of disease course (stability, improvement and progressive worsening) may succeed one another in the same patient throughout life, particularly in AGRN, DOK7 and COLQ. At the last visit, 55% of SCCMS and 36.3% of DOK7 patients required ventilation; 36.3% of DOK7 patients, 25% of GMPPB patients and 20% of GFPT1 patients were wheelchair-bound; most of the patients who were both wheelchair-bound and ventilated were DOK7 patients. Six patients died in this cohort. The positive impact of therapy was striking, even in severely affected patients. In conclusion, even if motor and/or respiratory deterioration could occur in patients with initially moderate disease, particularly in DOK7, SCCMS and GFPT1 patients, the long-term prognosis for most CMS patients was favourable, with neither ventilation nor wheelchair needed at last visit. CHRNE patients did not worsen during adulthood and RAPSN patients, often severely affected in early childhood, subsequently improved.

4.
Eur J Neurol ; 31(7): e16292, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38587143

RESUMEN

INTRODUCTION: Late-onset Pompe disease (LOPD) is characterized by a progressive myopathy resulting from a deficiency of acid α-glucosidase enzyme activity. Enzyme replacement therapy has been shown to be effective, but long-term treatment results vary. Avalglucosidase alfa demonstrated non-inferiority to alglucosidase alfa in a phase 3 study, allowing in France compassionate access for advanced LOPD patients unresponsive to alglucosidase alfa. METHODS: Data from the French Pompe registry were analyzed for patients who benefited from a switch to avalglucosidase alfa with at least 1 year of follow-up. Respiratory (forced vital capacity [FVC]) and motor functions (Six-Minute Walk Test [6MWT]) were assessed before and 1 year after switching. Individual changes in FVC and 6MWT were expressed as slopes and statistical analyses were performed to compare values. RESULTS: Twenty-nine patients were included (mean age 56 years, 11 years of prior treatment). The FVC and 6MWT values remained stable. The individual analyses showed a stabilization of motor worsening: -1 m/year on the 6MWT after the switch versus -63 m/year the year before the switch (i.e., a worsening of 33%/year before vs. an improvement of 3%/year later). Respiratory data were not statistically different. DISCUSSION: At the group level, gait parameters improved slightly with a stabilization of previous worsening, but respiratory parameters showed limited changes. At the individual level, results were discordant, with some patients with a good motor or respiratory response and some with further worsening. CONCLUSION: Switching to avalglucosidase alfa demonstrated varied responses in advanced LOPD patients with failing alglucosidase alfa therapy, with a general improvement in motor stabilization.


Asunto(s)
Terapia de Reemplazo Enzimático , Enfermedad del Almacenamiento de Glucógeno Tipo II , alfa-Glucosidasas , Humanos , Enfermedad del Almacenamiento de Glucógeno Tipo II/tratamiento farmacológico , Enfermedad del Almacenamiento de Glucógeno Tipo II/complicaciones , Masculino , Persona de Mediana Edad , Femenino , Francia , alfa-Glucosidasas/uso terapéutico , Terapia de Reemplazo Enzimático/métodos , Anciano , Adulto , Estudios de Cohortes , Resultado del Tratamiento , Sistema de Registros , Progresión de la Enfermedad , Prueba de Paso , Sustitución de Medicamentos
5.
J Neuroimmunol ; 390: 578346, 2024 05 15.
Artículo en Inglés | MEDLINE | ID: mdl-38648696

RESUMEN

The frequency of corticospinal tract (CST) T2/FLAIR hyperintensity in disorders with neuroglial antibodies is unclear. Herein, we retrospectively reviewed brain MRIs of 101 LGI1-antibody encephalitis patients, and observed CST hyperintensity in 30/101 (30%). It was mostly bilateral (93%), not associated with upper motor neuron signs/symptoms (7%), and frequently decreased over time (39%). In a systematic review including patients with other neuroglial antibodies, CST hyperintensity was reported in 110 with neuromyelitis optica (94%), myelin oligodendrocyte glycoprotein-associated disease (2%), Ma2-antibody (3%) and GAD65-antibody paraneoplastic neurological syndrome (1%). CST hyperintensity is not an infrequent finding in LGI1-Ab encephalitis and other disorders with neuroglial antibodies.


Asunto(s)
Autoanticuerpos , Encefalitis , Péptidos y Proteínas de Señalización Intracelular , Tractos Piramidales , Humanos , Autoanticuerpos/inmunología , Autoanticuerpos/sangre , Femenino , Persona de Mediana Edad , Masculino , Estudios Retrospectivos , Anciano , Adulto , Encefalitis/inmunología , Encefalitis/diagnóstico por imagen , Tractos Piramidales/diagnóstico por imagen , Tractos Piramidales/patología , Tractos Piramidales/inmunología , Péptidos y Proteínas de Señalización Intracelular/inmunología , Imagen por Resonancia Magnética , Adulto Joven , Neuroglía/patología , Neuroglía/inmunología , Adolescente , Anciano de 80 o más Años , Enfermedades del Sistema Nervioso Central/inmunología , Enfermedades del Sistema Nervioso Central/diagnóstico por imagen
6.
Sensors (Basel) ; 24(5)2024 Feb 23.
Artículo en Inglés | MEDLINE | ID: mdl-38474970

RESUMEN

Digital twin technology is a highly valued asset in the manufacturing sector, with its unique capability to bridge the gap between the physical and virtual parts. The impact of the rapid increase in this technology is based on the collection of real-world data, its standardization, and its widespread deployment on an existing manufacturing system. This encompasses sensor values, PLC internal states, and IoT, as well as how the means of linking these data with their digital counterparts. It is challenging to implement digital twins on a large scale due to the heterogeneity of protocols and data structuring of subsystems. To facilitate the integration of the digital twin into existing manufacturing architectures, we propose in this paper a framework that enables the deployment of scalable digital twins from sensors to services of digital twins in an iterative manner.

8.
J Stroke Cerebrovasc Dis ; 32(12): 107435, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37871401

RESUMEN

BACKGROUND AND OBJECTIVES: Intraluminal thrombus (ILT) is a relatively rare cause of stroke. MRI/MRA is increasingly used for stroke patients eligible for acute treatment. Radiological ILT characteristics have never been studied systematically on MRA. We aimed to analyse signal changes and other radiological characteristics of ILT in the acute phase on MRA in a case series of ILT patients. MATERIALS AND METHODS: Consecutive stroke patients registered in our stroke database between January 2013 and September 2022 were screened for supra-aortic ILT on MRA, in whom MRA was the first supra-aortic vessel imaging performed. Twelve patients were included for analysis. Radiological analysis on MRA included ILT localisation, diameter of the free-floating ILT component, total ITL length, degree of stenosis caused by ILT, and ILT signal intensity. RESULTS: Median age was 65 years and 92% were men. Median time interval between stroke onset and MRA was 0.5 days. Atherosclerosis was the underlying ILT aetiology in half of the patients. The most frequent ILT localisation was the internal carotid artery, always involving the C1 segment. Median ILT diameter was 4.35 mm, median length 20.9 mm, median stenosis 65%, and with a homogeneous hypointensity of the ILT in 67% and a mixed hypo-hyperintensity in 33% (with ILT showing central hyperintensity surrounded by peripheral hypointensity). DISCUSSION: When observed on MRA, ILT showed a homogeneous hypointensity in two-thirds and a mixed hypo-hyperintensity in one third of patients. In future studies, follow-up MRA scans should be performed to analyse whether these signal changes are time-related or influenced by antithrombotic treatment for ILT.


Asunto(s)
Aneurisma de la Aorta Abdominal , Accidente Cerebrovascular , Trombosis , Masculino , Humanos , Anciano , Femenino , Angiografía por Resonancia Magnética/métodos , Constricción Patológica , Trombosis/diagnóstico por imagen , Trombosis/etiología , Accidente Cerebrovascular/diagnóstico por imagen , Accidente Cerebrovascular/etiología
10.
Neurology ; 101(9): e966-e977, 2023 08 29.
Artículo en Inglés | MEDLINE | ID: mdl-37419682

RESUMEN

BACKGROUND AND OBJECTIVES: The French Pompe disease registry was created in 2004 for study of the natural course of the disease in patients. It rapidly became a major tool for assessing the long-term efficacy of enzyme replacement therapy (ERT) after the market release of alglucosidase-alfa. METHODS: Approximately 10 years after publication of the baseline characteristics of the 126 initial patients of the French Late-Onset Pompe Disease registry, we provide here an update of the clinical and biological features of patients included in this registry. RESULTS: We describe 210 patients followed at 31 hospital-based French neuromuscular or metabolic centers. The median age at inclusion was 48.67 ± 14.91 years. The first symptom was progressive lower limb muscle weakness, either isolated (50%) or associated with respiratory symptoms (18%), at a median age of 38 ± 14.9 years. At inclusion, 64% of the patients were able to walk independently and 14% needed a wheelchair. Positive associations were found between motor function measure, manual motor test, and 6-minute walk test (6MWT) results, and these parameters were inversely associated with the time taken to sit up from a lying position at inclusion. Seventy-two patients had been followed for at least 10 years in the registry. Thirty-three patients remained untreated a median of 12 years after symptom onset. The standard ERT dose was administered for 177 patients. DISCUSSION: This update confirms previous findings for the adult population included in the French Pompe disease registry, but with a lower clinical severity at inclusion, suggesting that this rare disease is now diagnosed earlier; thanks to greater awareness among physicians. The 6MWT remains an important method for assessing motor performance and walking ability. The French Pompe disease registry provides an exhaustive, nationwide overview of Pompe disease and can be used to assess individual and global responses to future treatments.


Asunto(s)
Enfermedad del Almacenamiento de Glucógeno Tipo II , Adulto , Humanos , Persona de Mediana Edad , Adulto Joven , Enfermedad del Almacenamiento de Glucógeno Tipo II/tratamiento farmacológico , Enfermedad del Almacenamiento de Glucógeno Tipo II/epidemiología , Enfermedad del Almacenamiento de Glucógeno Tipo II/diagnóstico , alfa-Glucosidasas/uso terapéutico , Debilidad Muscular/etiología , Francia/epidemiología , Sistema de Registros , Caminata , Terapia de Reemplazo Enzimático/efectos adversos , Terapia de Reemplazo Enzimático/métodos
11.
Cerebrovasc Dis ; 52(6): 706-710, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36878206

RESUMEN

BACKGROUND: Cerebellar superficial siderosis (SS) has been recently reported to be present in about 10% of both hereditary and sporadic cerebral amyloid angiopathy (CAA) on 3T MRI using primarily susceptibility-weighted imaging. OBJECTIVES: Our aim was to assess cerebellar SS in sporadic CAA patients using 1.5T T2*-weighted MRI and to evaluate possible underlying mechanisms. METHOD: We retrospectively screened MRI scans of sporadic probable CAA patients initially presenting with intracerebral hemorrhage-, acute subarachnoid hemorrhage- or cortical SS-related symptoms between September 2009 and January 2022 registered in our stroke database. Patients with familial CAA were excluded. On 1.5T T2*-weighted MRI, cerebellar SS (including kappa statistics for interobserver agreement) was assessed together with typical CAA hemorrhagic features and with the presence of supratentorial macrobleed and cortical SS adjacent to the tentorium cerebelli (TC) and TC hemosiderosis. RESULTS: We screened 151 patients and finally included 111 CAA patients (median age 77) with cerebellar SS observed in 6 (5%) patients. Cerebellar SS presence was associated with a higher number of supratentorial macrobleeds (median n = 3 vs. n = 1, p = 0.0012), presence of supratentorial macrobleed adjacent to the TC (p = 0.002), and TC hemosiderosis (p = 0.005). CONCLUSIONS: Cerebellar SS in CAA patients can be identified on 1.5T T2*-weighted imaging. Associated MRI characteristics suggest contamination from supratentorial macrobleeds.


Asunto(s)
Angiopatía Amiloide Cerebral , Hemosiderosis , Siderosis , Humanos , Anciano , Siderosis/etiología , Siderosis/complicaciones , Estudios Retrospectivos , Imagen por Resonancia Magnética , Angiopatía Amiloide Cerebral/complicaciones , Angiopatía Amiloide Cerebral/diagnóstico por imagen , Hemorragia Cerebral/complicaciones , Hemosiderosis/etiología , Hemosiderosis/complicaciones
13.
Acta Neurol Belg ; 123(2): 519-527, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36241843

RESUMEN

OBJECTIVES: Primary acute convexity subarachnoid hemorrhage (cSAH) in older patients can be observed in cerebral amyloid angiopathy (CAA) or idiopathic (with cSAH as potential initial manifestation of suspected CAA). We aimed to analyze baseline, clinical and MRI (including quantitative cSAH surface analysis and topographical probabilistic cSAH mapping) characteristics in elderly cSAH patients with CAA. MATERIALS AND METHODS: Baseline/clinical/MRI characteristics of 50 consecutive primary acute cSAH patients ≥ 55 years with suspected/possible/probable CAA were retrospectively analyzed. RESULTS: Median age was 74, with 26% of patients showing suspected, 22% possible and 52% probable CAA. Transient focal neurological episode (TFNE) was observed in 78%, with spreading symptoms in 79% (median spreading speed five minutes), a median of two episodes before cSAH diagnosis, and similar symptoms in 91% when multiple TFNE, with a median duration of 15 min. Motor/sensory/speech/visual symptoms were observed in 85%/69%/46%/8%, respectively, and brachiofacial/brachial was the most frequent distribution for sensory-motor symptoms. Positive clinical-radiological correlation was observed in 84%, headache in 22%, and antiepileptics started in 78%. MRI showed chronic intracerebral hemorrhage in 10%, cortical superficial siderosis in 68%, cerebral microbleeds in 48%, median total Fazekas score of 3, lacunes in 6% and DWI lesion (all unique/cortical/ < 10 mm) in 6%. cSAH involved a median of 1 sulcus, with central sulcus as most frequently (47.5%) involved followed by precentral sulcus (17%). Median cSAH surface was 2170 mm2. No baseline, clinical or MRI characteristics were associated with cSAH surface extent in multivariate analysis. CONCLUSIONS: Baseline, clinical, or MRI features seem not to influence CAA-related cSAH extent. CLINICAL TRIAL REGISTRATION-URL: http://www. CLINICALTRIALS: gov . Unique identifier: NCT04825808.


Asunto(s)
Angiopatía Amiloide Cerebral , Hemorragia Subaracnoidea , Anciano , Humanos , Encéfalo/patología , Angiopatía Amiloide Cerebral/complicaciones , Hemorragia Cerebral/complicaciones , Imagen por Resonancia Magnética , Estudios Retrospectivos , Hemorragia Subaracnoidea/diagnóstico por imagen , Hemorragia Subaracnoidea/complicaciones
14.
J Stroke Cerebrovasc Dis ; 32(2): 106907, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36442282

RESUMEN

BACKGROUND AND OBJECTIVES: In stroke patients treated with intravenous thrombolysis (IVT), presence and high number of strictly lobar cerebral microbleeds (compatible with cerebral amyloid angiopathy, CAA) seems to be associated with increased risk of hemorrhagic transformation, symptomatic hemorrhagic transformation, remote hemorrhage, and poor functional outcome. Some of these CAA patients with cerebral microbleeds also have chronic lobar intracerebral hemorrhage. Few data are available on IVT-treated CAA patients showing cortical superficial siderosis. There are no reports studying factors associated with brain hemorrhagic complication or functional outcome in IVT-treated CAA patients. We present a case series study of IVT-treated stroke patients with CAA features on pre-IVT MRI in whom we have evaluated brain hemorrhagic complications on 24 h-CT and functional outcome after IVT. MATERIAL AND METHODS: In our stroke center, IVT decision in patients with CAA MRI features is at the physician's discretion. We retrospectively screened our stroke database between January 2015 and July 2022 for pre-IVT imaging of 959 consecutive IVT-treated stroke patients without ongoing anticoagulation therapy for probable CAA MRI features defined by modified Boston criteria. After exclusion of 119 patients with missing MRI (n = 47), MRI showing motion artefacts (n = 49) or with alternative chronic brain hemorrhage cause on MRI (n = 23), 15 IVT-treated patients with probable CAA on pre-IVT MRI were identified. In these 15 patients, clinical, biological and MRI characteristics were compared between patients with vs. without post-IVT hemorrhage and between patients with poor (MRS 3-6) vs. good (MRS 0-2) functional outcome at discharge. RESULTS: Two patients showed brain hemorrhage on 24 h-CT and both died after 40 and 31 days respectively. The remaining patients had no brain hemorrhage and showed very good outcome except one. Atrial fibrillation (p = 0.029) and Fazekas scale (p = 0.029) were associated with brain hemorrhage whereas atrial fibrillation (p = 0.0022), NIHSS (p = 0.027), blood glucose level (p = 0.024), CRP (p = 0.022) and DWI ASPECT (p = 0.016) were associated with poor outcome. DISCUSSION: Consequences of IVT in CAA patients can be dramatic. Larger studies are needed to compare IVT risks and outcome between CAA and non-CAA patients, also including CAA patients with chronic intracerebral hemorrhage or cortical superficial siderosis. In addition, future studies should try to identify clinical, biological and radiological features at high risk for brain hemorrhage and poor outcome in order to assess the risk-benefit ratio for IVT in CAA. CLINICAL TRIAL REGISTRATION-URL: http://www. CLINICALTRIALS: gov. Unique identifier: NCT05565144.


Asunto(s)
Fibrilación Atrial , Angiopatía Amiloide Cerebral , Siderosis , Accidente Cerebrovascular , Humanos , Estudios Retrospectivos , Fibrilación Atrial/complicaciones , Accidente Cerebrovascular/diagnóstico por imagen , Accidente Cerebrovascular/tratamiento farmacológico , Accidente Cerebrovascular/etiología , Imagen por Resonancia Magnética/efectos adversos , Angiopatía Amiloide Cerebral/complicaciones , Angiopatía Amiloide Cerebral/diagnóstico por imagen , Angiopatía Amiloide Cerebral/tratamiento farmacológico , Hemorragia Cerebral/etiología , Hemorragia Cerebral/complicaciones , Hemorragias Intracraneales/inducido químicamente , Hemorragias Intracraneales/diagnóstico por imagen , Terapia Trombolítica/efectos adversos , Tomografía Computarizada por Rayos X
15.
Neurol Sci ; 44(1): 405-407, 2023 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-36097204

RESUMEN

We present the MRI of a 70-year-old patient showing unilateral subcortical extensive dilated perivascular spaces with surrounding fluid-attenuated inversion recovery hyperintensities associated with the presence of small cysts and tubular hypointensities in and near the superior sagittal sinus co-locating with draining cortical veins on gadolinium-enhanced T1-weighted imaging representing probably (dilated) spaces between pial sheath and cortical vein walls. These (peri)venous superior sagittal sinus cysts seem to represent either hydrops cause (by blocking interstitial fluid flow in perivenous subpial space, via meningeal lymphatics) or consequence (where cysts might have been formed due to subpial fluid flow obstruction by unknown cause).


Asunto(s)
Quistes , Seno Sagital Superior , Humanos , Anciano , Seno Sagital Superior/diagnóstico por imagen , Dilatación Patológica , Meninges , Imagen por Resonancia Magnética/métodos
16.
Neurol Sci ; 43(11): 6597-6599, 2022 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-35915270

RESUMEN

A 43-year-old woman presented 1 day after whiplash injury with behavior change, hypersomnia, and abulia. MRI showed symmetrical globus pallidus infarction and bilateral watershed hypoperfusion. Magnetic resonance angiography (MRA) showed bilateral carotid artery dissection. To our knowledge, isolated symmetrical globus pallidus infarction related to bilateral carotid dissection has never been reported earlier.


Asunto(s)
Disección Aórtica , Enfermedades de las Arterias Carótidas , Femenino , Humanos , Adulto , Globo Pálido/diagnóstico por imagen , Imagen por Resonancia Magnética , Enfermedades de las Arterias Carótidas/patología , Infarto , Arterias Carótidas
17.
Acta Neurol Belg ; 122(3): 597-613, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35428930

RESUMEN

MRI signal changes in the brainstem are observed in a multitude of disorders including vascular diseases, neoplastic lesions, degenerative diseases, inflammatory disorders, metabolic diseases, infections, and trauma. In some diseases, brainstem involvement is typical and sometimes isolated, while in other diseases, brainstem lesions are only observed occasionally in the presence of other typical extra-brainstem abnormalities. In this review, we will discuss the MRI characteristics of brainstem lesions observed in different disorders associated with frequent and less frequent brainstem involvement. Identification of the origin of the brainstem lesion depends on the exact localisation of the lesion(s) inside the brainstem, the presence and the characteristics of associated lesions seen outside the brainstem, the signal changes on different MRI sequences, the evolution over time of the radiological abnormalities, the history and clinical state of the patient, and other radiological and non-radiological examinations.


Asunto(s)
Enfermedades Metabólicas , Enfermedades del Sistema Nervioso , Tronco Encefálico/diagnóstico por imagen , Tronco Encefálico/patología , Humanos , Imagen por Resonancia Magnética , Enfermedades del Sistema Nervioso/patología , Radiografía
19.
Front Nutr ; 9: 919336, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36733470

RESUMEN

Background and aims: Multiple sclerosis (MS) is associated with osteoporosis, possibly due to neurological disability and decreased calcium intake. The objective of this study was to evaluate the efficacy of a personalized nutritional advice program by a dietitian compared to the delivery of a standard advice form to optimize dietary calcium intake in outpatients with MS. Methods: We performed a randomized, controlled, parallel trial comparing the efficacy of a personalized dietary advice (PDA) program to standard advice form (SAF) to increase daily calcium intake in MS patients. The study population was composed by patients with relapsing-remitting MS aged 18-69 years old. PDA program consisted in dietary advice delivered by a dietitian at baseline, 1 month, and 3 months. Calcium and nutrient intake in patients from both groups was evaluated at baseline and 6 months using a dietary survey. Results: Of the 194 patients screened for inclusion, 182 patients were included (79% female, median age of 42 years, and median EDSS of 2.0), and randomized to SAF (n = 92) or PDA (n = 90). At 6 months, median calcium intake increased by 241 mg/day in the PDA group and decreased by 120 mg/day in the SAF group (p < 0.0001). However, the median calcium intake was 947 mg/day in the SAF group and 778 mg/day in the PDA group at baseline (p = 0.0077), potentially favoring the effect of dietary advice. Complementary analyses focusing on patients with insufficient calcium intakes at baseline revealed comparable values in both groups (p = 0.69). Of those, patients included in the PDA group obtained significantly higher calcium intakes at 6 months than patients from the SAF group (p = 0.0086) independently of EDSS, PASAT, HADS and EQ-5D scores. Conclusion: This work shows the efficacy of dietary management based on personalized advice program over 3 months to durably increase calcium consumption in MS patients with insufficient calcium intake. Clinical trial registration: clinicaltrials.gov, identifier NCT02664623.

20.
Eur J Neurol ; 29(4): 1056-1061, 2022 04.
Artículo en Inglés | MEDLINE | ID: mdl-34941017

RESUMEN

BACKGROUND AND PURPOSE: Intraplaque hemorrhage is a key feature of vulnerable carotid atherosclerotic plaque (CAP), associated with low densities (<25 Hounsfield units [HU]) on computed tomographic angiography (CTA). This study aimed to analyze CAP on routine CTA performed in patients with symptomatic and asymptomatic carotid stenosis undergoing carotid endarterectomy (CEA) by assessing HU of the CAP area showing the lowest density (CAPALD) using radiological tools available in daily clinical practice, and to compare CAPALD values between symptomatic and asymptomatic carotids. METHODS: We retrospectively screened preoperative CTA scans of 206 consecutive adult patients undergoing CEA for symptomatic or asymptomatic stenosis. CAPALD values were compared between symptomatic and asymptomatic carotids. Asymptomatic carotids included arteries contralateral to the symptomatic CEA artery, and asymptomatic stenotic arteries undergoing CEA and their contralateral arteries. Carotids were excluded when there was <30% stenosis, or when CAP could not be identified or CAPALD could not be measured. RESULTS: In total, 95 symptomatic and 112 asymptomatic carotids (derived from 174 patients) were analyzed. In multivariate analysis, symptomatic arteries showed more severe stenosis (median 70% vs. 67%, p = 0.0228) and lower CAPALD values (median 17 vs. 25 HU, p = 0.049), whereas degree of stenosis and CAPALD values were not correlated (rho = -0.02, p = 0.77). HU values of <25 were more frequent in symptomatic than asymptomatic carotids (68% vs. 47%, p = 0.0022). CONCLUSIONS: On CTA, symptomatic carotids are associated with CAP areas with low densities. CTA analysis of CAP may be interesting to help identify vulnerable plaques at risk for future stroke, especially in patients lacking strict indications for CEA based on the current guidelines.


Asunto(s)
Estenosis Carotídea , Endarterectomía Carotidea , Placa Aterosclerótica , Adulto , Angiografía , Arterias Carótidas , Estenosis Carotídea/complicaciones , Estenosis Carotídea/diagnóstico por imagen , Estenosis Carotídea/cirugía , Humanos , Placa Aterosclerótica/complicaciones , Placa Aterosclerótica/diagnóstico por imagen , Estudios Retrospectivos
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