RESUMEN
BACKGROUND: In CADASIL (Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy), clinical severity is not related to the total burden of white matter hyperintensities (WMHs), presumably because of heterogeneous underlying tissue alterations. We aimed to investigate whether WMHs in the corpus callosum (WMHCC) are due to secondary degeneration and related to clinical severity. METHODS: We evaluated data from 228 CADASIL patients included in an ongoing prospective cohort with available 3-dimensional fluid-attenuated inversion recovery magnetic resonance imaging sequences. We analyzed in a blind manner WMHCC and lacunes in presumably connected areas to determine whether WMHCC are related to secondary degeneration. We evaluated the links between WMHCC and the Mattis dementia rating scale and the modified Rankin Scale-widely used measures of global cognitive performances and disability, respectively. Linear regression models were adjusted for age, sex, level of education, brain volume, number of lacunes, and volume of WMH. RESULTS: Among 228 patients, only 105 (46%) had WMHCC while all had WMH in the rest of the white matter. In 74% of cases, WMHCC crossed a presumably connected nearby lacune, which was significantly higher than the expected value if the spatial distributions of WMHCC and nearby lacunes were unrelated (11%; P<0.001). Patients with WMHCC had worse Mattis dementia rating scale (median [P25-P75], 138 [122-142] versus 143 [140-143]; P<0.001) and worse modified Rankin Scale (2 [1-3] versus 1 [0-1]; P<0.001). In adjusted models, Mattis dementia rating scale was significantly associated with WMHCC (estimate, -6.2 [95% CI, -11.8 to -0.1]). CONCLUSIONS: In CADASIL, WMHCC are likely related to secondary degeneration and are independently related to clinical severity, in contrast to the total burden of WMH.
Asunto(s)
CADASIL , Sustancia Blanca , Humanos , CADASIL/complicaciones , Estudios Prospectivos , Cuerpo Calloso/diagnóstico por imagen , Cuerpo Calloso/patología , Sustancia Blanca/diagnóstico por imagen , Sustancia Blanca/patología , Imagen por Resonancia Magnética , Encéfalo/patologíaRESUMEN
The first hospital-based registry of patients with multiple sclerosis (MS) was established at the University Department of Neurology, Sestre milosrdnice University Hospital Centre, Zagreb, Croatia, in 2014. The aim of the registry was to continuously provide data on the number of hospital-managed MS patients, patterns of disease progression, predictors of disability progression, changes in lifespan and long-term outcomes. Relevant medical data included age and gender of MS patients, family history of MS, data on previous immunization, disease course, Expanded Disability Status Scale (EDSS) score, cerebral magnetic resonance imaging (MRI) lesion load quantification, and cerebrospinal fluid analysis. Lifestyle habits in MS patients including smoking and alcohol consumption were also analyzed. All data were obtained from primary medical records between January 1, 2014 and January 1, 2015, and entered into the database. Data were evaluated retrospectively according to age and gender differences. Results showed that the majority of patients enrolled in the registry had the remitting relapsing course of disease, with low EDSS score indicating no disability or minimal disability. Cerebrospinal fluid analysis showed that oligoclonal bands were present in the majority of MS patients, with affected blood-brain-barrier permeability. According to the remitting relapsing course of the disease, cerebral MRI quantitative analysis demonstrated a significant lesion load in the majority of patients. When stratified by lifestyle habits, smokers and alcohol consumers were more prevalent among male patients. Our hospital-based registry might be considered as a prototype for the national MS registry and should be improved for reliable statistical analysis.
Asunto(s)
Esclerosis Múltiple , Adulto , Croacia/epidemiología , Evaluación de la Discapacidad , Progresión de la Enfermedad , Femenino , Hospitales Universitarios , Humanos , Masculino , Registros Médicos Orientados a Problemas/estadística & datos numéricos , Persona de Mediana Edad , Esclerosis Múltiple/diagnóstico , Esclerosis Múltiple/epidemiología , Sistema de Registros , Estudios RetrospectivosRESUMEN
We describe a 59-year-old stroke patient presented with sudden onset left side weakness and a speech disorder due to a major acute cerebral infarction in the area of the right middle cerebral artery, right posterior cerebral artery, and small infarctions in the area of the left middle and left posterior cerebral artery. For the previous month, the patient had been feeling weak and had no appetite. The patient had not been previously seriously ill. A chest x-ray showed a large mass in the upper zone of the right lung. Chest computed tomography scan and echocardiography were performed and revealed advanced lung cancer invading the left atrium. Spontaneous tumor embolism is a rare cause of stroke and should be considered in the differential diagnosis of stroke in a cancer patient. Also, sometimes stroke can be the first manifestation of advanced cancer.