Journal of neurology
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Journal of neurology · Apr 2018
Longitudinal predictors of caregiver burden in amyotrophic lateral sclerosis: a population-based cohort of patient-caregiver dyads.
Caregiver burden is a recognised consequence of caring for a patient with neurodegeneration. Amyotrophic lateral sclerosis (ALS) differs from other neurodegenerations by its rapid progression and impairment of motor, cognitive, and behavioural function, which contribute to caregiver burden. However, longitudinal factors that determine the extent of caregiver burden, and in particular the impact of psychological distress among caregivers, have not been fully established. ⋯ In a patient cohort with relatively preserved cognitive function (65%), anxiety and depression at Time 1, as measured by the HADS, were the best predictors of caregiver burden at Time 3. This observation provides a mechanism by which caregiver burden can be identified by health-care professionals and a stepped care programme of intervention initiated.
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Journal of neurology · Mar 2018
Structural cerebellar correlates of cognitive functions in spinocerebellar ataxia type 2.
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum and characterized by a typical motor syndrome. In addition, the presence of cognitive impairment is now widely acknowledged as a feature of SCA2. Given the extensive connections between the cerebellum and associative cerebral areas, it is reasonable to hypothesize that cerebellar neurodegeneration associated with SCA2 may impact on the cerebellar modulation of the cerebral cortex, thus resulting in functional impairment. ⋯ Specific cerebellar GM regions were found to be affected in patients. Additionally, GM loss in cognitive posterior lobules (VI, Crus I, Crus II, VIIB, IX) correlated with visuospatial, verbal memory and executive tasks, while additional correlations with motor anterior (V) and posterior (VIIIA, VIIIB) lobules were found for the tasks engaging motor and planning components. Our results provide evidence that the SCA2 neurodegenerative process affects the cerebellar cortex and that MRI indices of atrophy in different cerebellar subregions may account for the specificity of cognitive symptomatology observed in patients, as result of a cerebello-cerebral dysregulation.
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Journal of neurology · Mar 2018
CSF neurofilament proteins as diagnostic and prognostic biomarkers for amyotrophic lateral sclerosis.
Elevated cerebrospinal fluid (CSF), Neurofilament Light (NF-L) and phosphorylated Heavy (pNF-H) chain levels have been found in Amyotrophic Lateral Sclerosis (ALS), with studies reporting a correlation of both neurofilaments (NFs) with the disease progression. Here, we measured NF-L and pNF-H concentrations in the CSF of ALS patients from a single tertiary Center and investigated their relationship with disease-related variables. A total of 190 ALS patients (Bulbar, 29.9%; Spinal, 70.1%; M/F = 1.53) and 130 controls with mixed neurological diseases were recruited. ⋯ We found that CSF NF-L and pNF-H levels in ALS were significantly increased when compared to CTL-1 (NF-L: ALS, 4.7 ng/ml vs CTL-1, 0.61 ng/ml, p < 0.001; pNF-H: ALS, 1.7 ng/ml vs CTL-1, 0.03 ng/ml, p < 0.0001), but not to CTL-2. Analysis of different clinical and prognostic variables disclosed meaningful correlations with both NF-L and pNF-H levels. Our results, from a relatively large ALS cohort, confirm that CSF NF-L and pNF-H represent valuable diagnostic and prognostic biomarkers in ALS.
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Journal of neurology · Mar 2018
Review Case ReportsBilateral carotid cavernous sinus fistula: a case report and review of the literature.
Carotid cavernous fistula (CCF) is an abnormal vascular shunt from the carotid artery to the cavernous sinus. They are commonly classified based on hemodynamics, etiology or anatomically. Hemodynamic classification refers to whether the fistula is high or low flow. ⋯ First-line treatment consists of endovascular embolization with either a metallic coil, endovascular balloon or embolic agent. It is unclear in the literature if bilateral cases are more difficult to treat or have a different prognosis. Our patient required two endovascular procedures suggesting that endovascular intervention may have reduced efficacy in bilateral cases.
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Journal of neurology · Mar 2018
A dedicated neurologist at the emergency department during out-of-office hours decreases patients' length of stay and admission percentages.
Emergency departments (EDs) worldwide face crowding, which hampers patient flow. In this study, the impact of a dedicated neurologist present at the ED on patient flow during out-of-office hours was assessed. ⋯ In our hypothesis-generating study, a dedicated neurologist present at the ED during out-of-office hours was associated with decreased patients' LOS and a decreased admission percentage, indicating increased decisiveness when the neurologist is present at the ED.