Journal of neurology
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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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The aim of this study was to evaluate the feasibility of ocular ultrasound in the follow-up of pseudotumor cerebri syndrome and to correlate ultrasound with clinical parameters. In a cohort study of 22 consecutive adult patients, ocular ultrasound was performed measuring optic nerve sheath diameter, optic nerve diameter, and optic disc elevation compared with symptoms referred by the patients. The patients showed increased optic nerve sheath diameter [median, 6.51 mm (interquartile range 6.13-7.10)], optic nerve diameter [3.02 mm (2.86-3.27)], and optic disc elevation [0.90 mm (0.64-1.36)] at the time of admission and had ophthalmologically confirmed the presence of papilledema in all 22 patients. ⋯ In addition, a discrete negative correlation between optic nerve sheath diameter and headache change after 6 months was observed with ρ = - 0.477 and P = 0.02. No correlation was found between optic disc elevation and headache. In conclusion, longitudinal follow-up with ocular ultrasound combined with clinical information may provide support for treatment of this condition.
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Journal of neurology · Feb 2018
Randomized Controlled Trial Clinical TrialA randomized double-blind, placebo-controlled, cross-over trial (Vestparoxy) of the treatment of vestibular paroxysmia with oxcarbazepine.
Vestibular paroxysmia (VP) is characterized by short, often oligosymptomatic attacks of vertigo which occur spontaneously or are sometimes provoked by turning the head. Despite the description of the disease almost 40 years ago (first termed "disabling positional vertigo"), no controlled treatment trial has been published to date. The Vestparoxy trial was designed as a randomized, placebo-controlled, double-blind cross-over trial to examine the therapeutic effect of oxcarbazepine (OXA) in patients with definite or probable VP. ⋯ The Vestparoxy trial showed a significant reduction of VP attacks under OXA compared to placebo treatment, confirming the known and revealing no new side effects.
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Journal of neurology · Jan 2018
A nationwide survey of the influence of month of birth on the risk of developing multiple sclerosis in Sweden and Iceland.
Previous studies have shown that the risk of multiple sclerosis (MS) is associated with season of birth with a higher proportion of MS patients being born in spring. However, this relationship has recently been questioned and may be due to confounding factors. Our aim was to assess the influence from season or month of birth on the risk of developing MS in Sweden and Iceland. ⋯ No significant differences were found in subgroup analyses including data of latitude of birth, gender, clinical phenotype, and MS onset of 30 years or less. Our results do not support the previously reported association between season or month of birth and MS risk. Analysis of birth place and birth year as possible confounding factors showed no major influence of them on the seasonal MS risk in Sweden and Iceland.
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Journal of neurology · Jan 2018
Freezing of gait in Parkinson's disease: gray and white matter abnormalities.
Freezing of gait (FOG) is a disabling disorder that often affects Parkinson's disease (PD) patients in advanced stages of the disease. To study structural gray matter (GM) and white matter (WM) changes in PD patients with and without FOG, twenty-one PD patients with FOG (PD-FOG), 16 PD patients without FOG (PD-nFOG) and 19 healthy subjects (HS) underwent a standardized MRI protocol. For the gray matter evaluation, cortical volume (CV), cortical thickness (CTh), and surface area (SA) were analyzed using the FreeSurfer pipeline. ⋯ Moreover, significant WM changes were observed in PD-FOG patients in comparison with HS in the superior longitudinal fasciculus, uncinate fasciculus, cingulum cingulate gyrus and inferior longitudinal fasciculus (prevalently in the right hemisphere) and in the frontal radiations of the corpus callosum. DTI abnormalities in specific WM bundles correlated significantly with cognitive measures. The damage of multiple cortical areas involved in high-level gait control together with WM disruption between motor, cognitive and limbic structures may represent the anatomical correlate of FOG.