Acta neurologica Belgica最新文献

Purpose

To compare thymic features using unenhanced computed tomography (CT) images between children with non-thymomatous ocular myasthenia gravis (OMG) and healthy children and determine the image feature cut-off values to allow differentiation of the two populations.

Methods

We conducted a retrospective study involving 66 children who underwent chest CT, with 33 having non-thymomatous OMG and 33 healthy children without any abnormality. We recorded CT features of the thymus in each child, including the mean CT attenuation value, thymus area, area ratio, anteroposterior (AP) length, width (W), thickness (T), and transverse diameter (TD). These characteristics were analyzed to distinguish OMG from healthy controls. The receiver operating characteristic (ROC) analysis was used to clarify the best assessment, and the optimal cut-off values were determined.

Results

The mean CT attenuation value, the thymus area, area ratio, and TD were significantly different (P < 0.05) between the two groups. The mean CT attenuation value was the most significant characteristic in differentiating OMG and healthy controls (area under the curve [AUC] = 0.70). Using 50.41 as the cut-off value for mean CT attenuation, a specificity of 0.70 and sensitivity of 0.82 was observed for distinguishing the two groups (P = 0.003). Optimal differentiation was achieved by combining more than one characteristic including CT attenuation, thymus area, and TD with an optional area ratio (AUC = 0.72).

Conclusions

Non-enhanced CT of the thymus complemented the clinical workup of children with non-thymomatous OMG. The cut-off values of the CT features may allow robust differentiation of OMG children.

Background

Findings from functional neuroimaging techniques, such as single-photon emission computed tomography (SPECT), may add useful evidence improving Frontotemporal Dementia (FTD) diagnosis. The aim of the present study was to investigate patterns of hypoperfusion in a group of patients diagnosed with the behavioral variant of FTD (bvFTD) and to explore the relationship between brain perfusion and clinical characteristics.

Materials and methods

Brain perfusion of 23 bvFTD patients was measured with SPECT scintigraphy in lobes and Brodmann areas (BAs) and the NeurogamTM software was used for image analysis. To assess behavioral disturbances and dementia severity, patients’ informants completed the Frontotempotal Behavioral Inventory and the Frontotemporal Dementia Rating Scale. Descriptive statistics were used for the detection of pathological hypoperfusion in lobes and selected BAs. Associations among patients’ clinical characteristics and perfusion in lobes were explored via non-parametric correlations.

Results

Participants presented pathological hypoperfusion in frontal, limbic and temporal lobes. The most prominent deficit was observed in limbic lobes, where all participants showed pathological hypoperfusion. Decreased perfusion was also observed in limbic, frontal and temporal BAs. Perfusion in the left and right frontal lobe was associated with behavioral disturbances and disease severity, which was also correlated with perfusion in right limbic, left and right temporal areas.

Conclusion

Patterns of limbic, frontal and temporal hypopefusion were reported in the present study, along with associations between brain perfusion, behavioral disturbance and severity of dementia. Perfusion patterns can help to understand further associated brain biomarkers, contributing to early diagnosis and intervention in bvFTD.

Objectives

This study aimed to investigate relationships between cholesterol profile, brain volumetric MRI, and clinical measures in a large observational cohort of multiple sclerosis (MS) patients.

Materials and methods

We included 1.505 patients with 4.966 time points including complete lipid, clinical, and imaging data. The time among lipid, brain MRI and clinical measures was under 90 days. Cross-sectional statistical analysis at baseline was performed using an adjusted linear regression and analysis of longitudinal lipid and MRI measures data was performed using adjusted linear mixed models.

Results

We found associations between higher high-density lipoprotein cholesterol (HDL-C) and lower brain parenchymal fraction (BPF) at cross-sectional analysis at baseline (B = −0.43, CI 95%: −0.73, −0.12, p = 0.005), as well as in longitudinal analysis over follow-up (B = −0.32 ± 0.072, χ² = 36.6; p = < 0.001). Higher HDL-C was also associated with higher T2-lesion volume in longitudinal analysis (B = 0.11 ± 0.023; χ² = 23.04; p = < 0.001). We observed a weak negative association between low-density lipoprotein cholesterol (LDL-C) levels and BPF at baseline (B = −0.26, CI 95%: −0.4, −0.11, p = < 0.001) as well as in longitudinal analysis (B = −0.06 ± 0.03, χ² = 4.46; p = 0.03). T2-LV did not show an association with LDL-C. We did not find any association between lipid measures and disability. The effect of lipid levels on MRI measures and disability was minimal (Cohen f2 < 0.02).

Conclusions

Our results contradict the previously described exclusively positive effect of HDL-C on brain atrophy in patients with MS. Higher LDL-C was weakly associated with higher brain atrophy but not with higher lesion burden.

The Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare neuromuscular disorder characterized by proximal muscle weakness, hyporeflexia or areflexia, and dysautonomia. Ocular and bulbar symptoms may also occur, though respiratory failure is uncommon; we report the case of a 21-year-old woman diagnosed with LEMS, without evidence of a tumor, who was initially treated with symptomatic medication, immunoglobulin, and steroids, resulting in significant clinical improvement. However, she later developed psychotic symptoms, prompting the discontinuation of steroids. Brain MRI and antineuronal antibody tests were negative. Subsequently, her condition deteriorated, leading to respiratory distress that required urgent intubation, and prolonged dysphagia that necessitated the insertion of a gastrostomy tube for nutrition, along with the maintenance of a tracheostomy. Plasmapheresis was performed, resulting in partial motor recovery. Rituximab was then introduced, leading to sustained improvement in her neuromuscular symptoms, although her neuropsychiatric symptoms persisted; this case highlights a severe progression of young-onset LEMS, marked by prominent bulbar dysfunction and respiratory distress. Neuromuscular improvement followed rituximab treatment, while the concurrent psychotic symptoms appeared to follow an independent course, suggesting a primary psychiatric comorbidity.

Objective

Upper extremity dysfunction is frequently seen in Parkinson’s disease (PD). Existing research has shown that bradykinesia, which is main symptom of PD, is primarily responsible but the combined effects of spinal posture and axial rigidity on upper extremity functions were not investigated yet. The aim of this study was to investigate upper extremity functions in patients with PD and to evaluate relationship of these with spinal posture and axial rigidity.

Methods

This prospective controlled study included 40 patients with PD and 40 healthy controls. Upper extremity function was measured with the 9-Hole Peg Test. Spinal posture and axial rigidity were measured with a Spinal Mouse.

Results

Compared with the control group, a decrease in upper extremity functions (p < 0.001), decreased lumbar lordosis (p = 0.003), and posterior sacral tilt (p = 0.021) were determined in patients’ group. Thoracic and lumbar mobility in the sagittal (all p < 0.001) and frontal planes (p = 0.004, p < 0.001) was found to be reduced in the patient group. A correlation was determined between upper extremity functions and lumbar mobility in the sagittal (p = 0.022, r= -0.362) and frontal planes (p = 0.045, r= -0.319) and lumbar lordosis (p = 0.048, r = 0.302).

Conclusions

The results of this study demonstrated that altered spinal posture and increased axial rigidity were related with decreased upper extremity functions in patients with PD. There is a need for further studies to investigate effect of trunk-based therapies on upper extremity function in patients with PD.