Journal of neurology
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Journal of neurology · Oct 2013
Brain white matter diffusion tensor metrics from clinical 1.5T MRI distinguish between ALS phenotypes.
Patients with amyotrophic lateral sclerosis (ALS) can present with varying degrees of upper motor neuron (UMN) and lower motor neuron dysfunction. Previous diffusion tensor imaging (DTI) studies, in which ALS patients were not separated by the degree of UMN dysfunction, have resulted in conflicting or inconclusive results. We hypothesized that (1) categorizing ALS patients by their clinical phenotype can reveal differences in DTI abnormalities along the corticospinal tract (CST), and (2) data obtained from routine clinical DTI scans can provide this type of information. ⋯ Significant differences in AD values were not only seen between control and ALS patients but also between the ALS subgroups, suggesting divergent pathologies in these ALS patients. Classifying ALS patients by phenotype reveals differences in CST abnormalities between subgroups and may provide novel insights into disease mechanisms. The close similarity of our results from routine clinical scans with published research studies suggests wider accessibility to useful DTI metrics.
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Journal of neurology · Oct 2013
Ultrasonographic nerve enlargement of the median and ulnar nerves and the cervical nerve roots in patients with demyelinating Charcot-Marie-Tooth disease: distinction from patients with chronic inflammatory demyelinating polyneuropathy.
Demyelinating Charcot-Marie-Tooth disease (CMT) and chronic inflammatory demyelinating polyneuropathy (CIDP) are both demyelinating polyneuropathies. The differences in nerve enlargement degree and pattern at multiple evaluation sites/levels are not well known. We investigated the differences in nerve enlargement degree and the distribution pattern of nerve enlargement in patients with demyelinating CMT and CIDP, and verified the appropriate combination of sites/levels to differentiate between these diseases. ⋯ However, the nerve sizes of CNR were not significantly different between patients with either disease. When we evaluated ESN of four selected sites for screening from the intermediate region, the sensitivity and specificity to distinguish between demyelinating CMT and CIDP were 0.90 and 0.94, respectively, with the cut-off value set at four. Nerve ultrasonography is useful to detect nerve enlargement and can clarify morphological differences in nerves between patients with demyelinating CMT and CIDP.
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Journal of neurology · Oct 2013
Cerebral blood flow response to neural activation after acute ischemic stroke: a failure of myogenic regulation?
We tested two hypotheses: (1) neurovascular coupling is impaired after acute ischemic stroke, (2) subcomponent analysis of cerebral blood flow velocity can reveal significant differences between acute ischemic stroke and healthy controls. This was explored through the comparison of nineteen acute ischemic stroke patients with healthy controls. Recordings of cerebral blood flow velocity, blood pressure and end-tidal CO2 were obtained during 60s of passive elbow flexion. ⋯ Cerebral blood flow velocity responses to passive elbow flexion suggest an impairment of neurovascular coupling in acute ischemic stroke. Subcomponent analysis suggests an impairment of the myogenic pathways, giving a greater insight into the different mechanisms contributing to neurovascular coupling. Further research is needed to assess the clinical value of subcomponent analysis of neurovascular coupling and the natural history of such changes following acute ischemic stroke.
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To examine obstetrical epidural and spinal anesthesia use in women with multiple sclerosis (MS) and the relationship with MS clinical factors. This was a retrospective cohort study, linking clinical data from women with MS in the British Columbia (BC) MS database to obstetrical data (1998-2009) from the BC Perinatal Database Registry. We compared epidural use in 431 deliveries to women with MS and 2,959 deliveries from the general population, as well as spinal use in cesarean deliveries (128 to women with MS and 846 in the general population), considering parity and using multivariate models. ⋯ Women who delivered 5 to <10 years after MS onset were less likely to have an epidural (adjusted OR = 0.57, 95 % CI = 0.34-0.95, p = 0.03) vs. those delivering within 5 years. EDSS was not associated with use of either type of anesthesia (adjusted p > 0.1). Contrary to previous studies, epidural anesthesia use differed between women with MS and the general population and was influenced by parity and MS disease duration; these findings warrant further investigation.
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Journal of neurology · Oct 2013
Clinical, genetic, and brain sonographic features related to Parkinson's disease in Gaucher disease.
Homozygous or compound heterozygous mutations in the glucocerebrosidase gene cause Gaucher disease. Moreover, heterozygous glucocerebrosidase gene mutations represent the most common genetic risk factor for Parkinson's disease (PD) known so far. Substantia nigra (SN) hyperechogenicity, a sonographic feature thought to reflect iron accumulation, has been described in both PD and Gaucher disease patients. ⋯ While none of the five Gaucher disease patients with signs of PD (definite PD, n = 4; early PD, n = 1) had severe glucocerebrosidase gene mutations known to cause neuronopathic Gaucher disease, all carried a N370S allele, previously reported to predict non-neuronopathic Gaucher disease. Hyposmia, higher non-motor symptoms score (constipation, depression, executive dysfunction), and SN hyperechogenicity were characteristic features of Gaucher disease-related PD. We conclude that the combined clinical, genetic, and transcranial sonographic assessment may improve the PD risk evaluation in Gaucher disease.