Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jun 2015
SANDO syndrome in a cohort of 107 patients with CPEO and mitochondrial DNA deletions.
The sensory ataxic neuropathy with dysarthria and ophthalmoparesis (SANDO) syndrome is a subgroup of mitochondrial chronic progressive external ophthalmoplegia (CPEO)-plus disorders associated with multiple mitochondrial DNA (mtDNA) deletions. There is no systematic survey on SANDO in patients with CPEO with either single or multiple large-scale mtDNA deletions. ⋯ The SANDO syndrome seems to indicate a cluster of symptoms within the wide range of multisystemic symptoms associated with mitochondrial CPEO. SANO seems to be the most frequent phenotype associated with multiple mtDNA deletions in our cohort but not or is rarely associated with single, large-scale mtDNA deletions.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2015
The natural history of early versus late disability accumulation in primary progressive MS.
Primary progressive multiple sclerosis (PPMS) is the least common MS disease course and carries the worst prognosis. In relapsing-remitting multiple sclerosis (RRMS) disability accumulation occurs in two distinct phases, but it is unclear whether this is also true for PPMS. Here we investigate factors associated with early and late disability accumulation in PPMS. ⋯ Age at disease onset is the most important predictor of disability accumulation in PPMS. Bilateral motor onset symptoms were associated with quicker disease progression. In contrast to RRMS, we found no evidence for distinct phases of disability accumulation in PPMS. Disability accumulation in PPMS appears to be affected by the same factors throughout its course.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2015
Randomized Controlled Trial Comparative StudyLow-frequency versus high-frequency stimulation of the pedunculopontine nucleus area in Parkinson's disease: a randomised controlled trial.
To compare the influence of low-frequency (10-25 Hz) versus higher (60-80 Hz) frequency stimulation of the pedunculopontine nucleus area (PPNa) on akinaesia, freezing of gait and daytime sleepiness. ⋯ These results are in keeping with the benefits of chronic PPNa stimulation for gait and postural difficulties in patients with PD, and with regard to the influence of patients' clinical characteristics, differential neuronal loss in the PPNa and electrode location. We conclude that in patients with PPNa stimulation, low frequency provides a better outcome than high-frequency stimulation.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2015
Randomized Controlled Trial Comparative StudyNeuropsychological changes following deep brain stimulation surgery for Parkinson's disease: comparisons of treatment at pallidal and subthalamic targets versus best medical therapy.
Deep brain stimulation (DBS) improves motor symptoms in Parkinson's disease (PD), but questions remain regarding neuropsychological decrements sometimes associated with this treatment, including rates of statistically and clinically meaningful change, and whether there are differences in outcome related to surgical target. ⋯ In those with PD, the likelihood of significant decline in neuropsychological functioning increases with DBS, affecting a small minority of patients who also appear to respond less optimally to DBS by other indicators of QOL.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2015
ReviewThe use and misuse of short cognitive tests in the diagnosis of dementia.
Short cognitive tests are widely used in medicine to assess patients with memory problems but their role in the assessment of patients with cognitive problems is often misunderstood. They are a part of the examination of the patient and not tests for dementia or 'case-finding tools'. ⋯ Their use in clinical practice particularly in response to national directives aimed at increasing diagnosis rates in dementia needs a clear understanding of their role and limitations. A new classification of short cognitive tests is proposed with guidance on their use in clinical medicine.