Parkinsonism & related disorders
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Parkinsonism Relat. Disord. · Nov 2016
ReviewDeep brain stimulation and sleep-wake functions in Parkinson's disease: A systematic review.
Sleep-wake disturbances (SWD) are common nonmotor symptoms (NMS) and have a great impact on quality of life of patients with Parkinson's disease (PD). Deep brain stimulation (DBS) is an established treatment in PD. While the beneficial effects of DBS on cardinal PD motor symptoms are indisputable, the data for several NMS, including sleep-wake functions, are limited and often controversial. ⋯ The data concerning subthalamic DBS and wake functions are controversial and studies using objective, laboratory-based measures for the assessment of wake functions are lacking. Very few studies assessed the impact of other DBS targets (e.g. pallidal stimulation) on SWD. Further prospective observational DBS studies assessing subjectively and objectively specific sleep-wake parameters in patients with PD are needed.
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Parkinsonism Relat. Disord. · Oct 2016
Phenotypic differences based on staging of Alzheimer's neuropathology in autopsy-confirmed dementia with Lewy bodies.
The goal was to compare subgroups of dementia with Lewy Bodies (DLB) using neuropathological measures to differentiate 'pure' Lewy body (LB) dementia from 'mixed' DLB [co-occurring LB and Alzheimer's disease (AD) pathology] to facilitate diagnostic decision-making and future development of interventions based on predicted type(s) of neuropathology. Studies comparing these groups are rare relative to those differentiating 'pure' AD and all-cause DLB, and are limited by insufficient sample size, brief cognitive batteries, and/or absence of autopsy confirmation. To address these limitations, we assessed cognition and other features in a large, autopsy-confirmed DLB sample using an extensive neuropsychological battery. ⋯ Salient findings included greater impairment on visual tasks and speed of processing and more frequent reports of all-type hallucinations in DLB-LB compared to DLB-AD. Relatively intact confrontation naming in DLB-LB and no differences in reported delusions were of note. Identifying differences in phenotypic features can improve prediction of underlying neuropathology.
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Parkinsonism Relat. Disord. · Oct 2016
Neuroanatomical changes extend beyond striatal atrophy in X-linked dystonia parkinsonism.
X-linked dystonia-parkinsonism (XDP) is an inherited neurodegenerative adult-onset basal ganglia model disease associated with severe striatal atrophy. Anatomical changes exceeding striatal pathology were not yet described in XDP. The present study aimed to assess the microstructure of white matter tracts in XDP using magnetic resonance tomography. ⋯ The present study confirms striatal atrophy in XDP and provides evidence for a strong involvement of the white matter and the pallidum. This calls into question the previously held concept of exclusive striatal atrophy in this unique movement disorder. The spared occipital region may point towards a lack of anatomical connections with the atrophied striatum.
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Parkinsonism Relat. Disord. · Sep 2016
Case ReportsCytoplasmic aggregates of dynactin in iPSC-derived tyrosine hydroxylase-positive neurons from a patient with Perry syndrome.
Perry syndrome is a rare autosomal dominant disorder clinically characterized by parkinsonism with depression/apathy, weight loss, and central hypoventilation. Eight mutations in DCTN1 gene have been reported. A novel disease model is required because the detailed pathogenesis remains unclear. ⋯ TH-positive neurons from Perry syndrome iPSCs recapitulated an aspect of the disease phenotype of Perry syndrome.
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Parkinsonism Relat. Disord. · Jul 2016
Case ReportsHypertrophic olivary degeneration: A clinico-radiologic study.
The frequency and causes of hypertrophic olivary degeneration (HOD) are unknown. We compared the clinical and radiological characteristics of unilateral HOD and bilateral HOD. ⋯ Our study showed that bilateral HOD is more common than unilateral HOD. Half of bilateral HOD cases had no obvious cause and some worsened over time. This may implicate a possible primary neurodegenerative process.