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Neurology AND Movement disorders [keywords]
- Gender influence on selection and outcome of deep brain stimulation for Parkinson's disease. [Journal Article]
- Ann Indian Acad Neurol 2014 Jan; 17(1):66-70.
Gender differences exist in Parkinson's disease (PD), both in clinical manifestations and response to medical treatment. We investigated whether gender differences occur in the clinical characteristics of patients selected for bilateral subthalamic nucleus deep brain stimulation (STN DBS) or in the outcome when resource limits influence treatment choices made by patients.Fifty-one consecutive patients were evaluated 1 month before, and 12 months after bilateral STN DBS. All patients were rated using Unified Parkinson's Disease Rating Scale, Parkinson's Disease Quality of Life (PDQL) Scale, Addenbrooke's Cognitive Examination and Beck Depression Inventory.Pre-operative characteristics did not differ between the genders except for lower doses of drugs (P = 0.03), worse emotional scores in PDQL (P = 0.01) and worse depression (P = 0.03) in women. There was no gender difference in the surgical outcome, except a lesser reduction of dopaminergic drugs in women. Depression and quality of life (QOL) improved equally well in women and men.Bilateral STN DBS is equally efficacious in both genders as a treatment for motor complications of PD and for improving QOL. Women are likely to be undertreated because of more severe dyskinesia and may experience less emotional well-being, and could therefore potentially benefit from earlier surgical treatment.
- Cognitive impairment in multiple system atrophy: A position statement by the neuropsychology task force of the MDS multiple system atrophy (MODIMSA) study group. [JOURNAL ARTICLE]
- Mov Disord 2014 Apr 18.
Consensus diagnostic criteria for multiple system atrophy consider dementia as a nonsupporting feature, despite emerging evidence demonstrating that cognitive impairments are an integral part of the disease. Cognitive disturbances in multiple system atrophy occur across a wide spectrum from mild single domain deficits to impairments in multiple domains and even to frank dementia in some cases. Frontal-executive dysfunction is the most common presentation, while memory and visuospatial functions also may be impaired. Imaging and neuropathological findings support the concept that cognitive impairments in MSA originate from striatofrontal deafferentation, with additional contributions from intrinsic cortical degeneration and cerebellar pathology. Based on a comprehensive evidence-based review, the authors propose future avenues of research that ultimately may lead to diagnostic criteria for cognitive impairment and dementia associated with multiple system atrophy. © 2014 International Parkinson and Movement Disorder Society.
- Neuropathological features of multiple system atrophy with cognitive impairment. [JOURNAL ARTICLE]
- Mov Disord 2014 Apr 18.
Cognitive impairment (CI) is an exclusion criterion for the diagnosis of multiple system atrophy (MSA), according to the second consensus statement. This view was recently challenged by patients with pathologically confirmed MSA who were reported to have dementia. With an aim to investigate the pathological substrate of CI in MSA, quantitative assessment of the glial and neuronal cytoplasmic inclusions and semiquantitative assessment of neuronal loss in the cortical and limbic regions was performed. No differences in the severity of these MSA-related pathological findings were identified between nine MSA cases with CI and nine MSA cases with normal cognition. Alzheimer's-related pathological changes, cerebral amyloid angiopathy, and cerebrovascular disease did not differ between the two MSA groups. MSA-specific α-synuclein and secondary pathological conditions were not more severe in MSA cases with CI, suggesting that although CI may be intrinsic to the MSA disease process, further investigation into the pathological basis of cognitive impairment in MSA is warranted. © 2014 International Parkinson and Movement Disorder Society.
- Central motor conduction studies in patients with spinal cord disorders: a review. [JOURNAL ARTICLE]
- Spinal Cord 2014 Apr 22.
Study design:Topical review of the literature.Objectives:The evaluation of patients with myelopathies requires radiological investigations; however, for the correct interpretation of the neuroimaging findings, the functional assessment of corticospinal conduction is helpful or even mandatory in many conditions. The objective of this review article was to assess the utility of the motor evoked potentials (MEPs) in diagnosis and management of the most frequent spinal cord disorders.Setting:Salzburg (Austria) and Merano (Italy).Methods:A MEDLINE search was performed using following terms: 'motor evoked potentials', 'transcranial magnetic stimulation', 'central motor conduction', 'compressive myelopathy', 'spinal cord infarction', 'spinal cord injury', 'syringomyelia', 'myelitis', 'hereditary spastic paraparesis', 'subacute combined degeneration' and 'hepatic myelopathy'.Results:Central motor conduction abnormalities can be detected also in the absence of neuroradiological abnormalities-for example, in patients with subacute combined degeneration or hepatic myelopathy. In the most frequent patients with compressive myelopathies, MEPs were found to be very helpful in determining the functional significance of neuroimaging findings. MEP recording can supplement clinical examination and neuroimaging findings also in the assessment of the spinal cord injury level. In patients with spinal cord infarction, the MEP study can demonstrate spinal involvement even when radiological evidence for spinal cord damage is absent or equivocal, thus allowing an important early diagnosis.Conclusion:MEPs represent a highly sensitive and accurate diagnostic tool in many different spinal cord disorders. MEPs can also be useful in follow-up evaluation of motor function during treatment and rehabilitation.Spinal Cord advance online publication, 22 April 2014; doi:10.1038/sc.2014.48.
- Polysomnographic sleep characteristics of generally-anxious and healthy children assessed in the home environment. [Journal Article]
- J Affect Disord 2014 Jun.:79-83.
Using laboratory-based polysomnography (PSG) we recently provided evidence of significantly prolonged sleep onset latency (SOL) and reduced latency to rapid eye movement (REM) sleep among non-depressed children with generalized anxiety disorder (GAD) compared to healthy age-matched controls. In the current study we conducted unattended ambulatory PSG in a new sample of children with GAD and controls in order to examine sleeping characteristics in the home environment.Thirty-two children (ages of 7-11 years) including 16 children with primary GAD and 16 controls receiving no psychotropic medications were studied. The anxious group had a primary diagnosis of GAD without secondary mood disorders and controls were free of any medical or psychiatric diagnoses. All participants underwent structured diagnostic assessments and completed one night of home-based polysomnography (PSG).Children with GAD exhibited significantly higher sleep efficiency (SE) and fewer rapid eye movement (REM) sleep periods compared to controls. Self-reported somatic arousal during the pre-sleep period was negatively correlated with the percentage of total REM sleep among controls, but positively correlated with REM sleep percentage in the GAD group.A small sample size and one night of PSG only.Home-based PSG recording do not provide evidence of disrupted sleep patterns in children with GAD. Contextual factors that better elucidate differences between laboratory and home-based sleep findings are suggested as important directions for future research.
- CSF Aβ42 predicts early-onset dementia in Parkinson disease. [JOURNAL ARTICLE]
- Neurology 2014 Apr 18.
To test in vivo the proposal from clinicopathologic studies that β-amyloid (Aβ) pathology shortens the time to dementia in Parkinson disease (PD), and to explore the utility of CSF Aβ and related measures as early prognostic biomarkers of dementia in an incident PD cohort.We assessed a population-based incident cohort of 104 patients with PD who underwent lumbar puncture at diagnosis. We analyzed CSF concentrations of Aβ42, Aβ40, and Aβ38 using a multiplexed immunoassay with electrochemiluminescence (ECL) detection and levels of Aβ42, total tau, and phosphorylated tau using ELISA. Patients were followed prospectively for 5 years. Dementia was diagnosed according to published criteria.CSF levels of Aβ42 were significantly decreased in patients who developed dementia (n = 20, 19.2%) compared to those who did not (n = 84, 80.8%), as measured by ECL (-33%, p = 0.006) as well as ELISA (-36%, p < 0.001). No differences were observed for other markers. Low Aβ42 values predicted a substantially increased risk for subsequent dementia at high sensitivity (≥85%), with hazard ratios of 9.9 (95% confidence interval 2.3-43.5, p = 0.002) for Aβ42ECL <376 pg/mL and 7.6 (2.2-26.4, p = 0.001) for Aβ42ELISA <443 pg/mL, after adjustment for baseline age and PD-mild cognitive impairment (MCI) status. Aβ42 reductions tended to precede the onset of PD-MCI that progressed to dementia.These in vivo data support the role of Aβ pathology in the etiology and highlight the potential utility of CSF Aβ42 as an early prognostic biomarker of dementia associated with PD.
- [Functional coupling of the frontal and parietal lobes in action]. [English Abstract, Journal Article]
- Brain Nerve 2014 Apr; 66(4):451-60.
Abstract Apraxia is the inability to perform actions or move different parts of the body in the intended manner, despite normal physical capability of movement. Based on his studies, Liepmann divided apraxia into three types: ideational apraxia, ideomotor apraxia, and limb-kinetic apraxia. Clinical findings such as ideomotor apraxia in Broca's area and apraxia in patients with Parkinson's disease (PD) and parietal ataxia suggest sensorimotor integration and action control in the parietofrontal circuits. Based on studies of the mirror neuron system, disorders in gesture production could be associated with disorders of gesture understanding. We recently reported ideomotor apraxia in association with lesions in Broca's area. Broca's area is the center of speech production, and also mediates action production. Although apraxia is not a typical clinical feature of PD, varying degrees of apraxia have been reported. The dysfunction of the motor and premotor areas, which send projections to the basal ganglia, causes this apraxia. Parietal ataxia is caused by lesions in the parietal lobe, which are thought to be caused by disconnection between the cerebellum and Brodmann area 5 in the parietal lobe.
- The spatiotemporal dynamics of early attention processes: a high-resolution electroencephalographic study of N2 subcomponent sources. [JOURNAL ARTICLE]
- Neuroscience 2014 Apr 17.
The N2 subcomponents of event-related potentials are known to reflect early attentional processes. The anterior N2 may reflect conflict monitoring, whereas the posterior N2 may be involved in target detection. The aim of this study was to identify the brain areas involved in the generation of the N2 subcomponents, in order to define the spatiotemporal dynamics of these attentional processes.We recorded 128-channel electroencephalograms in 15 healthy controls performing a three-stimulus visual oddball task and identified standard-, distracter- and target-elicited N2 components. Individual N2 sources were localized using standardized-weighted-low-resolution-electromagnetic-tomography (swLORETA). Comparative analyses were performed with a non-parametric permutation technique.Common N2 generators were observed in the Brodmann area (BA) 24 of the anterior cingulate cortex. The posterior cingulate cortex and the central precuneus were more involved in distracter processing, whereas the anterior precuneus and Brodmann area 32 of the anterior cingulate cortex were target-specific.In accordance with previous demonstration of the frontoparietal cortex's critical role in attentional processes, these new data shed light on the anterior cingulate cortex's role in conflict monitoring and its interaction with other median and frontoparietal structures in early attentional processes.
- Hepatic encephalopathy is associated with slowed and delayed stimulus-associated somatosensory alpha activity. [JOURNAL ARTICLE]
- Clin Neurophysiol 2014 Mar 26.
Hepatic encephalopathy (HE) is associated with motor symptoms and attentional deficits, which are related to pathologically slowed oscillatory brain activity. Here, potential alterations of oscillatory activity in the somatosensory system were investigated.21 patients with liver cirrhosis and varying HE severity and 7 control subjects received electrical stimulation of the right median nerve while brain activity was recorded using magnetoencephalography (MEG). Oscillatory activity within the contralateral primary somatosensory cortex (S1) and its stimulus-induced modulation were analyzed as a function of disease severity.Median nerve stimuli evoked an early broadband power increase followed by suppression and then rebound of S1 alpha and beta activity. Increasing HE severity as quantified by the critical flicker frequency (CFF) was associated with a slowing of the alpha peak frequency and a delay of the alpha rebound.The present results provide the first evidence for a slowing of oscillatory activity in the somatosensory system in HE in combination with a previously unknown deficit of S1 in adjusting activation levels back to baseline.These findings advance the understanding of the manifold symptoms of HE by strengthening the theory that disease related slowing of oscillatory brain activity also affects the somatosensory system.
- Inherited manganism: The "cock-walk" gait and typical neuroimaging features. [JOURNAL ARTICLE]
- J Neurol Sci 2014 Apr 4.
Manganese (Mn) toxicity causes an extrapyramidal, parkinsonian-type movement disorder with characteristic magnetic resonance images of Mn accumulation in the basal ganglia. This letter highlights the neurological manifestations and neuroimaging features of inherited manganism (IMn), an unusual and treatable inborn error of Mn homeostasis. Early-onset dystonia with "cock-walk" gait and hyperintense signal in basal ganglia, associated to polycythemia, chronic liver disease and hypermanganesemia, promptly suggest IMn, and a genetic evaluation should be performed.