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Neurology AND Movement disorders [keywords]
- Neuropsychological and Brain Volume Differences in Patients with Left- and Right-Beginning Corticobasal Syndrome. [JOURNAL ARTICLE]
- PLoS One 2014; 9(10):e110326.
Corticobasal Syndrome (CBS) is a rare neurodegenerative syndrome characterized by unilaterally beginning frontoparietal and basal ganglia atrophy. The study aimed to prove the hypothesis that there are differences in hemispheric susceptibility to disease-related changes.Two groups of CBS patients with symptoms starting either on the left or right body side were investigated. Groups consisted of four patients each and were matched for sex, age and disease duration. Patient groups and a group of eight healthy age-matched controls were analyzed using deformation field morphometry and neuropsychological testing. To further characterize individual disease progression regarding brain atrophy and neuropsychological performance, two female, disease duration-matched patients differing in initially impaired body side were followed over six months.A distinct pattern of neural atrophy and neuropsychological performance was revealed for both CBS: Patients with initial right-sided impairment (r-CBS) revealed atrophy predominantly in frontoparietal areas and showed, except from apraxia, no other cognitive deficits. In contrast, patients with impairment of the left body side (l-CBS) revealed more widespread atrophy, extending from frontoparietal to orbitofrontal and temporal regions; and apraxia, perceptional and memory deficits could be found. A similar pattern of morphological and neuropsychological differences was found for the individual disease progression in l-CBS and r-CBS single cases.For similar durations of disease, volumetric grey matter loss related to CBS pathology appeared earlier and progressed faster in l-CBS than in r-CBS. Cognitive impairment in r-CBS was characterized by apraxia, and additional memory and perceptional deficits for l-CBS.
- Prevalence of Parkinson's disease in Baskale, Turkey: a population based study. [JOURNAL ARTICLE]
- Neurol Sci 2014 Oct 29.
Parkinson's disease (PD) is the second most common neurodegenerative disease, and its prevalence rate varies between 15 and 250/100.000. The data on the prevalence of PD in Turkey are limited. In this study, we aimed to estimate the prevalence of PD in Baskale, Turkey. The study area is a rural small area in the eastern part Turkey, with a population of 26.991 inhabitants. The first stage of the study was undertaken between February and October 2011. Field workers performed a door to door population screening for the cardinal symptoms of PD and identified cases were reevaluated by an experienced movement disorders specialist. In this population based study, 19 PD patients were identified in the screened population, indicating that the estimated age standardized prevalence of PD in Turkey was 202/100.000. This study is the first large population based study for identifying prevalence of PD in Turkey. Our prevalence rate is slightly lower than those of European countries, which may be caused by ethnical differences or environmental factors.
- Patients with scans without evidence of dopaminergic deficit: A long-term follow-up study. [JOURNAL ARTICLE]
- Mov Disord 2014 Oct 28.
We previously reported on a cohort of dystonic tremor and patients with scans without evidence of dopaminergic deficit (SWEDDs). We aim to report the long-term clinical and imaging follow-up of these patients.Patients with at least 5-year follow-up were included. These patients had an asymmetric arm tremor, a previous diagnosis of Parkinson's disease (PD), and a subsequent normal DaTscan. The imaging and clinical follow-up was done on the clinical basis.Sixteen patients were included. The mean gap between the first and subsequent scans was 5.4 years. Two patients (12.5%) had reduced nigrostriatal uptake on follow-up DaTscan, whereas 14 continued to have normal dopaminergic imaging.This is the longest follow up of patients with asymmetric rest tremor and normal DaT scans (SWEDDs) reported to date. We show here that only a minority of them show reduced striatonigral uptake over long term follow up.
- Child Neurology: PRRT2-associated movement disorders and differential diagnoses. [Journal Article]
- Neurology 2014 Oct 28; 83(18):1680-3.
- Genetic Movement Disorders in Patients of Jewish Ancestry. [JOURNAL ARTICLE]
- JAMA Neurol 2014 Oct 27.
Genetic diseases often cluster in different ethnic groups and may present with recognizable unique clinical manifestations.To summarize current knowledge about movement disorders overrepresented among patients of Jewish ancestry.We searched PubMed and the OMIM and Israeli National Genetic Databases for articles published from 1969 through March 31, 2014, using the search terms Parkinson's disease,movement disorders, ataxia, dystonia, chorea, and Creutzfeldt-Jakob with and Jewish. The final reference list was generated by giving priority to articles directly related to the topic, articles with the latest information, and comprehensive but relevant reviews.About one-third of patients with sporadic Parkinson disease (PD) and more than 40% of patients with familial PD of Ashkenazi Jewish descent likely carry the G2019S mutation in the LRRK2 gene, a mutation in the glucocerebrosidase (GBA) gene, or both. This finding contrasts with only a 10% frequency of these mutations in patients with PD who are of non-Jewish ancestry. A dystonia due to a TOR1A gene mutation is responsible for most early-onset autosomal dominant dystonia, and 90% of Ashkenazi Jews who develop early-onset disease have TOR1A-related dystonia. Familial Creutzfeldt-Jakob disease and cerebrotendinous xanthomatosis tend to cluster among Jews of North African descent, and Machado-Joseph disease is particularly frequent in Yemenite Jews.Genetic forms of PD are much more common in patients of Ashkenazi Jewish ancestry with sporadic and familial PD than in the non-Jewish population. The recognition of the particular movement disorder phenotype, coupled with information about the ethnic origin of the patients, may point to specific genetic testing and lead to early and correct diagnosis.
- Max Bielschowsky (1869-1940). [JOURNAL ARTICLE]
- J Neurol 2014 Oct 28.
Berlin neurologist and neurohistologist Max Bielschowsky counts among the most innovative microanatomical researchers at the beginning of the twentieth century. Although being quite underrated in the history of neurology today, Bielschowsky contributed substantially to the understanding of neurohereditary pathologies, such as Alzheimer's disease, Parkinsonism, and Huntington's chorea, as well as the assessment of structural changes in several movement disorders. Working with other leading research neurologists, such as Oskar and Cecile Vogt or Korbinian Brodmann at the newly founded Kaiser Wilhelm Institute for Brain Research in Berlin-Buch, he also pioneered neurohistological work on de- and regeneration processes in the Central Nervous System along with new morphological definitions of "nervous trauma."
- Which way is down? Positional distortion in the tilt illusion. [Journal Article]
- PLoS One 2014; 9(10):e110729.
Contextual information can have a huge impact on our sensory experience. The tilt illusion is a classic example of contextual influence exerted by an oriented surround on a target's perceived orientation. Traditionally, the tilt illusion has been described as the outcome of inhibition between cortical neurons with adjacent receptive fields and a similar preference for orientation. An alternative explanation is that tilted contexts could produce a re-calibration of the subjective frame of reference. Although the distinction is subtle, only the latter model makes clear predictions for unoriented stimuli. In the present study, we tested one such prediction by asking four naive subjects to estimate three positions (4, 6, and 8 o'clock) on an imaginary clock face within a tilted surround. To indicate their estimates, they used either an unoriented dot or a line segment, with one endpoint at fixation in the middle of the surround. The surround's tilt was randomly chosen from a set of orientations (±75°, ±65°, ±55°, ±45°, ±35°, ±25°, ±15°, ±5° with respect to vertical) across trials. Our results showed systematic biases consistent with the tilt illusion in both conditions. Biases were largest when observers attempted to estimate the 4 and 8 o'clock positions, but there was no significant difference between data gathered with the dot and data gathered with the line segment. A control experiment confirmed that biases were better accounted for by a local coordinate shift than to torsional eye movements induced by the tilted context. This finding supports the idea that tilted contexts distort perceived positions as well as perceived orientations and cannot be readily explained by lateral interactions between orientation selective cells in V1.
- Episodic and Electrical Nervous System Disorders Caused by Nonchannel Genes. [JOURNAL ARTICLE]
- Annu Rev Physiol 2014 Oct 17.
As noted in the separate introduction to this special topic section, episodic and electrical disorders can appear quite different clinically and yet share many overlapping features, including attack precipitants, therapeutic responses, natural history, and the types of genes that cause many of the genetic forms (i.e., ion channel genes). Thus, as we mapped and attempted to clone genes causing other episodic disorders, ion channels were always outstanding candidates when they mapped to the critical region of linkage in such a family. However, some of these disorders do not result from mutations in channels. This realization has opened up large and exciting new areas for the pathogenesis of these disorders. In some cases, the mutations occur in genes of unknown function or without understanding of molecular pathogenesis. Recently, emerging insights into a fascinating group of episodic movement disorders, the paroxysmal dyskinesias, and study of the causative genes and proteins are leading to the emerging concept of episodic electric disorders resulting from synaptic dysfunction. Much work remains to be done, but the field is evolving rapidly. As it does, we have come to realize that the molecular pathogenesis of electrical and episodic disorders is more complex than a scenario in which such disorders are simply due to mutations in the primary determinants of membrane excitability (channels). Expected final online publication date for the Annual Review of Physiology Volume 77 is February 10, 2015. Please see http://www.annualreviews.org/catalog/pubdates.aspx for revised estimates.
- Neuropsychiatry and Neural Cubism. [JOURNAL ARTICLE]
- Acad Med 2014 Oct 21.
The art movement known as Cubism did not represent a failure of perspective but, rather, was a movement aimed at advancing art by juxtaposing different perspectives. In this issue, Taylor and colleagues describe the current approach by neurologists and psychiatrists to patients with brain disorders as "Neural Cubism" because of the competing angles of these specialists' perspectives about these disorders. They advocate both integrated training for all residents in the two fields and a system of "nested hierarchies" to reclassify brain disorders according to their effect on levels of brain function. The unspoken premise of their article is that it is time for psychiatry and neurology to reunite.This Commentary takes the view that reuniting the long-separated specialties of neurology and psychiatry would not necessarily create better care for all patients with brain disorders but that trainees in both fields would benefit from increased training in the complementary specialty. The new Accreditation Council for Graduate Medical Education clinical neuroscience milestones for psychiatry training and psychiatry milestones for neurology training are steps in the right direction. Increasing opportunities for combined neurology-psychiatry training will help create a cadre of specialists equipped to efficiently care for complex patients within emerging accountable care organizations. Drawing from two fields in the service of understanding brain-behavior interactions increases the potential for innovation at their interface. The author concludes that the time has come to increase the neurological and neuroscience content of psychiatry training but not to unite the two fields.
- Beyond Neural Cubism: Promoting a Multidimensional View of Brain Disorders by Enhancing the Integration of Neurology and Psychiatry in Education. [JOURNAL ARTICLE]
- Acad Med 2014 Oct 21.
Cubism was an influential early-20th-century art movement characterized by angular, disjointed imagery. The two-dimensional appearance of Cubist figures and objects is created through juxtaposition of angles. The authors posit that the constrained perspectives found in Cubism may also be found in the clinical classification of brain disorders. Neurological disorders are often separated from psychiatric disorders as if they stemmed from different organ systems. Maintaining two isolated clinical disciplines fractionalizes the brain in the same way that Pablo Picasso fractionalized figures and objects in his Cubist art. This Neural Cubism perpetuates a clinical divide that does not reflect the scope and depth of neuroscience. All brain disorders are complex and multidimensional, with aberrant circuitry and resultant psychopharmacology manifesting as altered behavior, affect, mood, or cognition. Trainees should receive a multidimensional education based on modern neuroscience, not a partial education based on clinical precedent. The authors briefly outline the rationale for increasing the integration of neurology and psychiatry and discuss a nested model with which clinical neuroscientists (neurologists and psychiatrists) can approach and treat brain disorders.