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Chapter Fifteen - Secondary parkinsonism due to drugs, vascular lesions, tumors, trauma, and other insults
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Matthias HöllerhageAbstractIn addition to neurodegenerative disorders, there are many secondary forms of parkinsonism. The most common cause for secondary parkinsonism is the intake of distinct drugs. Neuroleptics and calcium channel blockers have been mainly described to induce parkinsonism, but also other drugs were suspected to cause or worsen parkinsonism. Another common cause for secondary parkinsonism are vascular lesions (i.e. vascular parkinsonism). Furthermore, also brain tumors have been described as rare causes for parkinso...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter One - How to approach a patient with parkinsonism – red flags for atypical parkinsonism
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Eoin Mulroy, Maria Stamelou, Kailash P. BhatiaAbstractParkinsonism is a clinical syndrome defined by bradykinesia plus rigidity or tremor. Though most commonly encountered in the setting of idiopathic Parkinson's disease, a number of neurodegenerative, structural, metabolic and toxic neurological disorders can result in parkinsonism. Accurately diagnosing the underlying cause of parkinsonism is of both therapeutic and prognostic relevance, especially as we enter the era of disease-modifying treatment trials for neurodegenerative disord...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Two - Classification of atypical parkinsonism per pathology versus phenotype
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Gesine Respondek, Maria Stamelou, Günter U. HöglingerAbstractThe umbrella term “atypical parkinsonism” refers to a clinical presentation with various causes, emphasizing the clinical commonality of diseases in which atypical parkinsonism can present. This term is useful for describing the phenomenology of a movement disorder and to classify patients according to their clinical presentation. In contrast to this classification per phenotype, a classification per pathology is needed when it comes to understanding the pathogenesis a...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Three - Progressive supranuclear palsy
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Nikolaos Giagkou, Günter U. Höglinger, Maria StamelouAbstractProgressive supranuclear palsy (PSP) is a neurodegenerative disease characterized pathologically by 4 repeat tau deposition in various cell types and anatomical regions. Richardson’s syndrome (RS) is the initially described and one of the clinical phenotypes associated with PSP pathology, characterized by vertical supranuclear gaze paly in particular downwards, postural instability with early falls and subcortical frontal dementia. PSP can manifest as several other clini...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Four - Corticobasal degeneration
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Gerard M. Saranza, Jennifer L. Whitwell, Gabor G. Kovacs, Anthony E. LangAbstractCorticobasal degeneration (CBD) is a rare neurodegenerative disease characterized by the predominance of pathological 4 repeat tau deposition in various cell types and anatomical regions. Corticobasal syndrome (CBS) is one of the clinical phenotypes associated with CBD pathology, manifesting as a progressive asymmetric akinetic-rigid, poorly levodopa-responsive parkinsonism, with cerebral cortical dysfunction. CBD can manifest as several clinical phenotype...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Five - Multiple system atrophy
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Alessandra Fanciulli, Iva Stankovic, Florian Krismer, Klaus Seppi, Johannes Levin, Gregor K. WenningAbstractMultiple system atrophy (MSA) is a sporadic, adult-onset, relentlessly progressive neurodegenerative disorder, clinically characterized by various combinations of autonomic failure, parkinsonism and ataxia. The neuropathological hallmark of MSA are glial cytoplasmic inclusions consisting of misfolded α-synuclein. Selective atrophy and neuronal loss in striatonigral and olivopontocerebellar systems underlie the division into two ...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Six - ‘Atypical’ Parkinson's disease – sporadic
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Kirsten E. Zeuner, Daniela BergAbstractParkinson's disease still is a clinical diagnosis. Also, the MDS Clinical Diagnostic Criteria for Parkinson's disease published in 2015 are based on clinical characteristics and were designed codifying the diagnostic process of an expert. The purpose was to support less experienced neurologists to achieve the diagnostic procedure up to the level of an expert. The criteria include both negative and positive properties. However, some features exclude patients with typical Parkinson's disease mainly ...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Seven - ‘Atypical’ Parkinson's disease – genetic
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Anne Weissbach, Christina Wittke, Meike Kasten, Christine KleinAbstractGenetic atypical Parkinson's disease (PD) describes monogenic forms of PD that resemble idiopathic PD but feature prominent atypical clinical signs and symptoms and can be sub-grouped into i) atypical monogenic forms caused by mutations in the ATP13A2, DNAJC6, FBXO7, SYNJ1, VPS13C, and DCTN genes; ii) monogenic PD more closely resembling idiopathic PD, but associated with atypical features in at least a subset of cases (SNCA-, LRRK2-, VPS35-, Parkin-, PINK1-, and DJ...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Eight - Parkinsonism in genetic and sporadic Alzheimer's disease
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Johannes LevinAbstractAlzheimer disease (AD) is a neurodegenerative disease characterized by deposition of pathologically aggregated amyloid-β in the extracellular space and pathologically aggregated tau protein in the intracellular space. Mainly affected brain areas are the temporal and the parietal lobe, which cause the classical AD phenotype consisting of increasing forgetfulness and difficulties to orientate. However, AD pathology is not restricted to these brain areas and spreads through the brain as the disease progresses, which...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Nine - Parkinsonism in frontotemporal dementias
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): James B. RoweAbstractFrontotemporal dementia is a clinically and pathologically heterogeneous group of neurodegenerative disorders, with progressive impairment of behavior and language. They can be closely related to amyotrophic lateral sclerosis, clinically and through shared genetics and similar pathology. Approximately 40% of people with frontotemporal dementia report a family history of dementia, motor neuron disease or parkinsonism, and half of these familial cases are attributed to mutations in three genes (C9orf72, MAPT and PGRN...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Ten - Parkinsonism in neurodegenerative diseases predominantly presenting with ataxia
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Matthis SynofzikAbstractThe number of molecularly defined degenerative ataxia diseases is rapidly increasing, many of them involving complex multisystemic presentations including parkinsonism. The increasing number of novel ataxia genes –with most of them being ultra-rare – often makes it difficult for clinicians and scientists to identify the molecular diagnosis underlying these ataxia-parkinsonism syndromes. Here we aim to provide an overview on the most frequent diseases and molecular causes underlying ataxia-parkinsonism, focus...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Eleven - Parkinsonism in Huntington's disease
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Ralf ReilmannAbstractHuntington's disease (HD) is usually characterized by involuntary hyperkinetic movements, called chorea. The intensity of chorea exhibits a peak in middle stages of HD and then decreases as HD progresses. In contrast, Pakinsonian signs of HD are often less appreciated. They typically progress in a fairly linear pattern over time. In fact, bradykinesia is detectable early on in premanifest gene carriers up to two decades prior to the clinical manifestation of HD symptoms using quantitative motor (Q-Motor) assessment...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Twelve - Parkinsonism in diseases predominantly presenting with dystonia
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Alessio Di Fonzo, Giulia Franco, Paolo Barone, Roberto ErroAbstractIf the presence of dystonia is a well-recognized phenomenon in disorders predominantly presenting with parkinsonism, including sporadic Parkinson Disease, the term dystonia-parkinsonism usually refers to rare conditions, often genetic, in which the severity of dystonia usually equates that of parkinsonism. At variance with parkinsonian syndromes with additional dystonia, the conditions reviewed in this chapter have usually their onset in childhood and their diagnostic w...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Thirteen - Genetic mimics of the non-genetic atypical parkinsonian disorders – the ‘atypical’ atypical
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Nikolaos Giagkou, Kailash P. Bhatia, Günter U. Höglinger, Maria StamelouAbstractWith the advent in genetics, many genetic parkinsonian conditions have been described that, in some cases, share features that resemble the widely recognized Richardson's syndrome (the commonest described phenotype of progressive supranuclear palsy pathology), corticobasal syndrome and multiple system atrophy syndromes. A positive family history, an earlier age at onset, and clinical features that are unusual for or characteristic of a certain condition,...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research

Chapter Fourteen - Parkinsonism in neurometabolic diseases
Publication date: 2019Source: International Review of Neurobiology, Volume 149Author(s): Miryam CarecchioAbstractNeurometabolic disease are a wide and variegate group of genetic disorders, mostly recessively inherited, causing an alteration of specific biochemical pathways within cells. In most cases, they are characterized by a multi-organ involvement, but some diseases specifically affect the brain resulting in progressive neurodegenerative conditions. Though rare or ultra-rare, these disorders must be considered in the differential of adult-onset parkinsonism in the presence of atypical additional elements, such as hepa...
Source: International Review of Neurobiology - November 27, 2019 Category: Neuroscience Source Type: research