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parkinson plus syndrome

Roberto Erro, Maria Stamelou
The clinical diagnosis of Parkinson's disease (PD) is centered on a specific motor syndrome that is characterized by the presence of bradykinesia, plus rest tremor, muscle rigidity, or both. Recently, novel criteria for diagnosing PD have been released that rehearse the motor syndrome as the core feature of PD. Beyond these three main symptoms, other motor features might be present in PD including gait difficulties and postural instability. Moreover, patients with PD usually develop motor complications 5-10 years into their disease...
2017: International Review of Neurobiology
Christopher Sami Oustwani, Alexander William Korutz, Malisa Siri Lester, Yasaman Kianirad, Tanya Simuni, Tarek Aref Hijaz
PURPOSE: To determine if loss of the swallow tail sign (STS) can distinguish Parkinson Disease (PD) from the Parkinson-Plus syndromes. METHODS: Twenty-five patients with PD, 21 with Parkinson-Plus syndromes, and 14 control patients were included. Presence of the STS was assessed. RESULTS: The STS was present in 79% of controls, statistically greater than the PD/Parkinson-Plus patients. There was no difference in the presence of the STS between the PD/Parkinson-Plus subgroups or when scanning at 1...
April 20, 2017: Clinical Imaging
Usman Saeed, Jordana Compagnone, Richard I Aviv, Antonio P Strafella, Sandra E Black, Anthony E Lang, Mario Masellis
Two centuries ago in 1817, James Parkinson provided the first medical description of Parkinson's disease, later refined by Jean-Martin Charcot in the mid-to-late 19th century to include the atypical parkinsonian variants (also termed, Parkinson-plus syndromes). Today, Parkinson's disease represents the second most common neurodegenerative disorder with an estimated global prevalence of over 10 million. Conversely, atypical parkinsonian syndromes encompass a group of relatively heterogeneous disorders that may share some clinical features with Parkinson's disease, but are uncommon distinct clinicopathological diseases...
2017: Translational Neurodegeneration
Stella Andrea Glasmacher, Peter Nigel Leigh, Romi Anirban Saha
OBJECTIVE: To undertake a systematic review and meta-analysis of studies that investigated prognostic factors and survival in patients with progressive supranuclear palsy (PSP) and multiple system atrophy (MSA). METHODS: Publications of at least 10 patients with a likely or confirmed diagnosis of PSP or MSA were eligible for inclusion. Methodological quality was rated using a modified version of the Quality in Prognostic Studies tool. For frequently examined prognostic factors, HRs derived by univariate and multivariate analysis were pooled in separate subgroups; other results were synthesised narratively and HRs could not be reported here...
May 2017: Journal of Neurology, Neurosurgery, and Psychiatry
Stéphane Prange, Marc Hermier, Teodor Danaila, Chloé Laurencin, Stéphane Thobois
Role of brain imaging for Parkinsonism T brain MRI is normal in Pakinson's disease. Brain MRI is useless when clinical presentation is typical of idiopathic Parkinson's disease. Brain MRI is the exam of choice for differentiating idiopathic Parkinson's disease and atypical parkinsonism. DATscan* confirms or rules out dopaminergic degeneration but does not separate idiopathic Parkinson's disease from Parkinson "plus" syndromes. FDG PET is helpful to separate idiopathic Parkinson's disease from Parkinson "plus" syndromes...
March 2017: La Presse Médicale
Sanjay Pandey, Soumya Sharma
'Meige's syndrome' is a type of cranial dystonia characterized by blepharospasm and oromandibular dystonia and can be associated with complex movement of lower facial muscles, mouth, jaw, tongue, pharyngeal and cervical muscles. Frequently, blepharospasm is the earliest clinical manifestation, which spreads over a period of time to involve other cranial and extra-cranial muscles. Common characteristics of this syndrome are well known, but their variety is wide. Different eponyms such as "Breughel syndrome", "Wood syndrome", "Blepharospasm plus", "Segmental cranial dystonia" and "Segmental cranio-cervical dystonia" have been used to describe this entity with numerous anatomical variations...
January 15, 2017: Journal of the Neurological Sciences
Carlos Guevara, Katherina Bulatova, Gareth J Barker, Guido Gonzalez, Nicolas A Crossley, Matthew J Kempton
BACKGROUND: The absence of markers for ante-mortem diagnosis of progressive supranuclear palsy (PSP), results in this disorder being commonly mistaken for other conditions, such as idiopathic Parkinson's disease (IPD). Such mistakes occur particularly in the initial stages, when "plus syndrome" has not yet clinically emerged. OBJECTIVE: To investigate the global brain volume and tissue loss in patients with PSP relative to patients with IPD and healthy controls and correlations between clinical parameters and magnetic resonance imaging (MRI)-derived brain volume estimates...
2016: Frontiers in Aging Neuroscience
Shlomit Yust-Katz, Ronly Hershkovitz, Tanya Gurevich, Ruth Djaldetti
OBJECTIVE: Pain is one of the most common nonmotor symptoms of Parkinson disease (PD) and other Parkinson plus syndromes, with a major effect on quality of life. The aims of the study were to examine the prevalence and characteristics of pain in PD and other Parkinson plus syndromes and patient use and response to pain medications. METHODS: The cohort consisted of 371 patients: 300 (81%) with PD and 71 (19%) with Parkinson plus syndromes. Data on clinical parameters and pain were collected by questionnaire...
July 2017: Clinical Journal of Pain
C Tranchant, M Anheim
Mitochondrial diseases (MIDs) are a large group of heterogeneous disorders due to mutations in either mitochondrial DNA (mtDNA) or nuclear DNA (nDNA) genes, the latter encoding proteins involved in mitochondrial function. A multisystem clinical picture that involves several organs, including both the peripheral and central nervous systems, is a common presentation of MID. Movement disorders, even isolated ones, are not rare. Cerebellar ataxia is common in myoclonic epilepsy with ragged red fibers (MERFF) due to mutations in the mitochondrial transfer RNA (tRNA) lysine gene, in Kearns-Sayre syndrome due to mtDNA deletions, in sensory ataxic neuropathy with dysarthria and ophthalmoplegia (SANDO) due to nuclear POLG1 gene mutations, and also in ARCA2, Friedreich's ataxia, SPG7, SCA28 and autosomal-recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) due to mutations in nuclear genes involved in mitochondrial morphology or function...
August 2016: Revue Neurologique
Karolien Goffin, Koen van Laere
Single-photon emission computed tomography (SPECT) is a functional nuclear imaging technique that allows visualization and quantification of different in vivo physiologic and pathologic features of brain neurobiology. It has been used for many years in diagnosis of several neurologic and psychiatric disorders. In this chapter, we discuss the current state-of-the-art of SPECT imaging of brain perfusion and dopamine transporter (DAT) imaging. Brain perfusion SPECT imaging plays an important role in the localization of the seizure onset zone in patients with refractory epilepsy...
2016: Handbook of Clinical Neurology
Michael H Smolensky, Ramon C Hermida, Alain Reinberg, Linda Sackett-Lundeen, Francesco Portaluppi
Biological processes are organized in time as innate rhythms defined by the period (τ), phase (peak [Φ] and trough time), amplitude (A, peak-trough difference) and mean level. The human time structure in its entirety is comprised of ultradian (τ < 20 h), circadian (20 h > τ < 28 h) and infradian (τ > 28 h) bioperiodicities. The circadian time structure (CTS) of human beings, which is more complicated than in lower animals, is orchestrated and staged by a brain central multioscillator system that includes a prominent pacemaker - the suprachiasmatic nuclei of the hypothalamus...
2016: Chronobiology International
Garima Shukla, Bhavna Kaul, Anupama Gupta, Vinay Goyal, Madhuri Behari
BACKGROUND: Circadian rhythm sleep disorder-advanced sleep-phase type is a relatively uncommon disorder, mostly seen among the elderly population. Impaired circadian rhythms have been reported in neurodegenerative conditions; however, there are no reports of any circadian rhythm sleep disorder among patients with Parkinsonian syndromes. We report two patients who presented with this circadian rhythm disorder, and were then diagnosed with a Parkinsonian syndrome. The cases. A 65-year-old retired man presented with history of abrupt change in sleep schedules, sleeping around 6...
September 2015: National Medical Journal of India
Emil K Gustavsson, Joanne Trinh, Ilaria Guella, Chelsea Szu-Tu, Jaskaran Khinda, Chin-Hsien Lin, Ruey-Meei Wu, Jon Stoessl, Silke Appel-Cresswell, Martin McKeown, Alex Rajput, Ali H Rajput, Maria Skaalum Petersen, Beom S Jeon, Jan O Aasly, Matthew J Farrer
INTRODUCTION: Mutations in dynactin DCTN1 (p150(glued)) have previously been linked to familial motor neuron disease or Perry syndrome (PS) consisting of depression, parkinsonism and hypoventilation. METHODS: We sequenced DCTN1 in 636 Caucasian patients with parkinsonism (Parkinson's disease and Parkinson-plus syndromes) and 508 healthy controls. Variants (MAF < 0.01) were subsequently genotyped in Caucasian (1360 cases and 1009 controls) and Asian cohorts (1046 cases and 830 controls), and the functional implications of pathogenic variants were assessed...
July 2016: Parkinsonism & related Disorders
Sudheeran Kannoth, Anandkumar Anandakkuttan, Annamma Mathai, Anuja Nirmala Sasikumar, Vivek Nambiar
BACKGROUND: Immunological causes of atypical parkinsonism/Parkinson plus syndromes are rare. OBJECTIVE: To study the clinical and laboratory features and treatment outcome of autoimmune atypical parkinsonism. METHODS: Retrospective case series. Patients with atypical parkinsonism and positive antibodies were identified retrospectively. Those who received immunotherapy (intravenous methyl prednisolone 1g daily for five days followed by mycophenylate mofetil 2g daily or azathioprine 2-3mg/kg/day) and consented for publication of non-anonymized videos were included...
March 15, 2016: Journal of the Neurological Sciences
P Gao, P-Y Zhou, G Li, G-B Zhang, P-Q Wang, J-Z Liu, F Xu, F Yang, X-X Wu
OBJECTIVE: To assess the imaging features of nigrosomes-1 in the substantia nigra through 3T MR susceptibility weighted imaging (SWI) and its disease-specific changes for the diagnosis of Parkinson's disease (PD). PATIENTS AND METHODS: A total of 116 subjects were included in this study and allocated into 3 groups: 54 patients diagnosed with PD were assigned to the PD group, 51 age- and sex-matched volunteers without PD served as the control N-PD group, and 11 clinically suspected PD patients were allocated to the undiagnosed (UD) group...
December 2015: European Review for Medical and Pharmacological Sciences
Richard M Zweig, Elizabeth A Disbrow, Vijayakumar Javalkar
Parkinsonian syndromes share clinical signs including akinesia/bradykinesia and rigidity, which are consequences of pathology involving dopaminergic substantia nigra neurons. Yet cognitive and psychiatric disturbances are common, even early in the course of disease. Executive dysfunction is often measurable in newly diagnosed Parkinson's disease. Treatment with dopaminergic medications, particularly dopamine agonists, has been associated with hallucinations and impulse control disorder. Older age, presence of APOE-4 gene, and/or other factors result in amyloid plaque deposition that, in turn, accelerates cortical Lewy body plus tau pathology, linking Dementia with Lewy Bodies and Parkinson's disease with early dementia with Alzheimer's disease...
February 2016: Neurologic Clinics
Antonio Suppa, Flavio Di Stasio, Luca Marsili, Neeraj Upadhyay, Daniele Belvisi, Antonella Conte, Nicola Modugno, Carlo Colosimo, Alfredo Berardelli
Whether the primary motor cortex (M1) contributes to the pathophysiology of corticobasal syndrome (CBS) remains unclear. In this study in patients with probable CBS, we tested whether M1 plasticity contributes to the pathophysiology of symptoms in the contralateral "less affected" limb, manifesting only parkinsonism, and in the contralateral "more affected" limb, manifesting parkinsonism plus other motor and nonmotor symptoms. In Experiment 1, we applied intermittent/continuous theta-burst stimulation (iTBS/cTBS) over the M1 contralateral to the less affected limb in 17 patients...
February 1, 2016: Journal of Neurophysiology
Richard E Gregg, Sophia H Zhou, Anne M Dubin
BACKGROUND: With increased interest in screening of young people for potential causes of sudden death, accurate automated detection of ventricular pre-excitation (VPE) or Wolff-Parkinson-White syndrome (WPW) in the pediatric resting ECG is important. Several recent studies have shown interobserver variability when reading screening ECGs and thus an accurate automated reading for this potential cause of sudden death is critical. We designed and tested an automated algorithm to detect pediatric VPE optimized for low prevalence...
January 2016: Journal of Electrocardiology
Lama M Chahine, Daniel Weintraub, Keith A Hawkins, Andrew Siderowf, Shirley Eberly, David Oakes, John Seibyl, Matthew B Stern, Kenneth Marek, Danna Jennings
OBJECTIVES: The Parkinson Associated Risk Syndrome Study identified a cohort of healthy adults with hyposmia and dopamine transporter binding reduction to characterize individuals at risk for Parkinson's disease (PD). We describe the cognitive profile of this cohort. METHODS: Individuals older than 50 y without PD were recruited. Two hundred twenty-five completed cognitive testing and were included in the final analysis. A neuropsychological test battery was administered and normative scores created for global cognition, memory, executive function/working memory, processing speed/attention, visuospatial abilities, and language domains...
January 2016: Movement Disorders: Official Journal of the Movement Disorder Society
Rikitha Menezes, Alexander Pantelyat, Izlem Izbudak, Julius Birnbaum
Patients with rheumatic diseases can present with movement and other neurodegenerative disorders. It may be underappreciated that movement and other neurodegenerative disorders can encompass a wide variety of disease entities. Such disorders are strikingly heterogeneous and lead to a wider spectrum of clinical injury than seen in Parkinson's disease. Therefore, we sought to stringently phenotype movement and other neurodegenerative disorders presenting in a case series of rheumatic disease patients. We integrated our findings with a review of the literature to understand mechanisms which may account for such a ubiquitous pattern of clinical injury...
August 2015: Medicine (Baltimore)
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