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Parkinson's Plus Syndrome

Julius Huebl, Anahita Poshtiban, Christof Brücke, Sandy Siegert, Antje Bock, Henryk Koziara, Tomasz Kmiec, Rafał Rola, Tomasz Mandat, Andrea A Kühn
OBJECTIVES: Neurodegeneration with Brain Iron Accumulation type I (NBIA-I) is a rare hereditary neurodegenerative disorder with pallidal degeneration leading to disabling generalized dystonia and parkinsonism. Pallidal or subthalamic deep brain stimulation can partially alleviate motor symptoms. Disease-specific patterns of abnormally enhanced oscillatory neuronal activity recorded from the basal ganglia have been described in patients with movement disorders undergoing deep brain stimulation (DBS)...
January 25, 2019: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Carlos Guevara, José de Grazia, Pablo Baabor, Wendy Soruco
INTRODUCTION: MSA is an adult-onset, sporadic, progressive parkinsonian syndrome characterised by the presence of akinesia, cerebellar dysfunction, autonomic failure and pyramidal signs. Annualized-whole-brain atrophy rate (a-WBAR) is an informative way to quantify disease progression. In this longitudinal work we investigate the correlations of a-WBAR with clinical scales for motor impairment, autonomic disability and cognitive decline in MSA and explore how atrophy progresses within the brain...
December 14, 2018: Autonomic Neuroscience: Basic & Clinical
Jan Roediger, Carlo Alberto Artusi, Alberto Romagnolo, Pierce Boyne, Maurizio Zibetti, Leonardo Lopiano, Alberto J Espay, Alfonso Fasano, Aristide Merola
INTRODUCTION: We sought to assess the effect of subthalamic deep brain stimulation (STN DBS) on Parkinson's disease (PD)-associated postural abnormalities. METHODS: A computerized analysis of posture was used to quantify the thoracolumbar, thoracic, and cervical-occipital ventral angles, as well as the thoracolumbar and cervical-occipital lateral angles from the video-repository of three specialized movement disorder centers (n = 158 patients). Data was extracted from frames from video-recordings in the pre-surgical medication-ON (dopaminergic therapy) and post-surgical stimulation-ON/medication-ON states (STN DBS plus dopaminergic therapy)...
January 8, 2019: Parkinsonism & related Disorders
Martijn Devrome, Donatienne Van Weehaeghe, Joke De Vocht, Philip Van Damme, Koen Van Laere, Michel Koole
BACKGROUND: 18 F-FDG brain PET measures metabolic changes in neurodegenerative disorders and may discriminate between different diseases even at an early stage. The objective of this study was to classify patients with amyotrophic lateral sclerosis (ALS) and Parkinson plus syndromes (PP). To this end, different approaches were evaluated using generalized linear models and corresponding glucose metabolic brain patterns. Besides direct classification, healthy controls were also included to generate disease-specific metabolic brain patterns and to perform a classification using disease expression scores...
December 13, 2018: EJNMMI Research
Bob Olsson, Erik Portelius, Nicholas C Cullen, Åsa Sandelius, Henrik Zetterberg, Ulf Andreasson, Kina Höglund, David J Irwin, Murray Grossman, Daniel Weintraub, Alice Chen-Plotkin, David Wolk, Leo McCluskey, Lauren Elman, Leslie M Shaw, Jon B Toledo, Jennifer McBride, Pilar Hernandez-Con, Virginia M-Y Lee, John Q Trojanowski, Kaj Blennow
Importance: Neuronal and axonal destruction are hallmarks of neurodegenerative diseases, but it is difficult to estimate the extent and progress of the damage in the disease process. Objective: To investigate cerebrospinal fluid (CSF) levels of neurofilament light (NFL) protein, a marker of neuroaxonal degeneration, in control participants and patients with dementia, motor neuron disease, and parkinsonian disorders (determined by clinical criteria and autopsy), and determine its association with longitudinal cognitive decline...
December 3, 2018: JAMA Neurology
Takuma Ohmichi, Masato Mitsuhashi, Harutsugu Tatebe, Takashi Kasai, Omar M Ali El-Agnaf, Takahiko Tokuda
INTRODUCTION: There is still a substantial unmet need for blood-based biomarkers to make an objective diagnosis of Parkinson's disease (PD) and the parkinsonism-plus syndromes. This study is aimed to determine whether enumeration of brain-derived exosomes (BDEs) in plasma is informative in the diagnosis of those diseases. METHODS: We have developed a specific method to enumerate the plasma levels of neuron-derived, astrocyte-derived, and oligodendrocyte-derived exosomes (NDEs, ADEs and ODEs, respectively), and quantified them individually in patients with PD (n = 15), multiple system atrophy (MSA, n = 15), progressive supranuclear palsy (PSP, n = 7) and disease controls (n = 15)...
November 20, 2018: Parkinsonism & related Disorders
Nahid Olfati, Ali Shoeibi, Irene Litvan
Progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), multiple system atrophy (MSA), and dementia with Lewy bodies (DLB) are the four major proteinopathic neurodegenerative disorders. Currently, there are no disease modifying therapies for these disorders. However, better understanding of the etiopathogenic mechanisms of these disorders has allowed the development of novel therapeutic approaches. These mainly include strategies directed to the pathologic conformational shift, seeding and aggregation, as well as transcellular spread of the proteins that aggregate in the brain which are α-synuclein and tau...
October 3, 2018: Parkinsonism & related Disorders
Silke Nuber, Molly Rajsombath, Georgia Minakaki, Jürgen Winkler, Christian P Müller, Maria Ericsson, Barbara Caldarone, Ulf Dettmer, Dennis J Selkoe
α-Synuclein (αS) regulates vesicle exocytosis but forms insoluble deposits in Parkinson's disease (PD). Developing disease-modifying therapies requires animal models that reproduce cardinal features of PD. We recently described a previously unrecognized physiological form of αS, α-helical tetramers, and showed that familial PD-causing missense mutations shift tetramers to aggregation-prone monomers. Here, we generated mice expressing the fPD E46K mutation plus 2 homologous E→K mutations in adjacent KTKEGV motifs...
October 10, 2018: Neuron
Stéphane Thobois, Stéphane Prange, Christian Scheiber, Emmanuel Broussolle
The diagnosis of a parkinsonian syndrome based on clinical criteria remains sometimes difficult, especially at disease onset. Brain or heart molecular imaging techniques (SPECT or PET) can provide a major help to improve and speed up diagnosis, influencing treatment strategies. Presynaptic dopaminergic imaging using either [18 F]-Dopa PET or 123 I -2β-Carbomethoxy-3β-(4-Iodophenyl)- N-(3-Fluoropropyl) Nortropane ([123 I]-Ioflupane)SPECT demonstrates or rules out the presence of a dopaminergic degenerative process...
August 30, 2018: Parkinsonism & related Disorders
Katharina Niedermayr, Gerhard Pölzl, Sabine Scholl-Bürgi, Christine Fauth, Ulrich Schweigmann, Edda Haberlandt, Ursula Albrecht, Manuela Zlamy, Wolfgang Sperl, Johannes A Mayr, Daniela Karall
OBJECTIVE: In general, a mitochondrial disorder is diagnosed on the basis of symptom combinations and confirmed by genetic findings. However, patients carrying the m.3243A>G mutation in the mitochondrial tRNA leucine 1 (MT-TL1) do not always meet all the proposed criteria for the most frequently encountered mitochondrial syndrome "MELAS," an acronym for Mitochondrial Encephalomyopathy, Lactic Acidosis, and at least one Stroke-like episode. We here present various phenotypic characteristics of the mitochondrial mutation m...
August 21, 2018: Congenital Heart Disease
Fudong Yan, Ying Chen, Min Li, Yingqing Wang, Wenmin Zhang, Xiaochun Chen, Qinyong Ye
Lewy bodies (LB) play an essential role in the development, survival, and function maintenance of midbrain dopaminergic (DA) neurons in Parkinson disease (PD). Alpha-synuclein (α-synuclein) is the major component of Lewy bodies and is a potential target for Parkinson's disease (PD) therapies. α-synuclein can be detected in the gastrointestinal (GI) nervous system, but whether there is any association between altered α-synuclein expression in the GI nervous system and the onset of PD is not known. The answer to this question presents the opportunity for a promising biomarker in the pre-clinical diagnosis of PD...
July 2018: Medicine (Baltimore)
Woong-Woo Lee, Beomseok Jeon, Ryul Kim
Previously, we defined DRD as a syndrome of selective nigrostriatal dopamine deficiency caused by genetic defects in the dopamine synthetic pathway without nigral cell loss. DRD-plus also has the same etiologic background with DRD, but DRD-plus patients have more severe features that are not seen in DRD because of the severity of the genetic defect. However, there have been many reports of dystonia responsive to dopaminergic drugs that do not fit into DRD or DRD-plus (genetic defects in the dopamine synthetic pathway without nigral cell loss)...
July 9, 2018: Journal of Korean Medical Science
Manuela Matesan, Santhosh Gaddikeri, Katelan Longfellow, Robert Miyaoka, Saeed Elojeimy, Shana Elman, Shu-Ching Hu, Satoshi Minoshima, David Lewis
BACKGROUND AND PURPOSE: Computer-based analysis of Dopamine transporter imaging (DaTscan) can aid in image interpretation. In this study, we examined the distribution of putamen-to-caudate ratios (PCRs) obtained by using a clinically available semiquantification method. METHODS: Medical records of 32 patients (M:16) with a diagnosis of Parkinson's disease (PD) (n = 22) or Parkinson's plus syndromes (PPS) (n = 10) based on clinical follow-up, were retrospectively reviewed...
November 2018: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
Kurt A Jellinger, Amos D Korczyn
BACKGROUND: Dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD), which share many clinical, neurochemical, and morphological features, have been incorporated into DSM-5 as two separate entities of major neurocognitive disorders with Lewy bodies. Despite clinical overlap, their diagnosis is based on an arbitrary distinction concerning the time of onset of motor and cognitive symptoms, namely as early cognitive impairment in DLB and later onset following that of motor symptoms in PDD...
March 6, 2018: BMC Medicine
Saleheddine Rekik, Francis Martin, Pauline Dodet, Stefania Redolfi, Smaranda Leu-Semenescu, Jean-Christophe Corvol, David Grabli, Isabelle Arnulf
OBJECTIVES: To determine the frequency of sleep breathing disorders in multiple systemic atrophy (MSA, combining Parkinsonism, cerebellar syndrome, and dysautonomia) and evaluate the benefit/tolerance of various modes of ventilation. METHODS: We retrospectively analyzed 45 patients with MSA having undergone a videopolysomnography. Their sleep characteristics were compared to those of 45 patients with Parkinson's disease and 45 healthy controls, matched for age and sex...
February 2018: Sleep Medicine
Jamilla Hussain, Victoria Allgar, David Oliver
BACKGROUND: The use of specific triggers has been suggested to help identify patients with progressive neurological disease who would benefit from palliative care. AIM: This study aimed to improve the evidence base for the use of triggers for patients with progressive neurological disease. DESIGN: An evaluation of palliative care services was undertaken using a retrospective case note review of the timing and presence of triggers in the last 2 years of life...
April 2018: Palliative Medicine
Bhagwant R Mittal, Apurva Sood, Jaya Shukla, Rakhee Vatsa, Priya Bhusari, Ritu Shree, Sahil Mehta, Manoj Goyal, Manish Modi
INTRODUCTION: Parkinson's disease (PD) and Parkinson plus syndromes (PPS) are neurodegenerative movement disorders caused by loss of dopamine in the basal ganglia. The diagnosis of both PD and PPS is complex as it is made solely on the basis of clinical features, with no established imaging modality to aid in the diagnosis. Technetium-99m-labeled tropane derivative (Tc-TRODAT-1) binds to the dopamine transporters present in the presynaptic membrane of the dopaminergic nerve terminal. The aim of this prospective study was to investigate the potential usefulness of Tc-TRODAT-1 imaging in the diagnosis of PD and PPS...
April 2018: Nuclear Medicine Communications
Meral E Kiziltan, Aysegul Gunduz, Melih Tutuncu, Sibel Ertan, Hulya Apaydin, Gunes Kiziltan
BACKGROUND AND OBJECTIVE: Late-onset myoclonus in the elderly is mainly related to dementia or systemic disease. In this report, we aimed to investigate the clinical and electrophysiological features of patients with late-onset myoclonus. PATIENTS AND METHOD: We retrospectively assessed the medical records of patients who were referred to our electromyography laboratory. From these records, we included all patients who had myoclonus which started after the age of 60 years and in whom it was confirmed by polymyography...
April 2018: Parkinsonism & related Disorders
C A Artusi, M Zibetti, A Romagnolo, M G Rizzone, A Merola, L Lopiano
OBJECTIVES: We sought to assess the efficacy of subthalamic nucleus deep brain stimulation (STN-DBS) in Parkinson's disease (PD)-associated trunk posture abnormalities retrospectively analyzing data from 101 patients reporting mild-to-severe trunk posture abnormalities of a cohort of 216 PD patients treated with STN-DBS at our center. METHODS: Abnormal trunk posture was rated on a scale of 0 (normal) to 4 (marked flexion with an extreme abnormality of posture) as per the grading score reported in the Unified Parkinson's Disease Rating Scale...
May 2018: Acta Neurologica Scandinavica
Kurt A Jellinger
Dementia with Lewy bodies (DLB) and Parkinson's disease-dementia (PDD), although sharing many clinical, neurochemical and morphological features, according to DSM-5, are two entities of major neurocognitive disorders with Lewy bodies of unknown etiology. Despite considerable clinical overlap, their diagnosis is based on an arbitrary distinction between the time of onset of motor and cognitive symptoms: dementia often preceding parkinsonism in DLB and onset of cognitive impairment after onset of motor symptoms in PDD...
April 2018: Journal of Neural Transmission
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