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Parkinsonism Relat Disord 2018 Dec 11. Epub 2018 Dec 11.

Neuroimaging Research Unit, Institute of Molecular Bioimaging and Physiology, National Research Council, Catanzaro, Italy; Neuroscience Research Centre, Magna Græcia University, Catanzaro, Italy. Electronic address:

Introduction: Several structural and functional neuroimaging studies have shown that the Supplementary Motor Area (SMA) is affected by tau pathology in patients with Progressive Supranuclear Palsy (PSP). The aim of the study was to investigate the biochemical profile of SMA in PSP patients, using proton magnetic resonance spectroscopy (H-MRS).

Methods: Sixteen PSP patients and 18 healthy controls participated in this study. Read More

Acta Neuropathol 2018 Dec 13. Epub 2018 Dec 13.

Institute of Neuroscience and Physiology, Department of Psychiatry and Neurochemistry, The Sahlgrenska Academy at University of Gothenburg, Göteborgsvägen 31, House V3/SU, 43180, Mölndal, Sweden.

Tau is an axonal microtubule-binding protein. Tau pathology in brain and increased tau concentration in the cerebrospinal fluid (CSF) are hallmarks of Alzheimer's disease (AD). Most of tau in CSF is present as fragments. Read More

Transl Psychiatry 2018 Dec 13;8(1):265. Epub 2018 Dec 13.

Department of Psychiatry, Washington University School of Medicine, 660S. Euclid Ave. Campus Box 8134, St. Louis, MO, 63110, USA.

Mutations in the microtubule-associated protein tau (MAPT) gene cause autosomal dominant frontotemporal lobar degeneration with tau inclusions (FTLD-tau). MAPT p.R406W carriers present clinically with progressive memory loss and neuropathologically with neuronal and glial tauopathy. Read More

J Neurol 2018 Dec 12. Epub 2018 Dec 12.

Department of Neurology, Institute of Psychological Medicine and Clinical Neurosciences, Cardiff University, Cardiff, CF14 4XW, UK.

Ann Indian Acad Neurol 2018 Oct-Dec;21(4):345-346

Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea.

JAMA Neurol 2018 Dec 3. Epub 2018 Dec 3.

Department of Psychiatry and Neurochemistry, The Sahlgrenska Academy, University of Gothenburg, Mölndal, Sweden.

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.

Design, Setting, And Participants: In this case-control study, we investigated NFL levels in CSF obtained from controls and patients with several neurodegenerative diseases. Read More

J Neurol 2018 Dec 1. Epub 2018 Dec 1.

Neurologische Klinik und Poliklinik, Ludwig Maximilians Universität München, Munich, Germany.

The applause sign, i.e., the inability to execute the same amount of claps as performed by the examiner, was originally reported as a sign specific for progressive supranuclear palsy (PSP). Read More

Parkinsonism Relat Disord 2018 Nov 20. Epub 2018 Nov 20.

Department of Neurology, Kyoto Prefectural University of Medicine, Kyoto, 602-0841, Japan; Department of Molecular Pathobiology of Brain Diseases, Kyoto Prefectural University of Medicine, Kyoto, 602-0841, Japan. Electronic address:

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). Read More

Parkinsonism Relat Disord 2018 Nov 5. Epub 2018 Nov 5.

Center for Neurological Restoration, Cleveland Clinic, Cleveland, OH, USA.

The proper diagnosis of parkinsonian disorders usually involves three steps: identifying core features of parkinsonism; excluding other causes; and collating supportive evidence based on clinical signs or investigations. While the recognition of cardinal parkinsonian features is usually straightforward, the appreciation of clinical features suggestive of specific parkinsonian disorders can be challenging, and often requires greater experience and skills. In this review, we outline the clinical features that are relevant to the differential diagnosis of common neurodegenerative parkinsonian disorders, including Parkinson's disease, multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration. Read More

J Int Neuropsychol Soc 2018 Dec 3:1-9. Epub 2018 Dec 3.

4Clinical Memory Research Unit, Department of Clinical Sciences Malmö,Lund University,Lund,Sweden.

Objectives: The aim of this study was to assess the psychometric properties of a Swedish version of the Hayling test (HT-S) and its clinical utility in a group of patients with different frontotemporal dementia (FTD) syndromes. Early diagnosis of FTD is a challenge and requires a broad arsenal of assessment methods, neuropsychological tests not the least. The Hayling test assesses executive functions including initiation, efficiency and response inhibition. Read More

Parkinsonism Relat Disord 2018 Nov 26. Epub 2018 Nov 26.

Department of Neurology, University of Alabama at Birmingham, Birmingham, AL, USA; Alzheimer's Disease Center, University of Alabama at Birmingham, Birmingham, AL, USA; Evelyn F. McKnight Brain Institute, University of Alabama at Birmingham, Birmingham, AL, USA; Center for Neurodegeneration and Experimental Therapeutics, University of Alabama at Birmingham, Birmingham, AL, USA.

Introduction: Cognitive dysfunction is common in progressive supranuclear palsy (PSP) but its effect on medical decision-making has not been well studied. To address this gap in the research literature, we compared the medical decision-making capacity of patients with PSP to groups of patients with other neurodegenerative disorders. We also investigated the cognitive correlates of medical decision-making in our PSP sample. Read More

Mov Disord 2018 Nov 28. Epub 2018 Nov 28.

Sorbonne University, Univ. Pierre & Marie Curie Paris 06, Cnrs, Inserm, AP-HP Pitié-Salpêtrière hospital, Brain and Spinal Cord Institute, Paris, France.

Background: Deep brain stimulation of the pedunculopontine nucleus has been performed to treat dopamine-resistant gait and balance disorders in patients with degenerative diseases. The outcomes, however, are variable, which may be the result of the lack of a well-defined anatomical target.

Objectives: The objectives of this study were to identify the main neuronal populations of the pedunculopontine and the cuneiform nuclei that compose the human mesencephalic locomotor region and to compare their 3-dimensional distribution with those found in patients with Parkinson's disease and progressive supranuclear palsy. Read More

Handb Clin Neurol 2018 ;159:195-203

Brain and Mind Centre, University of Sydney Medical School, Sydney, NSW, Australia.

Frontotemporal dementia (FTD) and related diseases are important causes of younger-onset dementia. Falls may be a source of morbidity and mortality in FTD, but remain underreported, and very few high-quality studies have been performed. In this chapter, we briefly review the clinical features of FTD and related syndromes such as motor neuron disease (MND) and atypical parkinsonian syndromes, such as progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). Read More

Hum Brain Mapp 2018 Nov 26. Epub 2018 Nov 26.

Department of Medical and Surgical Sciences, Institute of Neurology, Magna Graecia University, Catanzaro, Italy.

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by white matter (WM) changes in different supra- and infratentorial brain structures. We used track density imaging (TDI) to characterize WM microstructural alterations in patients with PSP-Richardson's Syndrome (PSP-RS). Moreover, we investigated the diagnostic utility of TDI in distinguishing patients with PSP-RS from those with Parkinson's disease and healthy controls (HC). Read More

Mov Disord 2018 Nov 23. Epub 2018 Nov 23.

Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

Background: Elevated uptake of the [ F]AV-1451 tau-PET ligand has been observed cross-sectionally in subjects with progressive supranuclear palsy (PSP). However, it is unknown how the ligand performs longitudinally in PSP. We aimed to determine how regional measures of change on [ F]AV-1451 PET perform as longitudinal biomarkers of PSP compared with the more established biomarker of rate of midbrain atrophy. Read More

Can J Neurol Sci 2018 Nov 20:1-5. Epub 2018 Nov 20.

Division of Neurology, Department of Medicine,University of Alberta,Edmonton, Alberta,Canada.

Front Neurosci 2018 2;12:798. Epub 2018 Nov 2.

Institute of Neuroimmunology, Slovak Academy of Sciences, Bratislava, Slovakia.

Neurofibrillary pathology comprised of pathological tau protein is closely tied to a range of neurodegenerative disorders, the most common of which is Alzheimer's disease. While they are individually rarer, a range of other disorders, the tauopathies (including Pick's disease, progressive supranuclear palsy, corticobasal degeneration, primary progressive aphasia, and ∼50% of behavioral variant frontotemporal dementia cases) display pronounced underlying tau pathology. In all cases, the distribution and amount of tau pathology closely correlates with the severity and phenotype of cognitive impairment, and with the pattern and degree of brain atrophy. Read More

Dement Geriatr Cogn Disord 2018 Nov 13;46(5-6):285-297. Epub 2018 Nov 13.

Centre for Neuroscience of Speech, The University of Melbourne, Melbourne, Victoria,

Background: Frontotemporal lobar degeneration (FTLD) can result in a decline in behavior, language, and motor function. Mealtime disturbances are a common and significant outcome of FTLD. Disturbances during mealtimes can arise from dysphagia or may occur secondary to behavioral changes such as rapid eating, mealtime rigidity, and altered diet preferences. Read More

Neuropathol Appl Neurobiol 2018 Nov 13. Epub 2018 Nov 13.

Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, Queen Square, London, UK.

Frontotemporal dementias (FTDs) are clinically, genetically and pathologically heterogeneous neurodegenerative disorders that affect the frontal and anterior temporal lobes of the brain. They are relatively common causes of young-onset dementia and usually present with behavioural disturbance (behavioural variant FTD) or language impairment (primary progressive aphasia), but there is also overlap with motor neurone disease and the atypical parkinsonian disorders, corticobasal syndrome and progressive supranuclear palsy. At post mortem, neuronal inclusions containing tau, TDP-43 or infrequently FUS protein are seen in most cases. Read More

Parkinsonism Relat Disord 2018 Oct 6. Epub 2018 Oct 6.

Department of Neurology, Santa Maria University Hospital, Terni, Italy.

To establish a clinical diagnosis of a parkinsonian disorder, physicians rely on their ability to identify relevant red flags, in addition to cardinal features, to support or refute their working diagnosis in an individual patient. The term 'red flag', was originally coined in 1989 to define the presence of non-cardinal features that may raise a suspicion of multiple system atrophy (MSA), or at least suggest alternative diagnosis to Parkinson's disease (PD). Since then, the term 'red flag', has been consistently used in the literature to denote the clinical history or signs that may signal to physicians the possibility of an atypical parkinsonian disorder (APD). Read More

Int Rev Neurobiol 2018 24;142:67-148. Epub 2018 Sep 24.

Department of Neurology, Medical University Innsbruck, Innsbruck, Austria; Neuroimaging Research Core Facility, Medical University Innsbruck, Innsbruck, Austria. Electronic address:

Qualitative and quantitative structural magnetic resonance imaging offer objective measures of the underlying neurodegeneration in atypical parkinsonism. Regional changes in tissue volume, signal changes and increased deposition of iron as assessed with different structural MRI techniques are surrogate markers of underlying neurodegeneration and may reflect cell loss, microglial proliferation and astroglial activation. Structural MRI has been explored as a tool to enhance diagnostic accuracy in differentiating atypical parkinsonian disorders (APDs). Read More

Int Rev Neurobiol 2018 29;142:37-65. Epub 2018 Aug 29.

University Hospital and Julius-Maximilians University, Würzburg, Germany. Electronic address:

Atypical parkinsonism is the second most common diagnosis for patients with hypokinetic movement disorders. Beside common parkinsonian symptoms (i.e. Read More

Int Rev Neurobiol 2018 8;142:3-36. Epub 2018 Oct 8.

Newcastle University Newcastle Magnetic Resonance Centre & Positron Emission Tomography Centre, Newcastle University Campus for Ageing & Vitality, Newcastle upon Tyne, United Kingdom; Department of Clinical Medicine-Positron Emission Tomography Centre, Aarhus University, Aarhus, Denmark. Electronic address:

Multiple System Atrophy, Progressive Supranuclear Palsy, and Corticobasal Degeneration are three neurodegenerative disorders characterized by parkinsonism along with involvement of other brain cortical and subcortical regions. The ante mortem diagnosis of these disorders is extremely challenging with up to a quarter of these patients being misdiagnosed, particularly in the early stages of disease. While highly specific and sensitive imaging biomarkers of individual atypical parkinsonisms have not been identified yet, molecular PET and SPECT imaging have improved our knowledge of the physiopathology and neuropathology of these disorders and are often used as supportive criteria for the differential diagnosis of these conditions. Read More

Int Rev Neurobiol 2018 25;142:149-173. Epub 2018 Oct 25.

Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy; Department of Neurology, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.

The present chapter reports the current knowledge on the use of functional MRI (fMRI) in patients with atypical parkinsonisms, including Multiple System Atrophy, Corticobasal Syndrome and Progressive Supranuclear Palsy syndrome. Both resting state functional connectivity and task-based brain activity abnormalities are reported in atypical parkinsonisms relative to healthy controls and Parkinson's disease patients. Functional alterations were observed earlier than structural damage and may help to make early diagnosis. Read More

Cortex 2018 Dec 11;109:272-278. Epub 2018 Oct 11.

Neurology Department, C. Besta Neurological Institute and Foundation (IRCCS), Milano, Italy. Electronic address:

Introduction: The behavioural variant of frontotemporal dementia (bvFTD), and the Richardson variant of progressive supranuclear palsy (PSP-RS) share several clinical signs and symptoms. Since stereotypic behaviours are fairly common in bvFTD, and are also described in other degenerative dementias including Alzheimer's disease, and parkinsonisms with dementia, we aimed to examine the extent to which stereotypies also characterise PSP-RS.

Methods: We compared 53 bvFTD patients with 40 demented PSP-RS patients, seen consecutively as outpatients at four Italian Hospitals. Read More

J Neurol Sci 2018 Oct 22;396:3-7. Epub 2018 Oct 22.

Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine, 67 Asashi-machi, Kurume 830-0011, Japan.

Background And Objectives: Meta-iodobenzylguanidine (MIBG) myocardial scintigraphy is an effective tool for distinguishing Parkinson's disease (PD) from other diseases accompanied by parkinsonism. Unlike other Parkinsonian diseases, in PD, MIBG accumulation in the heart tends to decrease. However, previous studies have reported that a decrease in MIBG accumulation also occurs in progressive supranuclear palsy (PSP). Read More

J Neuroinflammation 2018 Nov 3;15(1):305. Epub 2018 Nov 3.

Department of Neurology, Bispebjerg University Hospital, Bispebjerg Bakke 23, Copenhagen, Denmark.

Introduction: Neuroinflammation has been established to be part of the neuropathological changes in Parkinson's disease (PD) and atypical parkinsonism (APD). Activated microglia play a key role in neuroinflammation by release of cytokines. Evidence of the disparity, if any, in the neuroinflammatory response between PD and APD is sparse. Read More

Neurology 2018 Nov 31;91(22):e2045-e2056. Epub 2018 Oct 31.

From the Department of Pharmacology and Clinical Neuroscience (D.B., M.E.D., J.L., L.F.), Epidemiology and Global Health Unit, Department of Public Health and Clinical Medicine (G.G.), Department of Psychology (M.E.D.), and Department of Radiation Sciences, Diagnostic Radiology and Umeå Center for Functional Brain Imaging (S.J.M., K.R.), Umeå University; Institute of Neuroscience and Physiology (H.Z., K.B.), Department of Psychiatry and Neurochemistry, Sahlgrenska Academy at University of Gothenburg, Mölndal; Clinical Neurochemistry Laboratory (H.Z., K.B.), Sahlgrenska University Hospital, Mölndal, Sweden; Department of Molecular Neuroscience (H.Z.), University College London Institute of Neurology; and UK Dementia Research Institute at UCL (H.Z.), London, UK.

Objective: To examine mortality and associated risk factors, including possible effects of mild cognitive impairment, imaging, and CSF abnormalities, in a community-based population with incident parkinsonism and Parkinson disease.

Methods: One hundred eighty-two patients with new-onset, idiopathic parkinsonism were diagnosed from January 2004 through April 2009, in a catchment area of 142,000 inhabitants in Sweden. Patients were comprehensively investigated according to a multimodal research protocol and followed prospectively for up to 13. Read More

Acta Neuropathol 2018 Oct 29. Epub 2018 Oct 29.

Klinisches Institut für Neurologie, Medizinische Universität Wien, Vienna, Austria.

Neurol Res 2018 Oct 30:1-8. Epub 2018 Oct 30.

a Department of Radiology , Ege University Medical Faculty , Izmir , Turkey.

Objectives: To evaluate the magnetic resonance imaging (MRI)-derived parameters in differentiation of patients with progressive supranuclear palsy (PSP) from patients with Parkinson's disease (PD), multiple system atrophy (MSA), and control subjects was aimed.

Methods: Thirty-three patients [mean age, 65.21 ± 4. Read More

J Neuropathol Exp Neurol 2018 Dec;77(12):1085-1090

Queen Square Brain Bank, UCL Queen Square Institute of Neurology.

Fluorinert (perfluorocarbon) represents an inexpensive option for minimizing susceptibility artifacts in ex vivo brain MRI scanning, and provides an alternative to Fomblin. However, its impact on fixed tissue and histological analysis has not been rigorously and quantitatively validated. In this study, we excised tissue blocks from 2 brain regions (frontal pole and cerebellum) of 5 formalin-fixed specimens (2 progressive supranuclear palsy cases, 3 controls). Read More

Mov Disord Clin Pract 2018 Mar-Apr;5(2):145-148. Epub 2018 Mar 6.

Sobell Department of Motor Neuroscience and Movement Disorders University College London London UK.

Mov Disord Clin Pract 2018 Jul-Aug;5(4):406-412. Epub 2018 May 15.

Department of Neurology University Hospital of Münster Albert-Schweitzer-Campus 1, D- 48149, Münster Germany.

Background: Esophageal dysfunction is a frequent phenomenon in Parkinson's disease during all disease stages, but data about esophageal involvement in atypical parkinsonian syndromes as well as possible differences between alpha-synucleinopathies and tauopathies, including causative links to the origin of the dysfunction, are lacking so far.

Objective: To describe esophageal alternation patterns in different parkinsonian syndromes and to look for differences supporting the hypothesis of alpha-synuclein aggregation being linked to gastrointestinal impairment in parkinsonian syndromes.

Methods: We performed an analysis and comparison of esophageal high-resolution manometry examination parameters in 10 patients with Parkinson's disease, 10 patients with multiple system atrophy (both alpha-synucleinopathies), 10 patients with progressive supranuclear palsy (tauopathy), and 10 age-matched controls. Read More

Mov Disord Clin Pract 2018 Mar-Apr;5(2):141-144. Epub 2018 Mar 6.

Department of Neurology Technische Universität München Munich Germany.

http://onlinelibrary.wiley.com/journal/10. Read More

J Neurol Neurosurg Psychiatry 2018 Oct 25. Epub 2018 Oct 25.

Cognitive & Movement Disorders Clinic, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada

The frontotemporal dementia (FTD) spectrum is a heterogeneous group of neurodegenerative syndromes with overlapping clinical, molecular and pathological features, all of which challenge the design of clinical trials in these conditions. To date, no pharmacological interventions have been proven effective in significantly modifying the course of these disorders. This study critically reviews the construct and methodology of previously published randomised controlled trials (RCTs) in FTD spectrum disorders in order to identify limitations and potential reasons for negative results. Read More

Parkinsonism Relat Disord 2018 Oct 15. Epub 2018 Oct 15.

Department of Neurology, University of Luebeck, Luebeck, Germany. Electronic address:

Background: X-linked dystonia-parkinsonism (XDP) is characterized by the unique transition of dystonia to parkinsonism and striatal degeneration. Slowing of saccades on clinical examination has been taken as suggestive of a progressive supranuclear palsy (PSP) phenotype.

Objectives: To elucidate whether eye movement abnormalities in XDP patients reflect striatonigral impairment or deficits in the brainstem saccade generator as present in PSP. Read More

Neuropsychiatr Dis Treat 2018 9;14:2643-2656. Epub 2018 Oct 9.

IRCCS Neuromed, Pozzilli, Italy,

Multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD) are the most common atypical parkinsonisms. These disorders are characterized by varying combinations of autonomic, cerebellar and pyramidal system, and cognitive dysfunctions. In this paper, we reviewed the evidence available on the presence and type of neuropsychiatric disturbances in MSA, PSP, and CBD. Read More

Nat Commun 2018 10 22;9(1):4388. Epub 2018 Oct 22.

Department of Neuroscience, Mayo Clinic, Jacksonville, FL, 32224, USA.

Apolipoprotein E (APOE) ε4 allele is the strongest genetic risk factor for late-onset Alzheimer's disease mainly by modulating amyloid-β pathology. APOE ε4 is also shown to exacerbate neurodegeneration and neuroinflammation in a tau transgenic mouse model. To further evaluate the association of APOE genotype with the presence and severity of tau pathology, we express human tau via an adeno-associated virus gene delivery approach in human APOE targeted replacement mice. Read More

Neuroimage Clin 2018 4;20:1037-1043. Epub 2018 Oct 4.

Department of Radiology, Hotchkiss Brain Institute, University of Calgary, Canada. Electronic address:

Background: Parkinson's disease (PD) and progressive supranuclear palsy - Richardson's syndrome (PSP-RS) are often represented by similar clinical symptoms, which may challenge diagnostic accuracy. The objective of this study was to investigate and compare regional cerebral diffusion properties in PD and PSP-RS subjects and evaluate the use of these metrics for an automatic classification framework.

Material And Methods: Diffusion-tensor MRI datasets from 52 PD and 21 PSP-RS subjects were employed for this study. Read More

Eur J Neurosci 2018 Dec 9;48(12):3477-3497. Epub 2018 Nov 9.

Department of Pharmacology and Toxicology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA.

Progressive supranuclear palsy (PSP) is the most common atypical Parkinsonism. Although PSP shares some symptomology with Parkinson's disease (PD), PSP has a different underlying pathology characterized by tau aggregation. Furthermore, PSP sufferers respond poorly to PD medications and there are no effective alternative therapeutics. Read More

Mol Neurodegener 2018 10 11;13(1):53. Epub 2018 Oct 11.

Department of Neuroscience, Mayo Clinic Florida, Jacksonville, FL, 32224, USA.

Background: Rare coding variants ABI3_rs616338-T and PLCG2_rs72824905-G were identified as risk or protective factors, respectively, for Alzheimer's disease (AD).

Methods: We tested the association of these variants with five neurodegenerative diseases in Caucasian case-control cohorts: 2742 AD, 231 progressive supranuclear palsy (PSP), 838 Parkinson's disease (PD), 306 dementia with Lewy bodies (DLB) and 150 multiple system atrophy (MSA) vs. 3351 controls; and in an African-American AD case-control cohort (181 AD, 331 controls). Read More

Eur J Nucl Med Mol Imaging 2018 Oct 15. Epub 2018 Oct 15.

Neurodegenerative Diseases Group, Biocruces-Bizkaia Health Research Institute, Plaza de Cruces 12, CP 48903, Barakaldo, Bizkaia, Spain.

Purpose: To identify myocardial sympathetic denervation patterns suggestive of Lewy body (LB) pathology in patients with genetic and idiopathic parkinsonisms by I-metaiodobenzylguanidine (MIBG) scintigraphy.

Methods: We retrospectively analysed myocardial MIBG images acquired with a dual-head gamma camera and low-energy high-resolution collimator (LEHR) in 194 patients with suspected synucleinopathy or atypical parkinsonism, including 34 with genetic Parkinson's disease (PD; 4 PARK1, 8 PARK2 and 22 PARK8), 85 with idiopathic PD (iPD), 6 with idiopathic REM sleep behaviour disorder (iRBD), 17 with dementia with LB (DLB), 40 with multiple system atrophy (MSA) and 12 with progressive supranuclear palsy (PSP), and in 45 healthy controls. We calculated heart-to-mediastinum MIBG uptake ratios (HMR) at 15 min and 4 h (HMR4H) for the LEHR and standardized medium-energy collimators, to obtain classification accuracies and optimal cut-off values for HMR using supervised classification and ROC analyses. Read More

Neurol Sci 2018 Oct 15. Epub 2018 Oct 15.

Neuroimaging Research Unit, Institute of Molecular Bioimaging and Physiology, National Research Council, Catanzaro, Italy.

Front Neurol 2018 11;9:767. Epub 2018 Sep 11.

Neurodegeneration Imaging Group, Department of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology and Neuroscience, King's College London, London, United Kingdom.

Sleep dysfunction is recognized as a distinct clinical manifestation in movement disorders, often reported early on in the disease course. Excessive daytime sleepiness, rapid eye movement sleep behavior disorder and restless leg syndrome, amidst several others, are common sleep disturbances that often result in significant morbidity. In this article, we review the spectrum of sleep abnormalities across atypical Parkinsonian disorders including multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), as well as Parkinson's disease (PD) and Huntington's disease (HD). Read More

J Alzheimers Dis 2018 ;66(2):551-563

IRCCS Institute of Neurological Sciences of Bologna, Bellaria Hospital, Bologna, Italy.

Cerebrospinal fluid (CSF) neurofilament light chain protein (NfL) and Alzheimer's disease (AD) core biomarker levels have been evaluated in cohorts of patients with frontotemporal dementia spectrum (FTD), but the distribution of values across the different clinical syndromes and underlying proteinopathies, and the relative diagnostic accuracy appear discordant among studies. We measured CSF NfL, total (t)-tau, phosphorylated (p)-tau, and amyloid-β (Aβ)42 in healthy controls (n = 38) and subjects with a clinical, genetic, CSF biomarker-based, and/or neuropathological diagnosis of FTD (n = 141) or AD (n = 60). Sub-analyses were conducted in a proportion of subjects with definite and/or probable frontotemporal lobar degeneration with tau (FTLD-TAU) (n = 42) or TDP43 pathology (FTLD-TDP) (n = 36). Read More

Parkinsonism Relat Disord 2018 Oct 3. Epub 2018 Oct 3.

UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, CA, USA. Electronic address:

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. Read More

CNS Neurosci Ther 2018 Oct 7. Epub 2018 Oct 7.

IRCCS Istituto Centro San Giovanni di Dio Fatebenefratelli, Brescia, Italy.

Aims: Success in treating patients with atypical parkinsonian syndromes, namely progressive supranuclear palsy (PSP), cortico-basal degeneration (CBD), multiple system atrophy (MSA), Parkinson's disease with dementia (PDD), and Lewy body dementia with (LBD), remains exceedingly low. The present work overviews the most influential research literature collected on MEDLINE, ISI Web of Science, Cochrane Library, and Scopus for available treatment in atypical parkinsonisms without time restriction.

Discussion: Transdermal rotigotine, autologous mesenchymal stem cells, tideglusib, and coenzyme Q10 along with donepezil, rivastigmine, memantine, and the deep brain stimulation have shown some benefits in alleviating symptoms in APS. Read More

Neurocase 2018 Aug 6;24(4):204-212. Epub 2018 Oct 6.

b Dementia Research Centre , UCL Institute of Neurology , London , UK.

Verbal adynamia (impaired language generation, as during conversation) has not been assessed systematically in parkinsonian disorders. We addressed this in patients with Parkinson's dementia, progressive supranuclear palsy and corticobasal degeneration. All disease groups showed impaired verbal fluency and sentence generation versus healthy age-matched controls, after adjusting for general linguistic and executive factors. Read More

Curr Neurol Neurosci Rep 2018 Oct 6;18(12):86. Epub 2018 Oct 6.

Multimodal Neuroimaging Group, Department of Nuclear Medicine, University Hospital of Cologne, Cologne, Germany.

Purpose Of Review: Differential diagnosis of atypical Parkinson syndromes (APS) is difficult as clinical presentations may vary and as there is a strong overlap between disease entities. Aggregations of misfolded and hyperphosphorylated tau proteins are the common denominator of many of these diseases.

Recent Findings: Several tau targeting positron emission tomography (PET) tracers have been evaluated as possible biomarkers in APS in the recent years. Read More

Yonago Acta Med 2018 Sep 26;61(3):145-155. Epub 2018 Sep 26.

Division of Radiology, Department of Pathophysiological and Therapeutic Science, School of Medicine, Tottori University Faculty of Medicine, Yonago 683-8504, Japan.

An accurate diagnosis of Parkinson's disease (PD) is a prerequisite for therapeutic management. In spite of recent advances in the diagnosis of parkinsonian disorders, PD is misdiagnosed in between 6 and 25% of patients, even in specialized movement disorder centers. Although the gold standard for the diagnosis of PD is a neuropathological assessment, neuroimaging has been playing an important role in the differential diagnosis of PD and is used for clinical diagnostic criteria. Read More