Overview of sleep disturbances and their management in Parkinson plus disorders.

Sleep disturbance is one of the commonly reported non-motor symptoms in patients with Parkinson's disease (PD) as well as in Parkinson plus disorders such as multiple system atrophy (MSA), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS). Although there is a wealth of literature on sleep disturbances in PD, the same is not robust on the Parkinson plus disorders. This article aims to comprehensively review the sleep disturbances in Parkinson plus disorders.

DCTN1-related Parkinson-plus disorder (Perry syndrome).

Dynactin-1 (DCTN1)-related Parkinson-plus disorder (Perry syndrome) is an autosomal dominant neurodegenerative disorder characterised by levodopa-resistant parkinsonism, weight loss, mood change and central hypoventilation. Ventilatory insufficiency is the predominant cause of death. It has been previously described in 87 people from 20 families with a worldwide distribution.

Locus coeruleus pathology in progressive supranuclear palsy, and its relation to disease severity.

The locus coeruleus is the major source of noradrenaline to the brain and contributes to a wide range of physiological and cognitive functions including arousal, attention, autonomic control, and adaptive behaviour. Neurodegeneration and pathological aggregation of tau protein in the locus coeruleus are early features of progressive supranuclear palsy (PSP). This pathology is proposed to contribute to the clinical expression of disease, including the PSP Richardson's syndrome.

Parkinsonism in frontotemporal dementias.

Frontotemporal 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).

Urological function and dysfunction in aging: Diagnosis and treatment.

Urinary symptoms worsen further the quality of life of persons already burdened by neurologic disease. Urinary symptoms frequently occur in the setting of neurologic diseases such as Parkinson disease and Parkinson-plus syndromes, multiple sclerosis, and stroke. Urinary incontinence is associated with increased caregiver burden and enhances the risk of institutionalization among those living with dementia.

Recognizing and treating atypical Parkinson disorders.

Though less common than Parkinson's disease (PD), the atypical Parkinson disorders such as such as dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration are increasingly recognized and important to distinguish from PD. Atypical or "Parkinson-plus" disorders are multisystem disorders and generally progress more rapidly and respond poorly to current therapies compared to PD. Recent advances in our understanding of the pathophysiology of these disorders, however, have generated new interest in the development of novel diagnostics and disease-modifying therapeutics aimed at identifying and treating these disorders.

Dysphagia in Progressive Supranuclear Palsy.

Progressive supranuclear palsy (PSP) is the most common Parkinson-Plus syndrome and is associated with early onset of dysphagia relative to Parkinson Disease. The current study contributes to the growing understanding of swallowing dysfunction in PSP by describing oropharyngeal swallowing characteristics in a large prospective cohort of participants with PSP employing a nationally standardized videofluoroscopy protocol and a disease severity scale developed expressly for PSP. Participants were 51 adults diagnosed with PSP.

Survival, effect measures, and impact numbers after dementia diagnosis: a matched cohort study.

Background: Knowledge on survival after diagnosis is important for all stakeholders. We aimed to estimate the survival and life expectancy after a dementia diagnosis, and to quantify the impact of dementia subtypes on mortality.

Methods: Retrospective matched cohort study using a linkage between a dementia-specific registry and two primary care electronic medical records databases.

What Predicts Hospital Admissions in Community-Dwelling People With Parkinsonism?

Objectives: Previous studies have looked at the reasons for hospital admission in people with parkinsonism (PwP), yet few have looked at factors that precipitate admission.

Methods: People with parkinsonism with a diagnosis of idiopathic Parkinson disease of Hoehn and Yahr stage III-V and those with Parkinson plus syndromes were assessed for motor and nonmotor symptoms, quality of life, and functional performance. Logistic regression was used to investigate predictors of hospital admission over the subsequent 2 years.

Social perceptual function in parkinson's disease: A meta-analysis.

Social perceptual impairment is a common presenting feature of Parkinson's disease (PD) that has the potential to contribute considerably to disease burden. The current study reports a meta-analytic integration of 79 studies which shows that, relative to controls, PD is associated with a moderate emotion recognition deficit (g = -0.57, K = 73), and that this deficit is robust and almost identical across facial and prosodic modalities.

Introduction and classical environmental risk factors for Parkinson.

Several environmental toxics are known to induce or to increase occurrence of Parkinson disease while other toxics can provoke basal ganglia necrosis and dopa resistant parkinsonism. After this introduction, the relationship between environment and parkinsonism will be illustrated by 3 short papers: interaction gene-environment, manganese induced Parkinsonism and the Caribbean Food toxins Parkinson plus syndromes.

Genome-wide survey of copy number variants finds MAPT duplications in progressive supranuclear palsy.

Background: Progressive supranuclear palsy is a neurodegenerative tauopathy manifesting clinically as a progressive akinetic-rigid syndrome. In this study, we sought to identify genetic variants influencing PSP susceptibility through a genome-wide association analysis of a cohort of well-characterized patients who had participated in the Neuroprotection and Natural History in Parkinson Plus Syndromes and Blood Brain Barrier in Parkinson Plus Syndromes studies.

Methods: We genotyped single-nucleotide polymorphisms in 283 PSP cases from the United Kingdom, Germany, and France and compared these with genotypes from 4472 controls.

A Comparison of Pain between Parkinson's Disease and Multiple System Atrophy: A Clinical Cross-Sectional Survey.

Background: Pain is frequent in Parkinson's disease (PD) and Parkinson-plus syndrome. This study aimed to assess the prevalence, characteristics, therapy (especially the effect of dopaminergic therapy), and associated symptoms of pain in Parkinson's disease and multiple system atrophy (MSA) patients.

Methods: Seventy-one PD patients, sixty-five MSA patients, and forty age-matched healthy controls were enrolled and evaluated by using the German pain questionnaire and visual analogue scale (VAS).

Self-reported urinary impairment identifies 'fast progressors' in terms of neuronal loss in multiple system atrophy.

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.

Early perfusion and dopamine transporter imaging using F-FP-CIT PET/CT in patients with parkinsonism.

Combined use of F-N-(3-fluoropropyl)-2β-carboxymethoxy-3β-(4-iodophenyl)nortropane (FP-CIT) for dopamine transporter imaging and F-fludeoxyglucose (FDG) for glucose metabolism shows good diagnostic performance for differential diagnosis of Parkinson disease (PD) and Parkinson plus syndrome (multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, and dementia with Lewy bodies). A recent study showed that F-FP-CIT positron emission tomography (PET) with early perfusion imaging is useful for the differential diagnosis of PD and Parkinson plus syndrome with lower radiation exposure, time, and cost. In this review, we summarize the advantages of using F-FP-CIT PET for perfusion and dopamine transporter imaging, as well as clinical features useful for the differential diagnosis of PD and Parkinson plus syndrome.

Glucose metabolic brain patterns to discriminate amyotrophic lateral sclerosis from Parkinson plus syndromes.

Background: 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.

Progress in the treatment of Parkinson-Plus syndromes.

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.

What a neurologist should know about PET and SPECT functional imaging for parkinsonism: A practical perspective.

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 [F]-Dopa PET or I -2β-Carbomethoxy-3β-(4-Iodophenyl)- N-(3-Fluoropropyl) Nortropane ([I]-Ioflupane)SPECT demonstrates or rules out the presence of a dopaminergic degenerative process.

Jean-Martin Charcot and Parkinson's disease: Teaching and teaching materials.

James Parkinson's 1817 seminal article was not well known in France until 1861, when Jean-Martin Charcot and his friend, Alfred Vulpian, published a detailed description in French of paralysis agitans. Their article provided clinical information to help French physicians make an accurate diagnosis by considering gait, shaking and rigidity as well as masked facies. As Charcot always had a strong desire to teach, this article describes his lessons on Parkinson's disease from 1868 to 1888, and also examines the teaching approach he used to pass on his latest findings to his students and colleagues.

99mTc-TRODAT-1 SPECT/CT imaging as a complementary biomarker in the diagnosis of parkinsonian syndromes.

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.

Myoclonus in the elderly: A retrospective analysis of clinical and electrophysiological characteristics of patients referred to an electrophysiology laboratory.

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.

Clinical Profile of Cognitive Decline in Patients with Parkinson's Disease, Progressive Supranuclear Palsy, and Multiple System Atrophy.

Background: There are very less data on the comparison between the cognitive profile in Parkinson's disease (PD) and Parkinson's-plus groups, especially in India.

Aims: The aim of this study is to compare the cognitive profile across PD, progressive supranuclear palsy (PSP), and multiple system atrophy (MSA) groups and compare them using Mini-Mental State Examination (MMSE), frontal assessment battery (FAB), and verbal fluency tests.

Settings And Design: This was a cross-sectional study.

Phenotypic Variants of Patients with Progressive Supranuclear Palsy.

Introduction: Progressive supranuclear palsy (PSP) is a commonly observed disease among Parkinson plus syndromes, and its early and accurate diagnosis is usually not possible. PSP is currently reported to have eight different subtypes. This study aims to determine the phenotypic subtypes of PSP and reveal their accompanying characteristics.

CSF biomarkers β-amyloid, tau proteins and a-synuclein in the differential diagnosis of Parkinson-plus syndromes.

Introduction: Differential diagnosis of Parkinson-plus patients (PSP, CBD, MSA) and Parkinson's disease (PD) patients is often not straightforward, particularly in atypical cases or at the initial stages of the diseases. Classic CSF biomarkers (amyloid-beta - Aβ, tau protein - τ and phosphorylated tau protein - τ) are established biomarkers in the diagnosis of Alzheimer's disease (AD). CSF a-synuclein (α-syn) has emerged as a promising biomarker in patients with Parkinsonism.

Atypical parkinsonian syndromes: a general neurologist's perspective.

The differential diagnosis of atypical parkinsonian syndromes is challenging. These severe and often rapidly progressive neurodegenerative disorders are clinically heterogeneous and show significant phenotypic overlap. Here, clinical, imaging, neuropathological and genetic features of multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration and frontotemporal lobar degeneration (FTLD) are reviewed.