Parkinson's Plus Syndrome

Neurodegenerative diseases are increasingly recognised to be an important cause of brain disorders, particularly in late age. Associated with a wide range of pathologies, they lead to progressive loss of neurons in different regions of the nervous system. Although anhedonia is common in a variety of neurodegenerative diseases, to date it has not been extensively studied in most of these conditions. Here we review the current literature on studies assessing the association between anhedonia and neurodegenerative diseases including Parkinson's Disease, Dementia with Lewy Bodies, Parkinson's Plus Syndromes, Alzheimer's Disease, Vascular Dementia, Frontotemporal Dementia, Amyotrophic Lateral Sclerosis and Huntington's Disease...

Parkinson's Disease remains a diagnostic challenge. Misdiagnosis during life is approximately 25%. Diseases that resemble PD clinically, such as the Parkinsonianplus disorders usually have a poorer prognosis. A diagnostic biomarker is needed to differentiate PD from PPS. Geographical differences in PD prevalence, genetics and environmental factors may suggest a different pathogenesis of PD in Africa which may affect metabolic changes seen on 18F-FDG-PET. We investigated the utility of 18FFDG-PET in differentiating PD from PPS in a real-life clinical setting...

BACKGROUND: Rare cases of coexisting multiple sclerosis and parkinsonism have been reported in the literature. However, the true prevalence, clinical characteristics, and causal relation between the two entities have not been systematically evaluated. OBJECTIVE: To evaluate the prevalence of parkinsonism in patients with multiple sclerosis and examine the causal relationship, if any. METHODS: Consecutive patients referred to the multiple sclerosis clinic were evaluated by a neurologist with double training in both neuroimmunology and movement disorders...

Purpose: To report a case of unilateral posterior dislocation of the cataractous lens and subluxation of the lens in the fellow eye of a patient with Parkinson-plus syndrome. Observations: A 67-year-old-man who was a known case of Parkinson-plus syndrome on long-term dopamine agonists and anti-psychotic medications demonstrated apraxia of lid opening associated with moderate-to-severe blepharospasm. He had unilateral posterior dislocation of the cataractous lens and subluxation of the lens in the fellow eye with no prior history of trauma or other known ocular risk factors...

The possible impact of viral infections on the development or pathogenesis of neurodegenerative disorders remains largely unknown. However, there have been reports associating the influenza virus pandemic and long-term infection with the Japanese encephalitis virus with the development of post-encephalitic Parkinsonism or von Economo encephalitis. In the last one year plus, there has been a worldwide pandemic arising from infection with the novel coronavirus or severe acute respiratory syndrome coronavirus (SARS-CoV)-2 which causes a severe acute respiratory syndrome that has become associated with central nervous system symptoms or complications...

Introduction: Essential tremor (ET) is a tremor syndrome characterized by bilateral, upper limb action tremor. Essential tremor-plus (ET-plus) describes ET patients with additional neurologic signs. It is unknown whether there is a difference in response to treatment with ventralis intermedius nucleus deep brain stimulation (VIM DBS) in patients with ET and ET-plus. Due to potential variability in underlying etiology in ET-plus, there is a concern that ET-plus patients may have worse outcomes. The aim of this study was to identify whether patients with ET-plus have worse tremor outcomes after VIM DBS than patients with ET...

Context  Progressive supranuclear palsy (PSP) is a neurodegenerative disorder which comes under Parkinsonism plus syndrome. As this spectrum of disease has many overlapping clinical as well as imaging findings, some quantitative parameters like magnetic resonance Parkinsonism index and midbrain/pons ratio are useful to differentiate PSP from other PD patients. Aims  The study aimed to detect sensitivity and specificity of magnetic resonance Parkinsonism index in differentiating PSP from PD. Settings and Design  It was a retrospective case-control study conducted in Sri Manankula Vinayagar Medical College, Puducherry, during the period of January 2018 to June 2019...

Calcium is widely used in conjunction with vitamin D to prevent osteoporosis. The use of calcium supplementation is also promoted for its potential benefits in lowering the risk for metabolic syndromes and cancers. However, the causal link between calcium and various health outcomes remains unclear. This review focuses on the evidence from 24 Mendelian randomization (MR) studies that were designed to minimize bias from confounding and reverse causation. These MR studies evaluated the effect of lifelong genetically higher serum calcium levels on various health outcomes...

Context: Appropriate mitochondrial function and oxidative balance are critical to neuronal survival. Accumulation of reactive oxygen species leads to oxidative stress that can cause free radical damage to biomolecules of the cell components and the molecules in the cellular milieu that eventually lead to a variety of chronic diseases including neurodegenerative disorders. Mitochondrial dysfunction initiates neuronal apoptosis thereby leading to neurodegenerative diseases including Parkinson's disease (PD)...

BACKGROUND/PURPOSE: Orthostatic myoclonus (OM) is myoclonic jerks in both legs during standing. It may relate to gait unsteadiness and may be associated with various diagnoses, including neurodegenerative, systemic, and musculoskeletal diseases. Diagnosis is based on the surface electromyography (SEMG). METHODS: From January 2016 to June 2020, we retrospectively reviewed 35 patients diagnosed with OM based on the SEMG criteria and analyzed the electrophysiological data...

Corticobasal syndrome (CBS) is one of the Parkinson-plus disorders. While initially defined as a movement disorder rather than cognition, it is now known that CBS is related to various psychiatric symptoms. We describe a patient clinically diagnosed with CBS whose initial presentation was psychiatric and rather atypical. His clinical picture included psychotic depression and delusional jealousy. Misdiagnosing these syndromes may delay the initiation of the treatment and worsen the patients' condition, as well as increase the burden of the caretakers...

To delineate the impact of non-motor markers (REM sleep behavior disorder (RBD), orthostatic hypotension (OH), cardiac sympathetic denervation, hyposmia) on neuronal injury in early-stage Parkinson's disease (PD), we measured the plasma neurofilament light chain (NFL) level of PD patients and evaluated its relationship with these markers. The study population comprised a cohort of 77 patients with PD and 54 controls. OH was assessed using 5-min head-up tilt-table test. Other clinical parameters such as RBD, Unified Parkinson's Disease Rating Scale (UPDRS), cognition, Cross-Cultural Smell Identification Test (CCSIT), white matter hyperintensity (WMH), cardiac metaiodobenzylguanidine (MIBG) and striatal dopamine transporter (DAT) uptake were assessed...

INTRODUCTION: Constipation is a common and sometimes debilitating non-motor symptom of Parkinson's disease (PD) that can result in intestinal inflammation and microbial dysbiosis. The Mediterranean diet, rich in fermentable fibres and anti-inflammatory phenolic compounds, is associated with reduced risk of developing PD and slower progression of parkinsonism. The Mediterranean diet is often recommended for people with PD; however, no studies to date examine this diet as a therapeutic intervention to modulate gastrointestinal (GI) dysfunction...

Tremor is the most commonly encountered movement disorder in clinical practice. A wide range of pathologies may manifest with tremor either as a presenting or predominant symptom. Considering the marked etiological and phenomenological heterogeneity, it would be desirable to develop a classification of tremors that reflects their underlying pathophysiology. The tremor task force of the International Parkinson Disease and Movement Disorders Society has worked toward this goal and proposed a new classification system...

BACKGROUND: Telomeres are repetitive DNA sequences of TTAGGG at the ends of chromosomes. Many studies have shown that telomere shortening is associated with aging-related diseases, such as cardiovascular diseases, hypertension, diabetes, cancer, and various neurodegenerative diseases, including Alzheimer's disease, vascular dementia, Parkinson's disease, and dementia with Lewy bodies. However, changes in telomere length (TL) in patients with frontotemporal dementia (FTD) syndrome are unclear...

Corticobasal syndrome (CBS) is associated with diverse pathological substrates such as tau, prion protein, transactive response and, rarely, alpha synuclein. We report the case of a54-year-old man, who presented with asymmetric levodopa-poor-responsive parkinsonism, frontal lobe signs and behavioral changes. He was diagnosed with CBS, and postmortem analyses revealed Lewy body disease Braak stage VI without comorbid pathologies. Retrospectively, the clinical course of our patient and previous reports indicate that CBS plus mood changes and autonomic dysfunction, including reduced uptake of metaiodobenzylguanidine, are predictive factors of Lewy body pathology, even if the clinical picture is atypical...

Mood and anxiety disorders are the most common neuropsychiatric syndromes associated with Parkinson's disease (PD). The aim of our study was to estimate the prevalence of lifetime and current anxiety disorders in patients with Parkinson's Disease (PD), to explore possible distinctive neurological and psychiatric features associated with such comorbidity. One hundred patients were consecutively recruited at the Movement Disorders Section of the Neurological Outpatient Clinic of the University of Pisa. According to the MINI-Plus 5...

Objectives: The aim of this study was to evaluate the sensitivity and specificity of various outcomes of acute levodopa challenge test (ALCT) namely improvement of motor function, development of dyskinesia and intolerance; to predict the diagnosis of idiopathic Parkinson's disease (IPD) or Parkinson-plus syndrome; to predict levodopa responsiveness and levodopa-induced dyskinesia (LID) during long-term therapy. Methods: ALCT was performed on 89 patients with parkinsonism of <2 years and were followed up for 18 months...

BACKGROUND: Hashimoto's encephalopathy with serum anti-NH2 -terminal of α-enolase (NAE) antibodies occasionally displays clinical symptoms such as cerebellar ataxia and parkinsonism. We studied the frequency of anti-NAE antibodies in patients with Parkinson-plus syndrome. METHODS: We examined the positive rates of anti-NAE antibodies in 47 patients with multiple system atrophy (MSA), 29 patients with Parkinson's disease (PD), eight patients with corticobasal syndrome (CBS), and 18 patients with progressive supranuclear palsy (PSP) using conventional immunoblot analysis...

Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a combination of autonomic failure plus cerebellar syndrome and/or parkinsonism. Dysphagia is a frequent and disabling symptom in MSA and its occurrence within 5 years of motor onset is an additional diagnostic feature. Dysphagia can lead to aspiration pneumonia, a recognized cause of death in MSA. Guidelines for diagnosis and management of dysphagia in MSA are lacking. An International Consensus Conference among experts with methodological support was convened in Bologna to reach consensus statements for the diagnosis, prognosis, and treatment of dysphagia in MSA...