Parkinson's Plus Syndrome

A 68-year-old woman with positional dizziness and progressive imbalance presented for vestibular evaluation. Examination was notable for spontaneous downbeat nystagmus (DBN), horizontal and vertical gaze-evoked nystagmus (GEN) with centripetal and rebound nystagmus, and positional apogeotropic nystagmus. There was also mild-moderate slowing of saccades horizontally and vertically and poor fast phases with an optokinetic stimulus. Further consultation by a movement disorder specialist uncovered asymmetric decrementing bradykinesia and rigidity, masked facies, and a wide-based stance without camptocormia...

BACKGROUND: The locus coeruleus/subcoeruleus complex (LC/LsC) is a structure comprising melanized noradrenergic neurons. OBJECTIVE: To study the LC/LsC damage across Parkinson's disease (PD) and atypical parkinsonism in a large group of subjects. METHODS: We studied 98 healthy control subjects, 47 patients with isolated rapid eye movement sleep behavior disorder (RBD), 75 patients with PD plus RBD, 142 patients with PD without RBD, 19 patients with progressive supranuclear palsy (PSP), and 19 patients with multiple system atrophy (MSA)...

BACKGROUND: Globular glial tauopathy (GGT) has been associated with frontotemporal dementia syndromes; little is known about the clinical and imaging characteristics of GGT and how they differ from other non-globular glial 4-repeat tauopathies (N4GT) such as progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD). METHODS: For this case-control study the Mayo Clinic brain banks were queried for all cases with an autopsy-confirmed diagnosis of GGT between 1 January 2011 and 31 October 2021...

PURPOSE OF REVIEW: Essential tremor is a chronic, progressive syndrome that primarily presents with an action tremor involving the arms and hands. This article reviews the history and physical examination features pertinent for diagnosis, differential diagnoses, and treatments and approaches for optimal control of symptoms. RECENT FINDINGS: Essential tremor is a syndrome with symptoms extending beyond tremor to involve disturbances in gait, speech, cognition, and mood...

Movement disorders have been carefully clinically defined, based on clinico-pathological series; however there is often diagnostic and prognostic uncertainty, especially in early stage disease. Blood-based biomarkers for Alzheimer's disease (AD), particularly p-tau181 and p-tau217, may be useful in the movement disorder clinic, especially in identifying corticobasal syndrome due to AD pathology and in identifying Parkinson's disease (PD) patients at high risk for the future development of dementia. Serum or plasma neurofilament light (NfL) may be useful in separating Parkinson's plus syndromes (progressive supranuclear palsy-PSP, multiple system atrophy - MSA, and corticobasal syndrome-CBS) from PD...

BACKGROUND/OBJECTIVE: Neurosyphilis can be associated with a variety of clinical manifestations. There are only a few cases of neurosyphilis associated with parkinsonism-plus syndromes (PPSs) that have been reported in the literature. We describe a case of an elderly woman who presented with abnormal gait and progressive visual disturbance, probably secondary to neurosyphilis. METHODS: Literature search was performed in Embase, Google Scholar, Medline, Scielo, and ScienceDirect using a set of terms that included parkinsonism, tremor, and syphilis...

BACKGROUND: The construct of Essential Tremor plus (ET-plus) refers to patients who also have rest tremor and/or mild neurologic signs of unknown significance. It is unclear whether soft signs represent confounding factors or are useful in suspecting an alternative condition. METHODS: Using a Bayesian approach to ET-plus patients recruited in The ITAlian tremor Network (TITAN), we analyzed the probability that these patients do not have ET. RESULTS: The data of 274 ET-plus patients were extracted from the TITAN database...

Posterior cortical atrophy (PCA) is a clinico-radiological syndrome characterised by progressive decline in visual processing and other posterior cognitive functions, relatively preserved memory and language in the early stages, and atrophy of posterior brain regions. Often considered a "visual variant" of Alzheimer's disease, a number of other pathological substrates are recognised. Dementia with Lewy Bodies is the second most common neurodegenerative dementia and there is increasing recognition of presentations with little or no parkinsonism, highlighting significant under-recognition of this condition...

Background: Increased iron deposition in nigrosome as assessed by susceptibility-weighted imaging (SWI) is involved in the pathogenesis of Parkinson's disease (PD). This study investigated the effects of antiparkinson drugs on iron deposition in the nigrosome of PD patients. Methods: Based on the retrospective analysis of clinical data, alterations in iron deposition in the substantia nigra were investigated in 51 PD patients across different types of therapies and in nine Parkinson-plus syndrome patients. The Movement Disorder Society revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) Part Ⅲ/Ⅳ (UPDRS Ⅲ/Ⅳ) was utilized to evaluate motor function and complications...

OBJECTIVES: 123 I-ioflupane has been clinically applied to dopamine transporter imaging and visual interpretation assisted by region-of-interest (ROI)-based parameters. We aimed to build a multivariable model incorporating machine learning (ML) that could accurately differentiate abnormal profiles on 123 I-ioflupane images and diagnose Parkinson syndrome or disease and dementia with Lewy bodies (PS/PD/DLB). METHODS: We assessed 123 I-ioflupane images from 239 patients with suspected neurodegenerative diseases or dementia and classified them as having PS/PD/DLB or non-PS/PD/DLB...

BACKGROUND AND AIM: Hashimoto's encephalopathy (HE) is a rare clinical entity that is associated with encephalopathy with alteration of consciousness, presence of high levels of antithyroid antibodies, and exclusion of other suspected etiologies. METHODS: We describe a 60-year old lady who had been followed up for about 5 years for possible Alzheimer's disease plus Parkinson's disease and presented with akathisia overlapping a progressive encephalopathy compatible with the diagnosis of HE...

In a consensus statement, a task force of the "International Parkinson and Movement Disorder Society" (IPMDS) has recently proposed a two axes classification for tremor: axis I (clinical manifestations) and axis II (etiology). In the axis, I, the clinical features of tremor in a given patient are specified in terms of medical history, tremor characteristics, associated signs, and laboratory tests for some tremors leading to the discovery of axis 2 etiologies. Based on axis I sign and symptoms a specific clinical syndrome is diagnosed which have been categorized as isolated tremor syndrome (a syndrome consisting only of tremor) and combined tremor syndrome (consisting of tremor and other systemic or neurological signs)...

Background: Cannabidiol (CBD) is one of the main nonpsychoactive components of Cannabis sativa and may represent an alternative treatment for Restless Legs Syndrome/Willis-Ekbom Disease (RLS/WED) in patients with Parkinson's disease (PD) and REM (Rapid Eye Movement) sleep behavior disorder (RBD). Objective: Our purpose was a post hoc exploratory analysis to evaluate the CBD's efficacy to improve the severity of RLS/WED symptoms in patients with PD and RBD. Methods: A post hoc exploratory analysis of a phase II/III, a parallel, double-blind, placebo-controlled clinical trial was conducted in 18 patients with RLS/WED and PD plus RBD associated...

Background and Objectives  The loss of swallow tail sign (STS) has been studied for the diagnosis of Parkinson's disease (PD). The study aims to establish the role of STS on high-resolution 3D susceptibility-weighted images (SWI) on 3T MRI in clinically diagnosed cases of PD and compare with control population. Methods and Materials  Forty-five patients with clinically diagnosed PD and Parkinson plus syndrome (PPS) formed the study group and were compared with 45 controls without any neurological disease and normal brain magnetic resonance imaging (MRI)...

OBJECTIVES: To give an overview of the current evidence on the associations of general health conditions with masticatory performance and maximum bite force in older adults. DATA/SOURCES: Three electronic databases (Medline via PubMed, Embase via Ovid and CINAHL Plus via EBSCOhost) were searched up to September 2021 for cross-sectional studies on general health conditions and masticatory performance or maximum bite force in older adults. Methodological quality of the included studies was independently evaluated based on Joanna Briggs Institute critical appraisal checklist for analytical cross-sectional studies...

Since the initial description of Essential Tremor (ET), the entity of ET with rest tremor has proven to be a controversial concept. Some authors argued it could be a late manifestation of ET, others suggested it could be a variant of ET, yet others suggested it could represent a transitional state between ET and Parkinson's disease. The novel tremor classification has proposed the construct of ET-plus to differentiate patients with rest tremor from pure ET. However, there is no clarity of what ET-plus rest tremor represents...

BACKGROUND: There are currently no specific tests for either idiopathic Parkinson's disease or Parkinson-plus syndromes. The study aimed to investigate the diagnostic performance of features extracted from the whole brain using diffusion tensor imaging concerning parkinsonian disorders. MATERIALS AND METHODS: The retrospective data yielded 625 participants (average age: 61.4±8.2, men/women: 313/312; healthy controls/idiopathic Parkinson's disease/multiple system atrophy/progressive supranuclear palsy: 219/286/51/69) between 2008 and 2017...

Parkinsonism is a clinical syndrome presenting with bradykinesia, tremor, rigidity, and postural instability. Nonmotor symptoms have recently been included in the parkinsonian syndrome, which was traditionally associated with motor symptoms only. Various pathologically distinct and unrelated diseases have the same clinical manifestations as parkinsonism or parkinsonian syndrome. The etiologies of parkinsonism are classified as neurodegenerative diseases related to the accumulation of toxic protein molecules or diseases that are not neurodegenerative...

OBJECTIVE: To reveal the cerebral hypoperperfusion characteristics of White matter lesions (WMLs), we monitored the blood pressure (BP) fluctuation in patients with orthostatic hypotension (OH) and WMLs. METHODS: A total of 2265 syncope patients were enrolled in this retrospective study. Clinical outcomes of brain MRI or CT, tilt test and continuous electrocardiogram monitoring were reviewed. All patients were divided into two groups according to WMLs status, and the WMLs group was further classified into three subgroups according to Fazekas grade (1-3)...

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...