Progressive Supranuclear Palsy, Corticobasal Degeneration, and Multiple System Atrophy
PURPOSE OF REVIEW Patients who have parkinsonian features, especially without tremor, that are not responsive to levodopa, usually have one of these three major neurodegenerative disorders rather than Parkinson disease: progressive supranuclear palsy (PSP), multiple system atrophy (MSA), or corticobasal degeneration (CBD). Each of these disorders eventually develops signs and symptoms that distinguish it from idiopathic Parkinson disease, but these may not be present at disease onset. Although these conditions are not generally treatable, it is still important to correctly diagnose the condition as soon as possible. RECENT FINDINGS In recent years, it has been increasingly recognized that the symptoms of these diseases do not accurately predict the pathology, and the pathology does not accurately predict the clinical syndrome. Despite this, interest has grown in treating these diseases by targeting misfolded tau (in the case of PSP and CBD) and misfolded α-synuclein (in the case of MSA). SUMMARY Knowledge of the characteristic signs and symptoms of PSP, MSA, and CBD are essential in diagnosing and managing patients who have atypical parkinsonian syndromes.
AbstractPurpose of ReviewThere is emerging evidence that music therapy and other methods using music and rhythm may meaningfully improve a broad range of symptoms in neurological and non-neurological disorders. This review highlights the findings of recent studies utilizing music and rhythm-based interventions for gait impairment, other motor symptoms, and non-motor symptoms in Parkinson disease (PD) and other movement disorders. Limitations of current studies as well as future research directions are discussed.Recent FindingsMultiple studies have demonstrated short-term benefits of rhythmic auditory stimulation on gait pa...
Abstract INTRODUCTION/OBJECTIVE: In most cases, abnormal cardiac 123I-meta-iodobenzylguanidine (MIBG) scintigraphy increases the probability of a diagnosis of Parkinson's disease (PD) in patients with parkinsonian features. In our study, we validated the additional value of 123I-MIBG scintigraphy beyond providing information on neurological findings and response to dopaminergic therapy for the diagnosis of PDin the early phase. METHODS: We investigated 77 cases of PD (Hoehn and Yahr Stages I-III) and 73 cases of atypical parkinsonian disorder (APD), including 35 patients with multiple system atrophy, 19 with ...
AbstractProgressive 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. Each participant underwent a clinical interview and standardized videofluorographic assessmen...
Conclusions: Patients with PD/PSP are known to develop urinary symptoms during illness. Clinical complaints and UDS findings do not necessarily match. UDS is required to manage urinary symptoms. Most of the patients respond to oral antimuscarinic medications along with behavioral and supportive therapy.
Track density imaging (TDI) has been proven to be a useful approach able to investigate white matter (WM) anatomical integrity in several neurodegenerative conditions, such as Parkinson's disease (PD) and classical phenotype of Progressive Supranuclear Palsy (PSP) also known as Richardson's syndrome (RS). To the best of our knowledge, no studies have assessed WM changes in PSP-predominant parkinsonism (PSP –P) patients by using a TDI approach, and no studies have explored the potential role of these changes in discriminating patients with PSP-P from those with PSP-RS and PD.
This toolkit will support systems to understand the priorities in care for people living with various progressive neurological conditions including multiple sclerosis (MS), motor neurone disease (MND), Parkinson's and the atypical Parkinsonism's of multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and coticobasal degeneration (CBD). It provides the opportunity to assess and benchmark current systems to find opportunities for improvement.
The International Parkinson Disease and Movement Disorder Society PSP study group (IPMDS-PSP) recently published new clinical diagnostic criteria for progressive supranuclear palsy (PSP). Currently, there is no data regarding the accuracy of these sets of criteria for differentiating various PSP phenotypes. We discuss the accuracy of the IPMDS-PSP criteria for differentiation of patients with the PSP- Richardson phenotype (PSP-RS) from those with the PSP-Parkinsonism (PSP-P) using data from a sample of 274 clinically diagnosed PSP patients participating in the Environmental Genetic PSP (ENGENE-PSP) case control study.
CONCLUSIONS: Combined assessment of serum αSyn and Rab35 is a better biomarker for discriminating PD patients from NC or atypical parkinsonian patients, and is a useful predictive biomarker for younger sporadic PD patients. PMID: 31591837 [PubMed]
Substantia nigra hyperechogenicity (SN+) in transcranial sonography (TCS) is frequent in Parkinson's disease (PD), while lenticular nucleus hyperechogenicity (LN+) and 3rd ventricle enlargement (3V+) are typical of Atypical Parkinsonisms (AP). However, there are no studies assessing the diagnostic yield of all TCS biomarkers in the three AP (progressive supranuclear palsy, PSP, multiple system atrophy, MSA, corticobasal degeneration, CBD). Previous references lack homogeneous criteria and data are incomprehensive.
ConclusionQSM could be used for iron quantification of nigral and extranigral structures in all degenerative parkinsonisms and should be tested longitudinally in order to identify early microscopical changes.