Can loss of the swallow tail sign help distinguish between Parkinson Disease and the Parkinson-Plus syndromes?

Purpose: To determine if loss of the swallow tail sign (STS) can distinguish Parkinson Disease (PD) from the Parkinson-Plus syndromes.

Methods: Twenty-five patients with PD, 21 with Parkinson-Plus syndromes, and 14 control patients were included. Presence of the STS was assessed.

Imaging biomarkers in Parkinson's disease and Parkinsonian syndromes: current and emerging concepts.

Two centuries ago in 1817, James Parkinson provided the first medical description of Parkinson's disease, later refined by Jean-Martin Charcot in the mid-to-late 19th century to include the atypical parkinsonian variants (also termed, Parkinson-plus syndromes). Today, Parkinson's disease represents the second most common neurodegenerative disorder with an estimated global prevalence of over 10 million. Conversely, atypical parkinsonian syndromes encompass a group of relatively heterogeneous disorders that may share some clinical features with Parkinson's disease, but are uncommon distinct clinicopathological diseases.

Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis.

Objective: To undertake a systematic review and meta-analysis of studies that investigated prognostic factors and survival in patients with progressive supranuclear palsy (PSP) and multiple system atrophy (MSA).

Methods: Publications of at least 10 patients with a likely or confirmed diagnosis of PSP or MSA were eligible for inclusion. Methodological quality was rated using a modified version of the Quality in Prognostic Studies tool.

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Role of brain imaging for Parkinsonism T brain MRI is normal in Pakinson's disease. Brain MRI is useless when clinical presentation is typical of idiopathic Parkinson's disease. Brain MRI is the exam of choice for differentiating idiopathic Parkinson's disease and atypical parkinsonism.

Pain in Extrapyramidal Neurodegenerative Diseases.

Objective: Pain is one of the most common nonmotor symptoms of Parkinson disease (PD) and other Parkinson plus syndromes, with a major effect on quality of life. The aims of the study were to examine the prevalence and characteristics of pain in PD and other Parkinson plus syndromes and patient use and response to pain medications.

Methods: The cohort consisted of 371 patients: 300 (81%) with PD and 71 (19%) with Parkinson plus syndromes.

Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options.

There are currently no disease-modifying treatments for progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD), and no approved pharmacological or therapeutic treatments that are effective in controlling their symptoms. The use of most pharmacological treatment options are based on experience in other disorders or from non-randomized historical controls, case series, or expert opinion. Levodopa may provide some improvement in symptoms of Parkinsonism (specifically bradykinesia and rigidity) in PSP and CBD; however, evidence is conflicting and where present, benefits are often negligible and short lived.

Single-photon emission tomography.

Single-photon emission computed tomography (SPECT) is a functional nuclear imaging technique that allows visualization and quantification of different in vivo physiologic and pathologic features of brain neurobiology. It has been used for many years in diagnosis of several neurologic and psychiatric disorders. In this chapter, we discuss the current state-of-the-art of SPECT imaging of brain perfusion and dopamine transporter (DAT) imaging.

Autoimmune atypical parkinsonism - A group of treatable parkinsonism.

Background: Immunological causes of atypical parkinsonism/Parkinson plus syndromes are rare.

Objective: To study the clinical and laboratory features and treatment outcome of autoimmune atypical parkinsonism.

Methods: Retrospective case series.

Progress in unraveling the genetic etiology of Parkinson disease in a genomic era.

Parkinson disease (PD) and Parkinson-plus syndromes are genetically heterogeneous neurological diseases. Initial studies into the genetic causes of PD relied on classical molecular genetic approaches in well-documented case families. More recently, these approaches have been combined with exome sequencing and together have identified 15 causal genes.

Impaired detrusor contractility is the pathognomonic urodynamic finding of multiple system atrophy compared to idiopathic Parkinson's disease.

Introduction: To investigate differences in urodynamic parameters between two groups: Parkinson's disease (PD) and multiple system atrophy (MSA) patients.

Methods: A retrospective study was performed in patients with PD and MSA who presented to three referral centers between 2005 and 2012. Patients referred to the urology department for lower urinary tract symptoms underwent urodynamic studies.

Deep brain stimulation in multiple system atrophy mimicking idiopathic Parkinson's disease.

Deep brain stimulation (DBS) is approved for idiopathic Parkinson's disease (IPD) but has a poor evidence base in Parkinson-plus syndromes such as multiple system atrophy (MSA). We describe the clinical and neuropathological findings in a man who was initially diagnosed with IPD, in whom DBS was unsuccessful, and in whom MSA was unexpectedly diagnosed at a subsequent autopsy. This case report highlights that DBS is often unsuccessful in MSA and also demonstrates that MSA can masquerade as IPD, which may explain treatment failure in a small group of patients apparently suffering from Parkinson's disease.

Trial of Zolpidem, Eszopiclone, and Other GABA Agonists in a Patient with Progressive Supranuclear Palsy.

Progressive supranuclear palsy (PSP) is a progressive, debilitating neurodegenerative disease of the Parkinson-plus family of syndromes. Unfortunately, there are no pharmacologic treatments for this condition, as most sufferers of the classic variant respond poorly to Parkinson medications such as levodopa. Zolpidem, a gamma aminobutyric acid (GABA) agonist specific to the α-1 receptor subtype, has been reported to show improvements in symptoms of PSP patients, including motor dysfunction, dysarthria, and ocular disturbances.

Rotigotine is safe and efficacious in Atypical Parkinsonism Syndromes induced by both α-synucleinopathy and tauopathy.

Transdermal rotigotine (RTG) is a non-ergot dopamine agonist (D3>D2>D1), and is indicated for use in early and advanced Parkinson's disease (PD). RTG patch has many potential advantages due to the immediacy of onset of the therapeutic effect. Of note, intestinal absorption is not necessary and drug delivery is constant, thereby avoiding drug peaks and helping patient compliance.

Davunetide in patients with progressive supranuclear palsy: a randomised, double-blind, placebo-controlled phase 2/3 trial.

Background: In preclinical studies, davunetide promoted microtubule stability and reduced tau phosphorylation. Because progressive supranuclear palsy (PSP) is linked to tau pathology, davunetide could be a treatment for PSP. We assessed the safety and efficacy of davunetide in patients with PSP.

The value of novel MRI techniques in Parkinson-plus syndromes: diffusion tensor imaging and anatomical connectivity studies.

Conventional MRI is a well-described, highly useful tool for the differential diagnosis of degenerative parkinsonian syndromes. Nevertheless, the observed abnormalities may only appear in late-stage disease. Diffusion tensor imaging (DTI) can identify microstructural changes in brain tissue integrity and connectivity.

The applause sign in Parkinson's disease patients is related to dysexecutive syndrome.

Recent publications report that a positive applause sign is not only present in patients with neurodegenerative diseases where the subcortical structures are affected but also in patients with cortical dementia. The nature of this sign remains unknown. This study aimed to determine the frequency of the applause sign and its correlation with cognitive impairment in patients with idiopathic Parkinson's disease.

Surgical management of airway dysfunction in Parkinson's disease compared with Parkinson-plus syndromes.

Objectives: We sought to compare the laryngeal symptoms of Parkinson's disease (PD) with those of multiple system atrophy (MSA), a Parkinson-plus syndrome; to review the differences in surgical management of upper airway dysfunction between patients with PD and those with MSA; and to present a treatment algorithm for management of upper airway disorders in patients with PD and MSA.

Methods: We analyzed the airway manifestations of each disease, including clinical and physiological test results and management outcomes, in a case series of 30 patients (24 with PD and 6 with MSA).

Results: Vocal fold atrophy causing bowing with a midfold glottic gap was common in patients with PD.

Clinical utility of DaTscan™ (123I-Ioflupane Injection) in the diagnosis of Parkinsonian Syndromes.

The diagnosis of movement disorders including Parkinsonian syndromes and essential tremor is mainly clinical. The most common diagnostic errors for Parkinson's disease include misdiagnoses such as Parkinson plus syndromes and cases of essential tremor. In this article, we discuss the clinical utility of DaTscan™ (123I-Ioflupane injection) and its diagnostic value in Parkinson's disease and the other Parkinsonian syndromes.

Levodopa Responsiveness in Adult-onset Lower Limb Dystonia is Associated with the Development of Parkinson's Disease.

Background: Adult-onset primary lower limb dystonia (AOPLLD) has been reported as an early sign of Parkinson's disease (PD) or Parkinson-plus syndrome in case series. No prior systematic analysis has assessed clinical clues predicting later development of PD or Parkinson-plus syndrome.

Methods: We identified patients with AOPLLD from medical records.

Accuracy of the National Institute for Neurological Disorders and Stroke/Society for Progressive Supranuclear Palsy and neuroprotection and natural history in Parkinson plus syndromes criteria for the diagnosis of progressive supranuclear palsy.

Autopsy is the diagnostic gold standard for progressive supranuclear palsy (PSP). The National Institute of Neurological Disorders and Stroke and Society for Progressive Supranuclear Palsy (NINDS-SPSP) criteria for the clinical diagnosis of "probable" PSP are thought to possess high specificity and low sensitivity. The NINDS-SPSP criteria for "possible" PSP are considered to increase sensitivity at the expense of specificity.

[Vascular or "lower body Parkinsonism": rise and fall of a diagnosis].

Unlabelled: The "arteriosclerotic parkinsonism", which is called vascular parkinsonism (VP), was first described by Critchley'. The broad based slow gait, reduced stride lenght, start hesitation, freezing and paratonia was mentioned as "lower body parkinsonism" (LBP) which can be associated by slow speech, dysexecutive syndrome, and hand tremor of predominantly postural character. In VP the DAT-scan proved normal dopamine content of the striatum in contrast with Parkinson's disease (PD).

Serum iron, vitamin B12 and folic acid levels in Parkinson's disease.

We aimed to investigate possible associations between systemic iron metabolism deficiency and Parkinson's disease, and also to research any possible correlations between stage of the disease and vitamin B12 and folic acid levels. 33 male and 27 female patients diagnosed with idiopathic Parkinson's disease and 22 male and 20 female age- and sex-matched controls were enrolled in the study. Having the diagnosis of secondary Parkinsonism or Parkinson plus syndromes, and for the females, not being in the menopausal stage were considered as exclusion criteria.

Treatment options for tauopathies.

To date, there are no approved and established pharmacologic treatment options for tauopathies, a very heterogenous group of neuropsychiatric diseases often leading to dementia and clinically diagnosed as atypical Parkinson syndromes. Among these so-called Parkinson plus syndromes are progressive supranuclear palsy (PSP), also referred to as Steele-Richardson-Olszewski syndrome; frontotemporal dementia (FTD); and corticobasal degeneration (CBD). Available treatment strategies are based mainly on small clinical trials, miscellaneous case reports, or small case-controlled studies.

Neuroleptic malignant syndrome in a patient with corticobasal degeneration.

Parkinson's disease is a principal underlying disease of neuroleptic malignant syndrome (NMS) occurring in parkinsonian disorders, but NMS may occur in patients with progressive supranuclear palsy and multiple system atrophy. We report first patient with corticobasal degeneration (CBD) who developed NMS after abrupt reduction of antiparkinsonian medication and concurrent infection. It should be kept in mind that the prevention of infectious illness, which is common complication in parkinson-plus syndrome, is important, and dose reduction or withdrawal of anti-parkinsonian medications should be carefully performed even in the patients with CBD who are expected to be unresponsive to levodopa treatment.