Progressive supra nuclear palsy

OBJECTIVES: Parkinson's disease (PD) and progressive supranuclear palsy (PSP) are neurodegenerative conditions that have overlapping clinical and imaging features, thus making it difficult to distinguish and diagnose PSP from PD. Therefore, in this study, we aimed to investigate the optimal value of magnetic resonance planimetry and the parkinsonism index to differentiate between PSP and PD. METHODS: In this retrospective study, we recruited a total of 84 patients (27 patients with PSP, 27 patients with PD and 27 normal controls) who underwent MRI brain examinations...

PSP and CBD are usually multi system sporadic disorders characterized by tau inclusions in neurons and glia. The clinical and neuroimaging features are different .However in some cases overlapping of features are noted. Here we present a case of a 65 years old female patient, presenting a 3 years history of insidious onset of asymmetric right upper and lower limb dystonia, followed by slowness, falls and injuries to the back, Parkinsonism, urinary incontinence and cognitive dysfunction and upward gaze palsy...

UNLABELLED: Age and gender are the important factors for brain metabolic declines in both normal aging and neurodegeneration, and the confounding effects may influence early and differential diagnosis of neurodegenerative diseases based on the [18 F]fluorodeoxyglucose positron emission tomography ([18 F]FDG PET). We aimed to explore the potential of the adjustment of age- and gender-related confounding factors on [18 F]FDG PET images in differentiation of Parkinson's disease (PD), multiple system atrophy (MSA) and progressive supra-nuclear palsy (PSP)...

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: Multiple system atrophy (MSA) is a rare, progressive, neurodegenerative disorder presenting glia pathology. Still, disease etiology and pathophysiology are unknown, but neuro-inflammation and vascular disruption may be contributing factors to the disease progression. Here, we performed an ex vivo deep proteome profiling of the prefrontal cortex of MSA patients to reveal disease-relevant molecular neuropathological processes. Observations were validated in plasma and cerebrospinal fluid (CSF) of novel cross-sectional patient cohorts...

BACKGROUND: Advances in the understanding of the pathophysiology of frontotemporal dementia (FTD) and related disorders, along with the development of novel candidate disease modifying treatments, have stimulated the need for tools to assess the efficacy of new therapies. While perfusion imaging by arterial spin labeling (ASL) is an attractive approach for longitudinal imaging biomarkers of neurodegeneration, sources of variability between sessions including arterial transit times (ATT) and fluctuations in resting perfusion can reduce its sensitivity...

In order to make a correct diagnosis of idiopathic Parkinson's disease (PD), it is essential to exclude atypical parkinsonian features, such as early dementia, fall, and autonomic dysfunction. Rheumatoid arthritis (RA), which is a systemic inflammatory disorder, although most patients present in a polyarticular manner. Still some may also present with extra-articular involvement including skin, lung, heart, and the central or peripheral nervous systems. A possible pathogenetic link between RA and PD are proposed...

Mutations in sequestosome 1 ( SQSTM1 ) gene are associated with neurodegenerative diseases, such as frontotemporal dementia and amyotrophic lateral sclerosis. Recently, mutation in SQSTM1 was also found to cause a progressive childhood-onset cerebellar ataxia. We describe here a case of progressive childhood-onset cerebellar ataxia with vertical supra nuclear gaze palsy with no family history and a normal magnetic resonance imaging (MRI) of brain. The clinical exome sequencing in this patient showed a homozygous mutation in SQSTM1 ...

The vacuolar protein sorting 35 (VPS35) is a major component of the retromer recognition core complex which regulates intracellular protein sorting and trafficking. Deficiency in VPS35 by altering APP/Aβ metabolism has been linked to late-onset Alzheimer's disease. Here we report that VPS35 is significantly reduced in Progressive Supra-nuclear Palsy and Picks' disease, two distinct primary tauopathies. In vitro studies show that overexpression of VPS35 leads to a reduction of pathological tau in neuronal cells, whereas genetic silencing of VPS35 results in its accumulation...

Background: Semi-quantitative electroencephalogram (EEG) analysis is easy to perform and has been used to differentiate dementias, as well as idiopathic and vascular Parkinson's disease. Purpose: To study whether a semi-quantitative EEG analysis can aid in distinguishing idiopathic Parkinson's disease (IPD) from atypical parkinsonian disorders (APDs), and furthermore, whether it can help to distinguish between APDs. Materials and Methods: A comprehensive retrospective review of charts was performed to include patients with parkinsonian disorders who had at least one EEG recording available...

Within South Asia, Sri Lanka represents fastest aging with 13% of the population was aged over 60's in 2011, whereas in India it was 8%. Majority of the Sri Lankan population based genetic studies have confirmed their origin on Indian mainland. As there were inadequate data on aging cytoskeletal pathologies of these two nations with their close genetic affiliations, we performed a comparison on their elderly. Autopsy brain samples of 50 individuals from Colombo, Sri Lanka (mean age 72.1 yrs ± 7.8, mean ± S...

OBJECTIVES: Cardinal extrapyramidal features of progressive supranuclear palsy (PSP) help in clinically differentiating this condition from Parkinson's disease and other Parkinsonian syndromes. However, not all extrapyramidal features may be initially present, thus posing a difficulty in early diagnosis. We studied their frequency at the time of first presentation. METHODS: Patients diagnosed clinically with PSP using the National Institute for Neurological Disorders and Society for PSP (NINDS/SPSP) criteria and seen between August 2010 and April 2013 were examined for the presence, 'presence with deviation' or absence of six extrapyramidal features: axial rigidity, symmetry, extended posture, backward falls, absence of tremors and lack of levodopa response...

The authors reviewed the diffusion tensor imaging (DTI) and tractography (DTT) of the normal brainstem and cerebellar white matter in normal volunteers, correlating it with structural magnetic resonance (MR) imaging and DTI data obtained in patients evaluated in our institution with movement disorders, including multisystem atrophy (MSA), spinocerebellar ataxia (SCA), progressive supra-nuclear palsy (PSP) and idiopathic Parkinson's disease (PD). DTI and tractography data demonstrated major white-matter fibers within the brain stem and cerebellum, including cortico-spinal tracts, transverse pontine fibers, medial lemniscus and cerebellar peduncles...

BACKGROUND: Progressive supranuclear palsy (PSP) and parkinsonian variant of multiple system atrophy (MSA-P) are clinically difficult to differentiate from idiopathic Parkinson's disease (PD), particularly in the early stages of the disease. Previous reports indicated that the olfactory function is relatively intact or slightly reduced in patients with PSP and MSA-P, suggesting that the odor stick identification test for Japanese (OSIT-J), which is a short and simple noninvasive test that is potentially useful clinically for detecting early-stage PD in Japan, may be useful in the differential diagnosis of early-stage PD from MSA-P and PSP...

While the apolipoprotein E (ApoE) epsilon 4 allele is a recognized risk factor for Alzheimer's disease (AD), an association of epsilon 4 with other neurodegenerative diseases (NDs) has not been extensively explored. We examined 51 cases of neuropathologically confirmed ND. After eliminating 18 cases exhibiting pathology sufficient to warrant an additional diagnosis of AD, three disorders characterized by tau-related cytoskeletal pathology, i.e., Pick's disease, corticobasal degeneration, and progressive supra-nuclear palsy, showed increased epsilon 4 frequencies...

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Unusual paired helical filaments (PHF) coexisting with single filaments were observed in neurofibrillary degeneration (NFD) in the globus pallidus, subthalamic nucleus, substantia nigra, and pontine tegmentum of a typical case of progressive supra-nuclear palsy (PSP). Each filament had a diameter of 10-12 nm and showed central low density and a smooth contour. The thickest portion of a pair was 22-24 nm in diameter. The periodicity of twist varied from 150 nm to 300 nm, but each PHF had regular periodicity...

The endogenous opioid peptides have for some time been implicated in the regulation of motor behavior in animals. Recently, however, there is increased evidence to suggest a role for these peptides in the control of human motor functions as well as in the pathophysiology of abnormal movement disorders. Degeneration of opioid peptide-containing neurons in the basal ganglia has been demonstrated in Parkinson's disease and Huntington's chorea, but the clinical significance of these findings is largely unknown...

Progressive supra-nuclear palsy is a neurologically debilitating disease of unknown cause and with no effective treatment. Learned helplessness, a major cause of depression in the institutionalized elderly, is a feeling of worthlessness and powerlessness resulting in a decreased incentive to try. This article provides insight into these two problems and specific nursing interventions to enhance self care.

Clinical diagnosis of Parkinson's syndrome (PS) is reasonably easy in most cases but the distinction between different variants of PS may be difficult in early cases. The correct diagnosis is not only important for counselling and management of patients but also in conducting pharmacological and epidemiological studies. There is very little critical literature on the pathological verification of the clinical diagnosis in PS. We report our 22 years experience to address that issue. Between 1968 and 1990, 65 PS patients came to autopsy...