153 results match your criteria: "Parkinson-Plus Syndromes"

 Diagnosing movement disorders can be challenging owing to their similar clinical presentations with other neurodegenerative and basal ganglia disorders, like idiopathic Parkinson's disease (IPD), essential tremors (ET), vascular parkinsonism, multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). Technetium-99m labeled tropane derivative (99mTc-TRODAT-1) imaging can help in diagnosing Parkinson's disease at an early stage to help early initiation of the treatment. The current study aimed to evaluate the role of 99mTc-TRODAT-1 imaging in differentiating IPD and Parkinson-plus syndromes (PPS).

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The differential diagnosis of neurocognitive and psychiatric disorders, particularly when symptoms overlap significantly, poses a substantial challenge in clinical practice. Parkinson's disease (PD), Lewy body dementia, and catatonia are distinct conditions that can present with similar motor and cognitive symptoms, complicating accurate diagnosis and effective treatment. We report the case of a 45-year-old male patient who presented for electroconvulsive therapy (ECT) evaluation.

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Connected speech samples elicited by a picture description task are widely used in the assessment of aphasias, but it is not clear what their interpretation should focus on. Although such samples are easy to collect, analyses of them tend to be time-consuming, inconsistently conducted and impractical for non-specialist settings. Here, we analysed connected speech samples from patients with the three variants of primary progressive aphasia (semantic, svPPA = 9; logopenic, lvPPA = 9; and non-fluent, nfvPPA = 9), progressive supranuclear palsy (PSP Richardson's syndrome = 10), corticobasal syndrome (CBS = 13) and age-matched healthy controls ( = 24).

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Article Synopsis
  • Caregiver histories are valuable for diagnosing neurodegenerative diseases; the study analyzed 4952 caregiver questionnaires from 2481 participants.
  • Using advanced machine learning, researchers achieved high diagnostic accuracy (mean AUC 0.83) between different diseases, while observing changes in symptoms over time.
  • The findings stress the importance of structured caregiver reports for diagnosing dementia and suggest the Cambridge Behavioural Inventory can effectively track disease progression in clinical settings.
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While there is a link between homocysteine (Hcy), B12 and folic acid and neurodegeneration, especially in disorders like Parkinson's and Alzheimer's diseases, its role in Parkinson plus syndromes (PPS) has only been partially investigated. It appears that elevated Hcy, along with an imbalance of its essential vitamin cofactors, are both implicated in the development and progression of parkinsonian syndromes, which represent different disease pathologies, namely alpha-synucleinopathies and tauopathies. Attributing a potential pathogenetic role in hyperhomocysteinemia would be crucial in terms of improving the diagnostic and prognostic accuracy of these syndromes and also for providing a new target for possible therapeutic intervention.

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Article Synopsis
  • Progressive supranuclear palsy (PSP) is a neurodegenerative disease classified into different phenotypes, mainly PSP with Richardson's syndrome (PSP-RS) and PSP with predominant parkinsonism (PSP-P), based on clinical features.
  • The study aimed to analyze how speech disorders, particularly dysarthria, manifest differently in PSP-RS and PSP-P patients, with both groups showing similar demographics and disease severity.
  • Results indicated no significant differences in speech profiles between the two subtypes at mid-stage disease, though PSP-RS patients exhibited greater impairment in oculomotor function.
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Article Synopsis
  • A 54-year-old man experienced recurrent fainting spells (syncope) for two years, which didn't respond to medications like droxidopa and amezinium.
  • After several tests showed no clear cause, a positive Schellong test pointed to orthostatic hypotension as a likely diagnosis, along with mild cerebellar ataxia noted during neurological evaluation.
  • MRI results suggested a "cross sign" in the pons, and further imaging indicated reduced dopamine uptake, leading to a diagnosis of probable multiple system atrophy of the cerebellar type (MSA-C), prompting treatment with taltirelin hydrate.
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Monocyte to high-density lipoprotein cholesterol ratio reflects the peripheral inflammatory state in parkinsonian disorders.

Parkinsonism Relat Disord

December 2024

Department of Neurology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Gyeonggi, South Korea; Hallym Neurological Institute, Hallym University, Anyang, Gyeonggi, South Korea. Electronic address:

Article Synopsis
  • The study investigates inflammation's role in Parkinson's disease (PD) and Parkinson plus syndrome (PPS), focusing on early-stage patients' immune responses.
  • It evaluates various peripheral inflammatory markers using blood test data from 139 PD patients, 87 PPS patients, and 139 healthy controls, excluding factors that could skew inflammation results.
  • Findings show significantly higher monocyte to HDL-C (MHR) and neutrophil to HDL-C (NHR) ratios in PD and PPS patients compared to healthy controls, indicating possible inflammation-related differences in these conditions.
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Article Synopsis
  • Post-mortem studies reveal that patients who died from COVID-19 often show brainstem damage, which may result from immune responses during and after the infection.
  • Symptoms such as fatigue, breathlessness, and chest pain in post-hospitalization COVID-19 patients may be linked to these brainstem abnormalities.
  • Using advanced MRI techniques, a study found increased susceptibility in key brainstem regions of COVID-19 survivors, indicating a correlation between these changes and the severity of their illness and recovery outcomes.
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Atypical Parkinsonian Syndromes: Structural, Functional, and Molecular Imaging Features.

AJNR Am J Neuroradiol

December 2024

Neuroradiology Division (F.M., S.S., A.M.F.), Department of Radiology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Lenox Hill Hospital, New York, New York

Atypical parkinsonian syndromes, also known as Parkinson-plus syndromes, are a heterogeneous group of movement disorders, including dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), multisystem atrophy (MSA), and corticobasal degeneration (CBD). This review highlights the characteristic structural, functional, and molecular imaging features of these complex disorders. DLB typically demonstrates parieto-occipital hypometabolism with involvement of the cuneus on FDG-PET, whereas dopaminergic imaging, such as [I]-FP-CIT SPECT (DaTscan) or fluorodopa (FDOPA)-PET, can be utilized as an adjunct for diagnosis.

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Background/objective: The corticobasal syndrome (CBS) is a complex asymmetric movement disorder, with cognitive impairment. Although commonly associated with the primary 4-repeat-tauopathy of corticobasal degeneration, clinicopathological correlation is poor, and a significant proportion is due to Alzheimer's disease (AD). Synaptic loss is a pathological feature of many clinical and preclinical tauopathies.

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Background: Multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) show a high prevalence and rapid progression of dysphagia, which is associated with reduced survival. Despite this, the evidence base for gastrostomy is poor, and the optimal frequency and outcomes of this intervention are not known. We aimed to characterise the prevalence and outcomes of gastrostomy in patients with these three atypical parkinsonian disorders.

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Extended Wrist Rotation Simplified.

Cureus

February 2024

Neurological Surgery, Independent Researcher, Louisville, USA.

Extended wrist rotation provides a simple clinical measure of rigidity in movement disorders. The supinator-pronator muscles of the forearm form an agonist-antagonist pair that can be isolated for diagnosis and monitoring. Patients rarely can isolate these muscles without extraordinary training and body awareness.

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Lysosomal dysfunction plays an important role in neurodegenerative diseases, including Parkinson's disease (PD) and possibly Parkinson-plus syndromes such as progressive supranuclear palsy (PSP). This role is exemplified by the involvement of variants in the GBA1 gene, which results in a deficiency of the lysosomal enzyme glucocerebrosidase and is the most frequently identified genetic factor underlying PD worldwide. Pathogenic variants in the SMPD1 gene are a recessive cause of Niemann-Pick disease types A and B.

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Practical Overview of I-Ioflupane Imaging in Parkinsonian Syndromes.

Radiographics

February 2024

From the Department of Radiology and Radiological Science, Medical University of South Carolina, 96 Jonathan Lucas St, CSB 211N, MSC 323, Charleston, SC 29425 (M.K.M., S.E.); Department of Radiology, New York University Langone Health Long Island, New York, NY (J.W.R.); Department of Radiology, University of New Mexico, Albuquerque, NM (L.C.B.); Department of Radiology, Brooke Army Medical Center, San Antonio, Tex (K.P.B.); Department of Radiology, Eastern Virginia Medical School, Norfolk, Va (L.S.J., S.W.); Department of Radiology, University of Washington, Seattle, Wash (D.H.L.); and Departments of Medical Imaging, Medicine, and Biomedical Engineering, University of Arizona, Tucson, Ariz (P.H.K.).

Parkinsonian syndromes are a heterogeneous group of progressive neurodegenerative disorders involving the nigrostriatal dopaminergic pathway and are characterized by a wide spectrum of motor and nonmotor symptoms. These syndromes are quite common and can profoundly impact the lives of patients and their families. In addition to classic Parkinson disease, parkinsonian syndromes include multiple additional disorders known collectively as Parkinson-plus syndromes or atypical parkinsonism.

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Atypical Parkinsonian syndromes are a subset of progressive neurodegenerative disorders that present with signs of Parkinson's disease. However, due to multisystem degeneration, the atypical Parkinsonian syndromes have additional symptoms that are often referred to as Parkinson-plus syndromes. The most well-studied subsets include progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD), and Lewy body dementia.

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Key Clinical Message: Progressive supranuclear palsy (PSP) has many clinical features overlapping with other Parkinson syndromes and differentiation on clinical ground is difficult. This case highlights how a brain MRI can help diagnose PSP in settings with limited resources where histological diagnosis is difficult.

Abstract: Progressive supranuclear palsy (PSP) may be challenging to diagnose due to its widely acknowledged clinical complexity and challenges with diagnosis confirmation, particularly in resource-poor settings where the ability to obtain confirmatory tests is highly complicated, leading to an inaccurate or incomplete diagnosis of PSP.

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We present the case of a 54-year-old male, without any significant medical history, who insidiously developed speech disturbances and walking difficulties, accompanied by backward falls. The symptoms progressively worsened over time. The patient was initially diagnosed with Parkinson's disease; however, he failed to respond to standard therapy with Levodopa.

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There is a pressing need to understand the factors that predict prognosis in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), with high heterogeneity over the poor average survival. We test the hypothesis that the magnitude and distribution of connectivity changes in PSP and CBS predict the rate of progression and survival time, using datasets from the Cambridge Centre for Parkinson-plus and the UK National PSP Research Network (PROSPECT-MR). Resting-state functional MRI images were available from 146 participants with PSP, 82 participants with CBS, and 90 healthy controls.

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Article Synopsis
  • This study evaluates the effectiveness of I-metaiodobenzylguanidine (MIBG) cardiac scintigraphy for diagnosing Parkinson's disease, comparing its performance across different imaging time points in patients suspected of having the disease.
  • Researchers analyzed clinical records and various scintigraphy parameters (like heart-to-mediastinum ratio and washout rate) at several intervals post-injection to differentiate between Parkinson's-related conditions and other non-Parkinson's diseases.
  • Results indicated that the highest diagnostic accuracy for MIBG imaging occurred between 1 to 4 hours post-injection, with specific metrics reaching peak sensitivity and specificity at 4 hours, enhancing the clinical utility of this imaging technique.
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Background: Amplitude-based exercise training has been shown to be effective in the motor performance of individuals with idiopathic Parkinson's disease, with limited research investigating its effects on Parkinson plus syndromes such as olivopontocerebellar atrophy (OPCA). The purpose of this clinical case report is to examine the effects of amplitude-based training exercises on an individual with OPCA.

Case Description: A 68-year-old man with a 14-month history of OPCA presented to physical therapy with bradykinesia, rigidity, and postural instability.

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From hysteria to gait dementia: History of the concept of astasia-abasia.

Rev Neurol (Paris)

June 2023

Lauréat de l'Académie nationale de Médecine, 20, rue de Chartres, 28160 Brou, France. Electronic address:

Paul Blocq (1860-1896) and his teacher Jean-Martin Charcot (1825-1893) introduced the expression "astasia-abasia" into medical terminology in 1888 to designate a pathology they believed to be caused by hysteria. This condition makes it impossible to remain erect and to walk, whereas the ability to move the legs while lying down remains normal. At the turn of the 20th century, and now almost exclusively, this motor disturbance is recognised as a syndrome with multiple possible organic causes, and now described as "higher-level gait disorder".

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Verbal fluency is widely used as a clinical test, but its utility in differentiating between neurodegenerative dementias and progressive aphasias, and from healthy controls, remains unclear. We assessed whether various measures of fluency performance could differentiate between Alzheimer's disease, behavioural variant of frontotemporal dementia, non-fluent and semantic variants of primary progressive aphasia, progressive supranuclear palsy, corticobasal syndrome and healthy controls. Category and letter fluency tasks were administered to 33 controls and 139 patients at their baseline clinical visit.

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