1,965 results match your criteria Cortical Basal Ganglionic Degeneration

[Atypical Parkinson's syndrome in old age].

Z Gerontol Geriatr 2022 Jun 24. Epub 2022 Jun 24.

Klinikum Nürnberg, Paracelsus Medizinische Privatuniversität, Breslauerstr. 201, 90471, Nürnberg, Deutschland.

Atypical Parkinson syndromes represent a neuropathologically heterogeneous group and include the clinical entities dementia with Lewy bodies (DLB), multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The DLB and MSA are characterized by deposition of the protein alpha-synuclein (synucleinopathy), PSP and CBD are characterized by deposition of tau protein, often in the form of neurofibrillary tangles in nerve and glial cells (tauopathy). Misfolding and aggregation of the aforementioned proteins causes degeneration of the affected cell populations but the disease also spreads to anatomically neighboring brain regions, thus contributing to disease progression. Read More

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Differentially Expressed miRNAs in Age-Related Neurodegenerative Diseases: A Meta-Analysis.

Genes (Basel) 2022 Jun 9;13(6). Epub 2022 Jun 9.

Department of (Neuro)Pathology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, 1105 AZ Amsterdam, The Netherlands.

To date, no neurodegenerative diseases (NDDs) have cures, and the underlying mechanism of their pathogenesis is undetermined. As miRNAs extensively regulate all biological processes and are crucial regulators of healthy brain function, miRNAs differentially expressed in NDDs may provide insight into the factors that contribute to the emergence of protein inclusions and the propagation of deleterious cellular environments. A meta-analysis of miRNAs dysregulated in Alzheimer's disease, Parkinson's disease, multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, dementia with Lewy bodies and frontotemporal lobar degeneration (TDP43 variant) was performed to determine if diseases within a proteinopathy have distinct or shared mechanisms of action leading to neuronal death, and if proteinopathies can be classified on the basis of their miRNA profiles. Read More

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Complementary value of metabolic and tau PET imaging in the diagnosis of corticobasal degeneration.

Eur J Nucl Med Mol Imaging 2022 Jun 20. Epub 2022 Jun 20.

Department of Nuclear Medicine, DongchengDistrict, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, WangfujingBeijing, 100730, China.

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Neuropathology and emerging biomarkers in corticobasal syndrome.

J Neurol Neurosurg Psychiatry 2022 Jun 13. Epub 2022 Jun 13.

Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA.

Corticobasal syndrome (CBS) is a clinical syndrome characterised by progressive asymmetric limb rigidity and apraxia with dystonia, myoclonus, cortical sensory loss and alien limb phenomenon. Corticobasal degeneration (CBD) is one of the most common underlying pathologies of CBS, but other disorders, such as progressive supranuclear palsy (PSP), Alzheimer's disease (AD) and frontotemporal lobar degeneration with TDP-43 inclusions, are also associated with this syndrome.In this review, we describe common and rare neuropathological findings in CBS, including tauopathies, synucleinopathies, TDP-43 proteinopathies, fused in sarcoma proteinopathy, prion disease (Creutzfeldt-Jakob disease) and cerebrovascular disease, based on a narrative review of the literature and clinicopathological studies from two brain banks. Read More

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The Non-motor Symptoms, Disability Progression, and Survival Analysis of Atypical Parkinsonism: Case Series from Eastern India and Brief Review of Literature.

J Neurosci Rural Pract 2022 Apr 7;13(2):276-282. Epub 2022 Apr 7.

Department of Neurology, SCB Medical College and Hospital, Cuttack, Odisha, India.

 The objectives of this study are (1) to describe the non-motor profile, the motor disability progression, and survival analysis of atypical parkinsonism in a tertiary care hospital of eastern India and (2) to elucidate the neurocircuitry and the putative substrates responsible for non-motor manifestations.  In this prospective observational study, patients were diagnosed based on Consensus Criteria for Progressive Supranuclear Palsy (PSP), The Fourth Consensus Report of the Dementia with Lewy Body (DLBD) Consortium 2017, The Autonomic Neuroscience 2018 Criteria for Multiple System Atrophy (MSA), and Armstrong 2013 Criteria for Corticobasal Degeneration (CBD). Disease severity was assessed at baseline and 6 months of follow-up using the Unified Parkinson's Disease Rating Scales (UPDRS). Read More

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A Case of Pathologically Confirmed Corticobasal Degeneration Initially Presenting as Progressive Supranuclear Palsy Syndrome.

J Korean Med Sci 2022 Jun 6;37(22):e183. Epub 2022 Jun 6.

Department of Neurology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea.

Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) overlap clinically with parkinsonism or extrapyramidal signs and pathologically with tauopathy. Asymmetric parkinsonism and cortical dysfunctions are classical features of CBD. However, symmetric parkinsonism, frequent falls, and supranuclear gaze palsy are key features of PSP. Read More

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Histologic lesion type correlates of magnetic resonance imaging biomarkers in four-repeat tauopathies.

Brain Commun 2022 28;4(3):fcac108. Epub 2022 Apr 28.

Department of Radiology, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA.

Primary four-repeat tauopathies are characterized by depositions of the four-repeat isoform of the microtubule binding protein, tau. The two most common sporadic four-repeat tauopathies are progressive supranuclear palsy and corticobasal degeneration. Because tau PET tracers exhibit poor binding affinity to four-repeat pathology, determining how well MRI findings relate to underlying pathology is critical to evaluating their utility as surrogate markers to aid in diagnosis and as outcome measures for clinical trials. Read More

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Imaging of Synaptic Density in Neurodegenerative Disorders.

J Nucl Med 2022 Jun;63(Suppl 1):60S-67S

Neuroscience Discovery Research, Translational Imaging, AbbVie, North Chicago, Illinois.

PET technology has produced many radiopharmaceuticals that target specific brain proteins and other measures of brain function. Recently, a new approach has emerged to image synaptic density by targeting the synaptic vesicle protein 2A (SV2A), an integral glycoprotein in the membrane of synaptic vesicles and widely distributed throughout the brain. Multiple SV2A ligands have been developed and translated to human use. Read More

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F-FDG PET Imaging in Neurodegenerative Dementing Disorders: Insights into Subtype Classification, Emerging Disease Categories, and Mixed Dementia with Copathologies.

J Nucl Med 2022 Jun;63(Suppl 1):2S-12S

Department of Nuclear Medicine, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany.

Since the invention of F-FDG as a neurochemical tracer in the 1970s, F-FDG PET has been used extensively for dementia research and clinical applications. FDG, a glucose analog, is transported into the brain via glucose transporters and metabolized in a concerted process involving astrocytes and neurons. Although the exact cellular mechanisms of glucose consumption are still under investigation, F-FDG PET can sensitively detect altered neuronal activity due to neurodegeneration. Read More

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Automated Detection of Speech Timing Alterations in Autopsy-Confirmed Non-fluent/agrammatic Variant Primary Progressive Aphasia.

Neurology 2022 May 27. Epub 2022 May 27.

Memory and Aging Center, Department of Neurology, University of California, San Francisco, CA, USA

Background And Objectives: Motor speech function, including speech timing, is a key domain for diagnosing non-fluent/agrammatic variant primary progressive aphasia (nfvPPA). Yet, standard assessments employ subjective, specialist-dependent evaluations, undermining reliability and scalability. Moreover, few studies have examined relevant anatomo-clinical alterations in patients with pathologically-confirmed diagnoses. Read More

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The role of basket trials in drug development for neurodegenerative disorders.

Alzheimers Res Ther 2022 May 25;14(1):73. Epub 2022 May 25.

Department of Computer Science, Howard Hughes School of Engineering, University of Nevada Las Vegas (UNLV), Las Vegas, NV, USA.

Background: Drug development for neurodegenerative disorders (NDDs) is a long, complex, and expensive enterprise. Methods to optimize drug development for NDDs are needed. Basket trials have been widely used in oncology and have been promoted by the Food and Drug Administration as a means of enhancing the efficiency of drug development. Read More

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Genetic Architecture of Primary Tauopathies.

Neuroscience 2022 May 21. Epub 2022 May 21.

CIBERNED, Network Center for Biomedical Research in Neurodegenerative Diseases, National Institute of Health Carlos III, 28220 Madrid, Spain; Alzheimer's Centre Reina Sofia-CIEN Foundation-ISCIII, 28031 Madrid, Spain.

Primary Tauopathies are a group of diseases defined by the accumulation of Tau, in which the alteration of this protein is the primary driver of the neurodegenerative process. In addition to the classical syndromes (Pick's disease (PiD), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and argyrophilic grain disease (AGD)), new entities, like primary age-related Tauopathy (PART), have been recently described. Except for the classical Richardson's syndrome phenotype in PSP, the correlation between the clinical picture of the primary Tauopathies and underlying pathology is poor. Read More

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Combined CSF α-SYN RT-QuIC, CSF NFL and midbrain-pons planimetry in degenerative parkinsonisms: From bedside to bench, and back again.

Parkinsonism Relat Disord 2022 Jun 13;99:33-41. Epub 2022 May 13.

Parkinson's Disease & Movement Disorders Unit, Neurology Service, Hospital Clínic I Universitari de Barcelona; IDIBAPS, CIBERNED (CB06/05/0018-ISCIII), ERN-RND, Institut Clínic de Neurociències (Maria de Maeztu Excellence Centre), Universitat de Barcelona. Barcelona, Catalonia, Spain; Lab of Parkinson Disease and Other Neurodegenerative Movement Disorders: Clinical and Experimental Research; Department of Neurology, Hospital Clínic de Barcelona, Institut D'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Institut de Neurociències, Universitat de Barcelona, Barcelona, Catalonia, Spain.

Introduction: Differential diagnosis between Parkinson's disease (PD) and atypical parkinsonisms (APs: multiple system atrophy[MSA], progressive supranuclear palsy[PSP], corticobasal degeneration[CBD]) remains challenging. Lately, cerebrospinal fluid (CSF) studies of neurofilament light-chain (NFL) and RT-QuIC of alpha-synuclein (α-SYN) have shown promise, but data on their combination with MRI measures is lacking.

Objective: (1) to assess the combined diagnostic ability of CSF RT-QuIC α-SYN, CSF NFL and midbrain/pons MRI planimetry in degenerative parkinsonisms; (2) to evaluate if biomarker-signatures relate to clinical diagnoses and whether or not unexpected findings can guide diagnostic revision. Read More

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Clinical Aspects of the Differential Diagnosis of Parkinson's Disease and Parkinsonism.

J Clin Neurol 2022 May;18(3):259-270

Department of Neurology, Chung-Ang University College of Medicine, Seoul, Korea.

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. Read More

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Pathologically Verified Corticobasal Degeneration Mimicking Richardson's Syndrome Coexisting with Clinically and Radiologically Shunt-Responsive Normal Pressure Hydrocephalus.

Mov Disord Clin Pract 2022 May 12;9(4):508-515. Epub 2022 Apr 12.

Department of Neurology National Center Hospital, National Center of Neurology and Psychiatry Tokyo Japan.

Background: Normal pressure hydrocephalus (NPH) manifests as gait instability, cognitive impairment, and urinary incontinence. This clinical triad of NPH sometimes occurs with ventriculomegaly in patients with neurodegenerative disease. Patients with pathologically verified neurodegenerative diseases, such as progressive supranuclear palsy (PSP), have received antemortem diagnoses of NPH. Read More

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PET-based classification of corticobasal syndrome.

Parkinsonism Relat Disord 2022 05 25;98:92-98. Epub 2022 Apr 25.

National Institute for Quantum Medical Science, National Institutes for Quantum Science and Technology, Chiba, Japan. Electronic address:

Introduction: Corticobasal degeneration (CBD) is the most common neuropathological substrate for clinically diagnosed corticobasal syndrome (CBS), while identifying CBD pathology in living individuals has been challenging. This study aimed to examine the capability of positron emission tomography (PET) to detect CBD-type tau depositions and neuropathological classification of CBS.

Methods: Sixteen CBS cases diagnosed by Cambridge's criteria and 12 cognitively healthy controls (HCs) underwent PET scans with C-PiB, C-PBB3, and F-FDG, along with T1-weighted magnetic resonance imaging. Read More

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Amyloid and Tau Positron Emission Tomography Imaging in Alzheimer's Disease and Other Tauopathies.

Front Aging Neurosci 2022 22;14:838034. Epub 2022 Apr 22.

Institute for Regenerative Medicine, University of Zurich, Zurich, Switzerland.

The detection and staging of Alzheimer's disease (AD) using non-invasive imaging biomarkers is of substantial clinical importance. Positron emission tomography (PET) provides readouts to uncover molecular alterations in the brains of AD patients with high sensitivity and specificity. A variety of amyloid-β (Aβ) and tau PET tracers are already available for the clinical diagnosis of AD, but there is still a lack of imaging biomarkers with high affinity and selectivity for tau inclusions in primary tauopathies, such as progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and Pick's disease (PiD). Read More

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Comprehensive cross-sectional and longitudinal analyses of plasma neurofilament light across FTD spectrum disorders.

Cell Rep Med 2022 Apr 19;3(4):100607. Epub 2022 Apr 19.

Krembil Brain Institute, Tanz Centre for Research in Neurodegenerative Diseases, Division of Neurology, Department of Medicine, University of Toronto, Toronto, ON M5S 1A8, Canada.

Frontotemporal dementia (FTD) therapy development is hamstrung by a lack of susceptibility, diagnostic, and prognostic biomarkers. Blood neurofilament light (NfL) shows promise as a biomarker, but studies have largely focused only on core FTD syndromes, often grouping patients with different diagnoses. To expedite the clinical translation of NfL, we avail ARTFL LEFFTDS Longitudinal Frontotemporal Lobar Degeneration (ALLFTD) study resources and conduct a comprehensive investigation of plasma NfL across FTD syndromes and in presymptomatic FTD mutation carriers. Read More

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Diagnostic Accuracy of Magnetic Resonance Imaging Measures of Brain Atrophy Across the Spectrum of Progressive Supranuclear Palsy and Corticobasal Degeneration.

JAMA Netw Open 2022 04 1;5(4):e229588. Epub 2022 Apr 1.

Memory and Aging Center, Department of Neurology, University of California, San Francisco.

Importance: The accurate diagnosis of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) is hampered by imperfect clinical-pathological correlations.

Objective: To assess and compare the diagnostic value of the magnetic resonance parkinsonism index (MRPI) and other magnetic resonance imaging-based measures of cerebral atrophy to differentiate between PSP, CBD, and other neurodegenerative diseases.

Design, Setting, And Participants: This prospective diagnostic study included participants with 4-repeat tauopathies (4RT), PSP, CBD, other neurodegenerative diseases and available MRI who appeared in the University of California, San Francisco, Memory and Aging Center database. Read More

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Tau protein quantification in skin biopsies differentiates tauopathies from alpha-synucleinopathies.

Brain 2022 Apr 29. Epub 2022 Apr 29.

Laboratory for Biomedical Neurosciences, Neurocenter of Southern Switzerland, Ente Ospedaliero Cantonale, Lugano, Switzerland.

Abnormal accumulation of microtubule-associated protein tau (τ) is a characteristic feature of atypical parkinsonisms with tauopathies such as Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD). However, pathological τ has also been observed in α-synucleinopathies like Parkinson's Disease (PD) and Multiple System Atrophy (MSA). Based on the involvement of peripheral nervous system in several neurodegenerative diseases, we characterized and compared τ expression in skin biopsies of patients clinically diagnosed with PD, MSA, PSP, CBD, and in healthy control subjects. Read More

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The tauopathies: Neuroimaging characteristics and emerging experimental therapies.

J Neuroimaging 2022 Apr 25. Epub 2022 Apr 25.

Department of Radiology and Imaging Sciences, Indiana University School of Medicine, Indianapolis, Indiana, USA.

The tauopathies are a heterogeneous group of neurodegenerative disorders in which the prevailing underlying disease process is intracellular deposition of abnormal misfolded tau protein. Diseases often categorized as tauopathies include progressive supranuclear palsy, chronic traumatic encephalopathy, corticobasal degeneration, and frontotemporal lobar degeneration. Tauopathies can be classified through clinical assessment, imaging findings, histologic validation, or molecular biomarkers tied to the underlying disease mechanism. Read More

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Amyloid β, Tau, and α-Synuclein aggregates in the pathogenesis, prognosis, and therapeutics for neurodegenerative diseases.

Prog Neurobiol 2022 07 18;214:102270. Epub 2022 Apr 18.

Mitchell Center for Neurodegenerative Diseases, University of Texas Medical Branch, Galveston, TX, USA; Departments of Neurology, Neuroscience and Cell Biology, Pharmacology and Toxicology, University of Texas Medical Branch, Galveston, TX, USA. Electronic address:

Aggregation of specific proteins are histopathological hallmarks of several neurodegenerative diseases, such as, Amyloid β (Aβ) plaques and tau neurofibrillary tangles in Alzheimer's disease (AD); morphologically different inclusions of ratiometric 3 repeat (3 R) and 4 repeat (4 R) tau isoforms in progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and Pick's disease (PiD); α-Synuclein (α-Syn) containing Lewy bodies (LBs) and dystrophic Lewy neurites (LNs) in Parkinson's disease (PD) and dementia with Lewy bodies (DLB). However, mixed brain protein pathologies have been frequently observed in many of these diseases and in normal aging brains, among which Aβ/tau and tau/α-Syn crosstalks have received increased attention. Interestingly, studies have also shown synergistic interplay among Aβ, tau, and α-Syn in several neurodegenerative diseases, suggesting a protein triumvirate. Read More

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Neuropathological fingerprints of survival, atrophy and language in primary progressive aphasia.

Brain 2022 Apr 20. Epub 2022 Apr 20.

Mesulam Center for Cognitive Neurology and Alzheimer's Disease, Feinberg School of Medicine, Chicago, IL 60611, USA.

Primary progressive aphasia is a neurodegenerative disease that selectively impairs language without equivalent impairment of speech, memory or comportment. In 118 consecutive autopsies on patients with primary progressive aphasia, primary diagnosis was Alzheimer's disease neuropathological changes (ADNC) in 42%, corticobasal degeneration or progressive supranuclear palsy neuropathology in 24%, Pick's disease neuropathology in 10%, transactive response DNA binding proteinopathy type A [TDP(A)] in 10%, TDP(C) in 11% and infrequent entities in 3%. Survival was longest in TDP(C) (13. Read More

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Diminished preparatory physiological responses in frontotemporal lobar degeneration syndromes.

Brain Commun 2022 4;4(2):fcac075. Epub 2022 Apr 4.

Department of Psychology, University of California, Berkeley, 2121 Berkeley Way, Berkeley, CA 94720-1650, USA.

Researchers typically study physiological responses either after stimulus onset or when the emotional valence of an upcoming stimulus is revealed. Yet, participants may also respond when they are told that an emotional stimulus is about to be presented even without knowing its valence. Increased physiological responding during this time may reflect a 'preparation for action'. Read More

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Utility of 18F FDG-PET in Parkinsonism in an African population.

eNeurologicalSci 2022 Jun 31;27:100399. Epub 2022 Mar 31.

Department of Nuclear Medicine, Inkosi Albert Luthuli Central Hospital, Cato Manor, Durban, South Africa, Head of Nuclear Medicine Department, Sefako Makgatho Health Sciences University, Ga-Rankuwa, South Africa.

Parkinson's Disease remains a diagnostic challenge. Misdiagnosis during life is approximately 25%. Diseases that resemble PD clinically, such as the Parkinsonianplus disorders usually have a poorer prognosis. Read More

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F-THK5351 PET for visualizing predominant lesions of pathologically confirmed corticobasal degeneration presenting with frontal behavioral-spatial syndrome.

J Neurol 2022 Apr 13. Epub 2022 Apr 13.

Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, 187-8551, Japan.

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REM Sleep Behavior Disorder and Visual Hallucinations in a Pathologically Confirmed Case of Corticobasal Degeneration.

Mov Disord Clin Pract 2022 Apr 6;9(3):383-385. Epub 2022 Jan 6.

Department of Clinical and Movement Neurosciences, Queen Square Brain Bank for Neurological Disorders UCL Queen Square Institute of Neurology London United Kingdom.

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[Idiopathic Normal Pressure Hydrocephalus and Neurodegenerative Diseases: a Short Review of Differential Diagnosis].

No Shinkei Geka 2022 Mar;50(2):319-330

Department of Neurology, Kyoto Prefectural University of Medicine.

There are a variety of neurodegenerative diseases that require differentiation from idiopathic normal pressure hydrocephalus(iNPH), including Alzheimer's disease, Parkinson's disease, dementia with Lewy bodies, frontotemporal dementia, progressive supranuclear palsy, and corticobasal degeneration. As the clinical features and structural imaging findings of these diseases may overlap with iNPH, biomarkers reflecting disease-specific pathology are necessary for differential diagnosis. In addition, these diseases often coexist with iNPH in elderly patients, and it is important to confirm the coexistence of their pathology even in cases clinically diagnosed as iNPH. Read More

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Brainstem atrophy is linked to extrapyramidal symptoms in frontotemporal dementia.

J Neurol 2022 Apr 4. Epub 2022 Apr 4.

Institute of Clinical Medicine - Neurology, University of Eastern Finland, P.O. Box 1627 (Yliopistonranta 1C), 70211, Kuopio, Finland.

Extrapyramidal (EP) symptoms are a known feature in a subpopulation of patients with behavioral variant frontotemporal dementia (bvFTD). Concomitant EP symptoms with FTD-like neuropsychiatric symptoms are also core features in progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). This complicates the early diagnosis of these disorders. Read More

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Regional Selectivity of Neuromelanin Changes in the Substantia Nigra in Atypical Parkinsonism.

Mov Disord 2022 Jun 29;37(6):1245-1255. Epub 2022 Mar 29.

ICM, Centre de NeuroImagerie de Recherche-CENIR, Paris, France.

Background: Neurodegeneration in the substantia nigra pars compacta (SNc) in parkinsonian syndromes may affect the nigral territories differently.

Objective: The objective of this study was to investigate the regional selectivity of neurodegenerative changes in the SNc in patients with Parkinson's disease (PD) and atypical parkinsonism using neuromelanin-sensitive magnetic resonance imaging (MRI).

Methods: A total of 22 healthy controls (HC), 38 patients with PD, 22 patients with progressive supranuclear palsy (PSP), 20 patients with multiple system atrophy (MSA, 13 with the parkinsonian variant, 7 with the cerebellar variant), 7 patients with dementia with Lewy body (DLB), and 4 patients with corticobasal syndrome were analyzed. Read More

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