Corticobasal Ganglionic Degeneration and Progressive Supranuclear Palsy: Clinical and Speech-Language Characteristics

2010 ◽  
Vol 20 (2) ◽  
pp. 45-49 ◽  
Author(s):  
Edythe A. Strand
Keyword(s):  
Progressive Supranuclear Palsy ◽  
Clinical Characteristics ◽  
Clinical Presentation ◽  
Corticobasal Degeneration ◽  
Apraxia Of Speech ◽  
Progressive Aphasia ◽  
Fluent Aphasia ◽  
Language Characteristics ◽  
Corticobasal Ganglionic Degeneration ◽  
Speech And Language Disorders

Abstract Purpose: The purpose of this article is to present a brief description of the neuroanatomical correlates, pathophysiology, and clinical presentation of corticobasal ganglionic degeneration, (often referred to as corticobasal degeneration, or CBD) and progressive supranuclear palsy (PSP). Methods: Descriptions of the clinical characteristics of the two degenerative syndromes are presented, including a discussion of the similarities and differences. The speech and language disorders associated with each are described, including progressive aphasia, progressive non-fluent aphasia (PNFA), progressive apraxia of speech (AOS), and dysarthria. Results and Conclusions: CBD and PSP have a number of commonalities in clinical characteristics and in pathology. Both progressive aphasia and apraxia of speech have been associated with CBD and PSP. Clinicians who evaluate patients exhibiting a progressive neurologic disease and who identify either progressive aphasia, PNFA and/or progressive AOS should consider CBD or PSP as a possible medical diagnosis.

scholarly journals Western Aphasia Battery–Revised Profiles in Primary Progressive Aphasia and Primary Progressive Apraxia of Speech

2020 ◽  
Vol 29 (1S) ◽  
pp. 498-510 ◽  
Author(s):  
Heather M. Clark ◽  
Rene L. Utianski ◽  
Joseph R. Duffy ◽  
Edythe A. Strand ◽  
Hugo Botha ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Relative Performance ◽  
Apraxia Of Speech ◽  
Motor Speech ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
R Classification ◽  
The Mean ◽  
Fluent Aphasia ◽  
Speech And Language Disorders

Purpose The primary aim was to examine the utility of the Western Aphasia Battery–Revised (WAB-R; Kertesz, 2007 ) for classifying variants of primary progressive aphasia (PPA). Traditional WAB-R metrics of Aphasia Quotient (AQ), subtest scores, WAB-R classification, and several novel metrics were examined. A secondary aim was to examine these same WAB-R metrics in individuals with primary progressive apraxia of speech (PPAOS). Method A retrospective analysis of WAB-R records from 169 participants enrolled in a study of neurodegenerative speech and language disorders was conducted. PPA/PPAOS classification was determined by consensus review of speech, language, and cognitive profiles. Scores on each of the WAB-R subtests were obtained to derive AQ, WAB-R aphasia profile, and 3 ratios reflecting relative performance on subtests. Results Mean AQ was significantly higher in the PPAOS group compared to all PPA variants except primary fluent aphasia. AQ above the normal cutoff was observed for 20% of participants with PPA. Significant main effects of group were noted for each of the subtests. Follow-up comparisons most frequently discriminated PPAOS, primary agrammatic aphasia (PAA), and logopenic progressive aphasia. Primary fluent aphasia and semantic dementia (SD) subtest scores were less distinctive, with the exception of Naming for SD, which was significantly lower than for PAA and PPAOS. When the WAB-R AQ detected aphasia, a classification of anomic aphasia was most frequently observed; this pattern held true for each of the PPA variants. The mean Information Content:Naming ratio was highest for SD, and the mean Comprehension:Fluency ratio was highest for PAA. Conclusions In the current study, AQ underestimated the presence of PPA and WAB-R classification did not distinguish among PPA classification determined by consensus. Performance on individual subtests and relative performance across subtests demonstrated inconsistent alignment with PPA classification. We conclude the WAB-R in isolation is inadequate to detect or characterize PPA. We instead suggest utilizing the WAB-R as 1 component of a comprehensive language and motor speech assessment when PPA is suspected.

scholarly journals A molecular pathology, neurobiology, biochemical, genetic and neuroimaging study of progressive apraxia of speech

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Keith A. Josephs ◽  
Joseph R. Duffy ◽  
Heather M. Clark ◽  
Rene L. Utianski ◽  
Edythe A. Strand ◽  
...  
Keyword(s):  
Progressive Supranuclear Palsy ◽  
Molecular Pathology ◽  
Age At Onset ◽  
Haplotype Frequency ◽  
Corticobasal Degeneration ◽  
Apraxia Of Speech ◽  
Speech Characteristics ◽  
Biochemical Genetic ◽  
Neuroimaging Study ◽  
Longitudinal Imaging

AbstractProgressive apraxia of speech is a neurodegenerative syndrome affecting spoken communication. Molecular pathology, biochemistry, genetics, and longitudinal imaging were investigated in 32 autopsy-confirmed patients with progressive apraxia of speech who were followed over 10 years. Corticobasal degeneration and progressive supranuclear palsy (4R-tauopathies) were the most common underlying pathologies. Perceptually distinct speech characteristics, combined with age-at-onset, predicted specific 4R-tauopathy; phonetic subtype and younger age predicted corticobasal degeneration, and prosodic subtype and older age predicted progressive supranuclear palsy. Phonetic and prosodic subtypes showed differing relationships within the cortico-striato-pallido-nigro-luysial network. Biochemical analysis revealed no distinct differences in aggregated 4R-tau while tau H1 haplotype frequency (69%) was lower compared to 1000+ autopsy-confirmed 4R-tauopathies. Corticobasal degeneration patients had faster rates of decline, greater cortical degeneration, and shorter illness duration than progressive supranuclear palsy. These findings help define the pathobiology of progressive apraxia of speech and may have consequences for development of 4R-tau targeting treatment.

Illustrative Cases

2017 ◽  
Author(s):  
John R. Hodges
Keyword(s):  
Cognitive Impairment ◽  
Mild Cognitive Impairment ◽  
Progressive Supranuclear Palsy ◽  
Frontotemporal Dementia ◽  
Corticobasal Degeneration ◽  
Semantic Dementia ◽  
Case Histories ◽  
The Family ◽  
Fluent Aphasia ◽  
Addenbrooke’S Cognitive Examination

This chapter comprises 16 case histories that illustrate methods of assessment described in the rest of this book and the use of the Addenbrooke’s Cognitive Examination (ACE)-III. Each case begins with a brief history from the patient and observations by the family followed by findings on cognitive examination focusing on the profile shown on the ACE-III, the results of imaging investigations, and a discussion of the diagnosis and its differential, with a final summary of the principal conclusions, indicating whether the services of a neuropsychologist are required or not. The cases present important common conditions (such as mild cognitive impairment, Alzheimer’s disease in the mild and moderate stages, behavioural variant frontotemporal dementia, progressive non-fluent aphasia, semantic dementia, corticobasal degeneration, progressive supranuclear palsy, and Huntington’s disease) as well as interesting neuropsychological syndromes (such as prosopagnosia, amnestic stoke, and transient epileptic amnesia).

scholarly journals Features of Patients With Nonfluent/Agrammatic Primary Progressive Aphasia With Underlying Progressive Supranuclear Palsy Pathology or Corticobasal Degeneration

2016 ◽  
Vol 73 (6) ◽  
pp. 733 ◽  
Author(s):  
Miguel A. Santos-Santos ◽  
Maria Luisa Mandelli ◽  
Richard J. Binney ◽  
Jennifer Ogar ◽  
Stephen M. Wilson ◽  
...  
Keyword(s):  
Progressive Supranuclear Palsy ◽  
Corticobasal Degeneration ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive

Hydrocephalus in Spinal Muscular Atrophy: A Case Report and Review of the Literature

2020 ◽  
Author(s):  
Jeetendra P. Sah ◽  
Aaron W. Abrams ◽  
Geetha Chari ◽  
Craig Linden ◽  
Yaacov Anziska
Keyword(s):  
Spinal Muscular Atrophy ◽  
Clinical Characteristics ◽  
Clinical Presentation ◽  
Muscular Atrophy ◽  
Communicating Hydrocephalus ◽  
Type I ◽  
Review Of The Literature ◽  
Radiographic Findings ◽  
Comprehensive Review ◽  
The Relationship

AbstractIn this article, we reported a case of spinal muscular atrophy (SMA) type I noted to have tetraventricular hydrocephalus with Blake's pouch cyst at 8 months of age following intrathecal nusinersen therapy. The association of hydrocephalus with SMA is rarely reported in the literature. Development of hydrocephalus after intrathecal nusinersen therapy is also reported in some cases, but a cause–effect relationship is not yet established. The aim of this study was to describe the clinical characteristics of a patient with SMA type I and hydrocephalus, to review similar cases reported in the literature, and to explore the relationship between nusinersen therapy and development of hydrocephalus. The clinical presentation and radiographic findings of the patient are described and a comprehensive review of the literature was conducted. The adverse effect of communicating hydrocephalus related to nusinersen therapy is being reported and the authors suggest carefully monitoring for features of hydrocephalus developing during the course of nusinersen therapy.

scholarly journals Mechanisms of Neurodegeneration in Various Forms of Parkinsonism—Similarities and Differences

Cells ◽  
2021 ◽  
Vol 10 (3) ◽  
pp. 656
Author(s):  
Dariusz Koziorowski ◽  
Monika Figura ◽  
Łukasz M. Milanowski ◽  
Stanisław Szlufik ◽  
Piotr Alster ◽  
...  
Keyword(s):  
Neurodegenerative Diseases ◽  
Tau Protein ◽  
Protein Gene ◽  
Clinical Presentation ◽  
Neuronal Loss ◽  
Corticobasal Degeneration ◽  
Inflammatory Factors ◽  
Parkinsonian Disorders ◽  
Genetic Features ◽  
The Brain

Parkinson's disease (PD), dementia with Lewy body (DLB), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and multiple system atrophy (MSA) belong to a group of neurodegenerative diseases called parkinsonian syndromes. They share several clinical, neuropathological and genetic features. Neurodegenerative diseases are characterized by the progressive dysfunction of specific populations of neurons, determining clinical presentation. Neuronal loss is associated with extra- and intracellular accumulation of misfolded proteins. The parkinsonian diseases affect distinct areas of the brain. PD and MSA belong to a group of synucleinopathies that are characterized by the presence of fibrillary aggregates of α-synuclein protein in the cytoplasm of selected populations of neurons and glial cells. PSP is a tauopathy associated with the pathological aggregation of the microtubule associated tau protein. Although PD is common in the world's aging population and has been extensively studied, the exact mechanisms of the neurodegeneration are still not fully understood. Growing evidence indicates that parkinsonian disorders to some extent share a genetic background, with two key components identified so far: the microtubule associated tau protein gene (MAPT) and the α-synuclein gene (SNCA). The main pathways of parkinsonian neurodegeneration described in the literature are the protein and mitochondrial pathways. The factors that lead to neurodegeneration are primarily environmental toxins, inflammatory factors, oxidative stress and traumatic brain injury.

scholarly journals Cerebrospinal Fluid α-Synuclein Species in Cognitive and Movements Disorders

2021 ◽  
Vol 11 (1) ◽  
pp. 119
Author(s):  
Vasilios C. Constantinides ◽  
Nour K. Majbour ◽  
George P. Paraskevas ◽  
Ilham Abdi ◽  
Bared Safieh-Garabedian ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Multiple System Atrophy ◽  
Progressive Supranuclear Palsy ◽  
Movement Disorders ◽  
Corticobasal Degeneration ◽  
Healthy Controls ◽  
Frontotemporal Degeneration ◽  
Blood Contamination ◽  
Dementia Patients ◽  
Specificity And Sensitivity

Total CSF α-synuclein (t-α-syn), phosphorylated α-syn (pS129-α-syn) and α-syn oligomers (o-α-syn) have been studied as candidate biomarkers for synucleinopathies, with suboptimal specificity and sensitivity in the differentiation from healthy controls. Studies of α-syn species in patients with other underlying pathologies are lacking. The aim of this study was to investigate possible alterations in CSF α-syn species in a cohort of patients with diverse underlying pathologies. A total of 135 patients were included, comprising Parkinson’s disease (PD; n = 13), multiple system atrophy (MSA; n = 9), progressive supranuclear palsy (PSP; n = 13), corticobasal degeneration (CBD; n = 9), Alzheimer’s disease (AD; n = 51), frontotemporal degeneration (FTD; n = 26) and vascular dementia patients (VD; n = 14). PD patients exhibited higher pS129-α-syn/α-syn ratios compared to FTD (p = 0.045), after exclusion of samples with CSF blood contamination. When comparing movement disorders (i.e., MSA vs. PD vs. PSP vs. CBD), MSA patients had lower α-syn levels compared to CBD (p = 0.024). Patients with a synucleinopathy (PD and MSA) exhibited lower t-α-syn levels (p = 0.002; cut-off value: ≤865 pg/mL; sensitivity: 95%, specificity: 69%) and higher pS129-/t-α-syn ratios (p = 0.020; cut-off value: ≥0.122; sensitivity: 71%, specificity: 77%) compared to patients with tauopathies (PSP and CBD). There are no significant α-syn species alterations in non-synucleinopathies.

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