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Clinical presentation and differential diagnosis of dementia in younger people
Published in Marjolein de Vugt, Janet Carter, Understanding Young Onset Dementia, 2021
Yolande A.L. Pijnenburg, Cynthia Klaassen
Corticobasal degeneration (CBD) is a rare syndrome consisting of both cognitive symptoms such as apraxia and motor symptoms (usually asymmetric rigidity, decreased use of one limb, termed alien limb). While the clinical entity has a poor correlation with the pathological entity of CBD, clinicians nowadays prefer to use the term corticobasal syndrome. Apart from CBD, Alzheimer's disease and Lewy body disease can underly this syndrome.
Hyperkinetic Movement Disorders
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Morales-Briceno Hugo, Victor S.C. Fung, Annu Aggarwal, Philip Thompson
Neurodegenerative: Parkinson's disease (PD).Huntington's disease (HD).Spinocerebellar ataxia (SCA1, 2, 3, 6, 17).Dentatorubral-pallidoluysian atrophy (DRPLA).Corticobasal syndrome (CBS).Progressive supranuclear palsy (PSP).Multiple system atrophy (MSA).
Neuropsychiatry
Published in Jeremy Playfer, John Hindle, Andrew Lees, Parkinson's Disease in the Older Patient, 2018
Patients with corticobasal degeneration (CBD) have more depression and irritability, but less apathy than those with PSP.171 Patients may demonstrate cognitive abnormalities reflecting cortical disease including apraxias, alien hand, visual and sensory neglect. Gesture problems are said to be specific for CBD. Visuospatial function is intact in MSA, mildly impaired in PSP and significantly impaired in CBD, suggesting differential distribution of object-based and spatial functions in the brain.172 Using the Addenbrookes Cognitive Examination (ACE-R) as a screening tool, there is more cognitive deterioration in patients with CBD than there is in those with PSP, with no deterioration in the case of patients with MSA.173 Rarely, corticobasal syndrome can be due to motor neurone disease (MND), which itself can be associated with parkinsonism. MND may prove to be a multisystem disease and a cause of frontotemporal dementia.
The changing landscape of neuroimaging in frontotemporal lobar degeneration: from group-level observations to single-subject data interpretation
Published in Expert Review of Neurotherapeutics, 2022
Mary Clare McKenna, Aizuri Murad, William Huynh, Jasmin Lope, Peter Bede
A formal literature review search was conducted using the PubMed repository (last accessed on 11 February 2021). The following search strategy was used: (‘Frontotemporal lobar degeneration’[Mesh] OR ‘Frontotemporal lobar degeneration’ OR ‘frontotemporal dementia’ [Mesh] OR ‘frontotemporal dementia’ OR ‘frontotemporal degeneration’ OR ‘Primary Progressive Aphasia’ [Mesh] OR ‘behavioural variant frontotemporal dementia’ OR ‘non-fluent variant primary progressive aphasia’ OR ‘semantic-variant primary progressive aphasia’ OR ‘FTLD’ OR ‘FTD’ OR ‘bvFTD’ OR ‘PPA’ OR ‘nfvPPA’ OR ‘svPPA’) AND (‘Magnetic Resonance Imaging’[Mesh] OR ‘MRI’ OR ‘diffusion tensor imaging’ OR ‘functional MRI’ OR ‘fMRI’ OR ‘DTI’) AND (‘Machine Learning’[Mesh] OR ‘classification’ OR ‘accuracy’ OR ‘deep learning’ OR ‘support vector machine ’OR ‘supervised machine learning’ OR ‘unsupervised machine learning’). Pathological subgroups ‘tau’ and ‘pTDP-43’ were not included in the search strategy. Our search was limited to studies written in English that involved human subjects. All papers were screened by title and abstract and the full text of selected articles were then reviewed. The inclusion criteria included: (1) original research articles investigating single-subject classification of FTLD, bvFTD, PPA, nfvPPA or svPPA and (2) used classification features derived from structural or functional MRI only. We excluded studies that investigated other phenotypes such as corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP). The reference lists of selected articles were also reviewed to identify additional relevant papers.
Emerging drugs for progressive supranuclear palsy
Published in Expert Opinion on Emerging Drugs, 2019
Nikolaos Giagkou, Maria Stamelou
RS, however, is not the only phenotype associated with the disease. In fact, in a series of 100 autopsy-proven PSP cases, only one-fourth had RS [3]. The broad spectrum of syndromes that has been linked to PSP pathology also includes PSP-parkinsonism, a variant that presents with features suggestive of Parkinson’s disease (PD), pure akinesia with gait freezing, corticobasal syndrome, non-fluent variant primary progressive aphasia, behavioural variant frontotemporal dementia and even PSP presenting with cerebellar ataxia [1]. This overlap with clinical features of other neurodegenerative diseases explains why a clinical diagnosis can be difficult to make [3]. The presence of classic features of the disease, i.e. supranuclear gaze palsy and postural instability, is associated with more accurate and earlier diagnosis [3]. However, these features are not diagnostic and might not develop early in the disease course [4]. Patients with PSP pathology are often misdiagnosed as having Parkinson’s disease, the most common misdiagnosis, or other atypical parkinsonian syndromes, frontotemporal dementia or Alzheimer’s disease [4]. Conversely, patients with other pathologies may present with RS phenotype [4].
Primary lateral sclerosis: a distinct entity or part of the ALS spectrum?
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2019
Eoin Finegan, Rangariroyashe H. Chipika, Stacey Li Hi Shing, Orla Hardiman, Peter Bede
Similar to ALS, there is an increasing recognition of extra-motor features in PLS. The term “PLS-plus” has been used to describe cases of PLS associated with dementia or extrapyramidal features [11,14]. A recent study found evidence of comorbid frontotemporal dementia in 6 out of 181 (3.3%) PLS patients, and noted that none of the 6 PLS-FTD patients had C9orf72 hexanucleotide expansions [15]. Another study reported the development of corticobasal syndrome in 3 out of 35 (8.6%) PLS patients, 4–7 years after their diagnosis [16]. Mills syndrome [17], an uncommon, unilateral or asymmetric variant of PLS [18,19] and is thought to be associated with asymmetric frontal lobe changes on MRI [20] and PET [21,22].