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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
In recent nomenclature, the former term progressive nonfluent aphasia has been replaced by the nonfluent variant of PPA (Gorno-Tempini et al., 2011; Neary et al., 1998). Patients with nfPPA have a reduced rate of speech. They may make grammatical and phonemic errors. Speech is effortful and there are sound distortions. Dysartria and aprosodia may be present. These patients often become dependent on electronic speech devices, although emerging executive deficits will eventually hinder their use. A proportion of patients progress to PSP or corticobasal degeneration. Another proportion proceeds to frontal lobe syndrome, with behavioural change and eventual mutism.
Neurology
Published in Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan, Essential Notes for Medical and Surgical Finals, 2021
Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan
This is a group of disorders characterised by frontal/temporal lobe atrophy, often with young onset (<65 years). There are three main syndromes: frontotemporal dementia – personality change/behavioural symptoms.semantic dementia – loss of semantic memory, i.e. conceptual knowledge.progressive non-fluent aphasia – impaired speech production.Other causes of dementia – it is important to rule out ‘reversible’ causesMetabolic: Vitamin B12/folate deficiency, hypothyroidism, Wilson’s diseaseInflammation/infection: HIV, multiple sclerosis, cerebral vasculitis, SLE, neurosyphilisDegenerative: Huntington’s disease, progressive supranuclear palsy, CJD Alcohol/toxicStructural: chronic subdural haematoma, tumours
Frontotemporal Dementia
Published in Marc E. Agronin, Alzheimer's Disease and Other Dementias, 2014
The progressive aphasia (PA) subtype of FTD has also been called progressive nonfluent aphasia as well as primary progressive aphasia in previous literature. It is associated with left hemispheric degeneration in the perisylvian cortex (Neary, Snowden, & Mann, 2005) and characterized by an evolving expressive aphasia with slowed, nonfluent speech with marked word-finding difficulty and eventual progression to muteness (Ash et al., 2006). Individuals may also demonstrate stuttering, alexia, and dysgraphia (Cardarelli et al., 2010). Although memory disturbances are not prominent early in the course of PA, many cases progress to a more generalized dementia within several years that may include behavioral symptoms.
Differences of apathy perfusion correlates between Alzheimer’s disease and frontotemporal dementia. A 99mTc-HMPAO SPECT study with automated Brodmann areas analysis
Published in International Journal of Psychiatry in Clinical Practice, 2022
Varvara Valotassiou, Nikolaos Sifakis, Chara Tzavara, Evi Lykou, Niki Tsinia, Vasiliki Kamtsadeli, Dimitra Sali, George Angelidis, Dimitrios Psimadas, Ioannis Tsougos, Sokratis G. Papageorgiou, Panagiotis Georgoulias, John Papatriantafyllou
Neuropsychiatric symptoms (NPSs) in dementia have received relatively little attention for many years, compared with cognitive deficits, although they can affect patients’ life and increase the likelihood of institutionalisation and cause great caregiver distress (Cummings et al. 1994). Among the spectrum of NPSs, apathy is one of the most common and clinically important NPSs (Starkstein 2000; Chase 2011), which may affect the majority of demented patients over the course of their disease and may be present even in predementia stages (Guercio et al. 2015). It has been reported that apathy affects 36–42% of patients with mild dementia and up to 80% of those with moderate dementia (Lyketsos et al. 2002). More specifically, in Alzheimer’s disease (AD), its average prevalence is 65.1% (Van Reekum et al. 2005), while in fronto-temporal dementia (FTD) apathy is more frequent and severe than in AD with a prevalence ranging from 60% to 90% (Cummings 1997; Shinagawa et al. 2006). In patients with FTD, apathy is most commonly seen in the behavioural variant (bvFTD) (Chow et al. 2009), following by the progressive non fluent aphasia (PNFA) (Rohrer and Warren 2010). It is also common in progressive supranuclear palsy (PSP) (Belvisi et al. 2018), while patients with FTD motor neuron disease (FTD-MND) may also exhibit the symptom (Raaphorst et al. 2012). Finally, the prevalence and severity of apathy is lower in semantic dementia (SD) compared with bvFTD, with a trend to increase with progression of disease (Rohrer and Warren 2010; Merrilees et al. 2013).
The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) for Japanese ALS and FTD patients
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2021
Yasuhiro Watanabe, Mieko Ogino, Hiroo Ichikawa, Ritsuko Hanajima, Kenji Nakashima
Patients with ALS were recruited from the Department of Neurology, Tottori University. The patients were diagnosed using the revised El Escorial criteria (probable or definite) (14). Diagnosis of behavioural variant FTD (bvFTD) was made according to the Rascovsky criteria (15) based on a comprehensive full neuropsychological assessment. Patients who met both the revised El Escorial and Rascovsky criteria were classified as ALS-FTD (8). No patients were diagnosed with Alzheimer’s disease (AD), ALS with progressive non-fluent aphasia, or ALS with semantic dementia, based on respective criteria (16,17). Patients with other neurological, psychiatric, or medical conditions that can affect cognition were excluded. None of the patients in this cohort received non-invasive positive pressure ventilation or tracheotomy positive pressure ventilation. In addition, no patient was noted as receiving alternative nutrition such as percutaneous endoscopic gastrostomy. Baseline characteristics (sex, age at onset, years of education, disease duration, site of onset, and presence or absence of bulbar involvement) were obtained for the patient group.
Primary progressive aphasia and the FTD-MND spectrum disorders: clinical, pathological, and neuroimaging correlates
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2019
Giulia Vinceti, Nicholas Olney, Maria Luisa Mandelli, Salvatore Spina, H. Isabel Hubbard, Miguel A. Santos-Santos, Christa Watson, Zachary A. Miller, Catherine Lomen-Hoerth, Paolo Nichelli, Bruce L. Miller, Lea T. Grinberg, William W. Seeley, Maria Luisa Gorno-Tempini
The cognitive profile of the FTD-MND spectrum is most often described as social cognition and behavioral disturbances or executive dysfunction, compatible with a diagnosis of behavioral variant of FTD (bvFTD){Phukan, 2007 #1440; Mioshi, 2014 #242; Woollacott, 2016 #1319; Lansdall, 2017 #1312}. Although speech and language disorders associated with MND have been previously investigated and a few cases of primary progressive aphasia (PPA) with MND have also been described {Caselli, 1993 #1425; Tsuchiya, 2000 #1311; da Rocha, 2007 #1426; Ostberg, 2011 #1431}, an isolated speech and language disorder is not considered the most common presentation of FTD-MND. It is also still a matter of debate if language impairment in MND patients represents true language deficit or downstream manifestation of other compromised cognitive functions (4). Only in very recent years, some studies have drawn attention to the possibility that language dysfunction could recur as frequently as the executive dysfunction in ALS (8–14) and their contribution was revised in a recent review (15). Another study considered the relative frequencies of FTD phenotypes (bvFTD, semantic dementia and progressive non-fluent aphasia) in a clinical consecutive cohort of patients diagnosed with FTD with or without associated ALS; they concluded that pure language phenotypes (semantic dementia and progressive non-fluent aphasia) occur infrequently in FTD cases with coexistent ALS (16). In another paper, the authors focused on the different symptoms’ profile of bvFTD as opposed to bvFTD with ALS (termed ALS-FTD), the same authors observed a comparatively greater degree of language impairment in the ALS-FTD group (17).