Neuropsychological differentiation of progressive aphasic disorders

Harris, Jennifer; Saxon, Jennifer A.; Jones, Matthew; Snowden, Julie S.; Thompson, James C.

doi:10.1111/jnp.12149

Cited by 31 publications

(34 citation statements)

References 61 publications

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“…The latter requires patients to rearrange five randomly presented printed words to form a sentence (eg, they went to the beach) and was included because of its proven sensitivity to grammatical impairments in FTD. 25 Executive tests comprised letter and category fluency, and sorting tests from the Delis-Kaplan Executive Function System battery (DKEFS), 26 and the Hayling and Brixton tests 27 to assess response inhibition and rule abstraction and set shifting. Social cognition was assessed by a Judgement of Preference from Eye Gaze task 28 and emotion recognition using the Ekman and Friesen faces.…”

Section: Methodsmentioning

confidence: 99%

Cognition and behaviour in frontotemporal dementia with and without amyotrophic lateral sclerosis

Saxon

Thompson

Harris

et al. 2020

J Neurol Neurosurg Psychiatry

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ObjectiveThe precise relationship between frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) is incompletely understood. The association has been described as a continuum, yet data suggest that this may be an oversimplification. Direct comparisons between patients who have behavioural variant FTD (bvFTD) with and without ALS are rare. This prospective comparative study aimed to determine whether there are phenotypic differences in cognition and behaviour between patients with FTD-ALS and bvFTD alone.MethodsPatients with bvFTD or FTD-ALS and healthy controls underwent neuropsychological testing, focusing on language, executive functions and social cognition. Behavioural change was measured through caregiver interview. Blood samples were screened for known FTD genes.Results23 bvFTD, 20 FTD-ALS and 30 controls participated. On cognitive tests, highly significant differences were elicited between patients and controls, confirming the tests’ sensitivities to FTD. bvFTD and FTD-ALS groups performed similarly, although with slightly greater difficulty in patients with ALS-FTD on category fluency and a sentence-ordering task that assesses grammar production. Patients with bvFTD demonstrated more widespread behavioural change, with more frequent disinhibition, impulsivity, loss of empathy and repetitive behaviours. Behaviour in FTD-ALS was dominated by apathy. The C9ORF72 repeat expansion was associated with poorer performance on language-related tasks.ConclusionsDifferences were elicited in cognition and behaviour between bvFTD and FTD-ALS, and patients carrying the C9ORF72 repeat expansion. The findings, which raise the possibility of phenotypic variation between bvFTD and FTD-ALS, have clinical implications for early detection of FTD-ALS and theoretical implications for the nature of the relationship between FTD and ALS.

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Section: Methodsmentioning

confidence: 99%

Cognition and behaviour in frontotemporal dementia with and without amyotrophic lateral sclerosis

Saxon

Thompson

Harris

et al. 2020

J Neurol Neurosurg Psychiatry

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“…When assessing language ability, it is important to bear in mind that aphasia typically involves all aspects of language to varying degrees, and thus the PPA classification depends on the relative impairment. Naming and word-finding difficulties are common to all PPA variants, 25 although patients with nonfluent/agrammatic variant PPA should recognize the target word if provided. Typically, patients with nonfluent/agrammatic variant PPA are not significantly impaired on testing of repetition, word meaning, semantic association, or writing and reading of irregularly spelled words.…”

Section: Speech and Language Examinationmentioning

confidence: 99%

“…36 That being said, behavioral symptoms are more common in semantic variant PPA than in any of the other PPA variants. 25…”

Section: Clinical Presentationmentioning

confidence: 99%

Primary Progressive Aphasias and Apraxia of Speech

Botha¹,

Josephs²

2019

CONTINUUM: Lifelong Learning in Neurology

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PURPOSE OF REVIEW: This article reviews two of the primary progressive aphasias (PPAs), disorders characterized by the early and predominant impairment of language, and primary progressive apraxia of speech, a degenerative motor speech disorder that is closely related to PPA. An outline of the history and controversy surrounding how these disorders are classified is provided before the article focuses on each disorder's clinical and imaging features. RECENT FINDINGS: Over the past decade, the classification of degenerative speech and language disorders has been refined. Clinical, imaging, and pathologic evidence suggests that primary progressive apraxia of speech is a distinct degenerative disorder. Furthermore, multiple lines of evidence have highlighted issues with nonfluent/agrammatic variant PPA, which complicates the diagnosis, prognosis, and study of this disorder. Semantic variant PPA, while not without controversy, remains one of the most well-defined disorders, with good clinicopathologic correlation. SUMMARY: Accurate classification and diagnosis of these degenerative speech and language disorders is crucial in clinical practice and ongoing research efforts. For nonfluent/agrammatic variant PPA, the authors suggest emphasizing agrammatism as the core inclusion criterion and taking care not to include patients with isolated or predominant apraxia of speech. Isolated apraxia of speech can be the manifestation of a degenerative disease and, based on the different prognosis, should be recognized as distinct from PPA. Finally, it is important to recognize that some patients with semantic dementia, despite sharing the same pathologic associations, may not meet criteria for PPA.

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“…Z uwagi na wpływ zaburzeń językowych na wykonanie testów pamięci słownej szczególnie przydatna w neuropsychologicznej diagnostyce różnicowej wariantu logopenicznego i niepłynnego jest ocena pamięci materiału wzrokowo-przestrzennego z użyciem Testu Figury Złożonej Reya (Ramanan et al, 2016). W lvPPA obserwuje się nie tylko głębsze trudności z odtwarzaniem materiału po odroczeniu, ale też większe nasilenie zaburzeń pamięci bezpośredniej słownej i wzrokowej (Harris et al, 2019). Zaburzenia pamięci operacyjnej w lvPPA początkowo dotyczą głównie zadań wymagających przetwarzania materiału słownego (Foxe et al, 2013).…”

Section: Wariant Logopeniczny Afazji Pierwotnej Postępującej -Obraz Kunclassified

Progression of language and cognitive impairment in patients with the logopenic variant of primary progressive aphasia – case series

Kluj-Kozłowska¹,

Sitek²,

Skrzypkowska³

et al. 2020

Aktualn Neurol

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Aim: The paper presents longitudinal observation of four patients diagnosed with the logopenic variant of primary progressive aphasia (lvPPA). Materials and methods: The results of 3–4 language and cognitive assessments were available for four individuals (three women, one man) with lvPPA. The length of the observation period was 2–4 years. Language evaluation was comprehensive and addressed narrative speech, naming, word and sentence comprehension, repetition, reading and writing. Neuropsychological examination, whenever feasible, assessed visuospatial function, praxis, memory and executive functions. Results: Anomia was not an isolated symptom at the time of lvPPA diagnosis. Rapid deterioration of both spoken and written communication was observed. Reading aloud single words and high-frequency word comprehension were preserved longer than other linguistic competences. The narrative speech was progressively impoverished in terms of idea density, manifested by a reduced use of nouns and verbs in particular and an increased use of pronouns. At the advanced stage of the disease, the idea density was very low. There was marked deterioration of visuospatial functions, praxis and episodic memory in all patients. Progression to full-blown dementia was observed in all patients. Conclusions: The progression of linguistic and cognitive symptoms in lvPPA, albeit slightly heterogeneous, is relatively fast. The patients usually reach dementia stage within 1–2 years of the diagnosis and sometimes even earlier than 2 years of the declared symptom onset. When planning long-term management of lvPPA cases, rapid deterioration and progression to full-blown dementia within a relatively short time should be considered.

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Neuropsychological differentiation of progressive aphasic disorders

Cited by 31 publications

References 61 publications

Cognition and behaviour in frontotemporal dementia with and without amyotrophic lateral sclerosis

Cognition and behaviour in frontotemporal dementia with and without amyotrophic lateral sclerosis

Primary Progressive Aphasias and Apraxia of Speech

Progression of language and cognitive impairment in patients with the logopenic variant of primary progressive aphasia – case series

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