scholarly journals Primary Progressive Nonfluent Aphasia

2020 ◽  
Author(s):  
Author(s):  
Jennifer M. Ogar

Abstract Speech-language pathologists are increasingly treating patients with progressive disorders, including primary progressive aphasia (PPA). For many years, two variants of PPA were recognized: a nonfluent type—progressive nonfluent aphasia (PNFA)—and a fluent form—semantic dementia (SD). In 2004, a third variant—logopenic progressive aphasia (LPA)—was described. This article will review clinical symptoms, neuroimaging correlates, and the neuropathologies that are typically associated with PNFA, SD, and LPA. Case studies are included to further illustrate the characteristics of each of these three PPA variants.


2014 ◽  
Vol 8 (3) ◽  
pp. 291-296 ◽  
Author(s):  
Thais Helena Machado ◽  
Aline Carvalho Campanha ◽  
Paulo Caramelli ◽  
Maria Teresa Carthery-Goulart

The non-fluent and agrammatic variant of Primary Progressive Aphasia (NFPPA) is characterized by reduced verbal production with deficits in building grammatically correct sentences, involving dysfunctions in syntactic and morphological levels of language. There are a growing number of studies about non-pharmacological alternatives focusing on the rehabilitation of functional aspects or specific cognitive impairments of each variant of PPA. This study reports a short-term treatment administered to a patient with NFPPA focusing on the production of sentences. The patient had significant reduction in verbal fluency, use of keywords, phrasal and grammatical simplifying as well as anomia. Using the method of errorless learning, six sessions were structured to stimulate the formation of sentences in the present and past with the cloze technique. The patient had improvement restricted to the strategy, with 100% accuracy on the trained phrases and generalization to untrained similar syntactic structure after training. These results persisted one month after the treatment.


Author(s):  
Raksha Anand ◽  
John Hart ◽  
Patricia S. Moore ◽  
Sandra B. Chapman

Abstract Purpose: Frontotemporal lobar degeneration (FTLD) encompasses a group of neurodegenerative disorders characterized by gradual and progressive decline in behavior and/or language. Identifying the subtypes of FTLD can be challenging with traditional assessment tools. Growing empirical evidence suggests that language measures might be useful in differentiating FTLD subtypes. Method: In this paper, we examined the performance of five individuals with FTLD (two with frontotemporal dementia, two with semantic dementia, and one with progressive nonfluent aphasia) and 10 cognitively normal older adults on measures of semantic binding (Semantic Object Retrieval Test and semantic problem solving) and abstracted meaning (generation of interpretive statement and proverb interpretation). Results and Conclusion: A differential profile of impairment was observed in the three FTLD subtypes on these four measures. Further examination of these measures in larger groups will establish their clinical utility in differentiating the FTLD subtypes.


Author(s):  
Jacqueline Dominguez ◽  
Arlene Ng ◽  
Jeryl Yu ◽  
Anne Cristine Guevarra ◽  
Maria Luisa Daroy ◽  
...  

<b><i>Background:</i></b> Compared to Western populations, familial frontotemporal lobar degeneration (FTLD) is rare among Asians. Progranulin (GRN) gene mutation, which is a major cause of FTLD, is likewise rare. We present a family with FTLD from the Philippines with an autosomal dominant pattern of inheritance and GRN mutation and briefly review reports of GRN mutations in Asia. <b><i>Case Presentation:</i></b> The proband is 66 years old with progressive nonfluent aphasia (PNFA)-corticobasal syndrome . We assessed 3 generations of her pedigree and found 11 affected relatives with heterogenous phenotypes, usually behavioral variant frontotemporal dementia (FTD) and PNFA. Neuroimaging showed atrophy and hypometabolism consistent with FTD syndromes. White matter hyperintensities were seen in affected members even in the absence of vascular risk factors. A GRN mutation R110X was found in 6 members, 3 with symptoms and 3 were asymptomatic. Plasma GRN was low (&#x3c;112 ng/mL) in all mutation carriers. No mutations were found in microtubule-associated protein tau, APP, PSEN1, and PSEN2 genes, and all were APOE3. <b><i>Conclusion:</i></b> This is the first Filipino family with autosomal dominant FTD documented with GRN mutation. Identifying families and cohorts would contribute to therapeutic developments in an area with FTD-GRN.


Author(s):  
Melanie Fried-Oken ◽  
Charity Rowland ◽  
Chris Gibbons

Abstract Augmentative and alternative communication (AAC) intervention offers people diagnosed with progressive nonfluent aphasia (PNFA) an opportunity to continue to communicate even as verbal expression declines. To date, there are no well-controlled studies reporting the effectiveness of AAC intervention with people who present with PNFA. Further, there is a pressing need for evidence about specific AAC intervention tools, techniques, and training protocols for persons with PNFA and their communication partners. We have engaged in research studies at the Oregon Health & Science University to quantify low-tech AAC supports for people with PNFA in highly controlled, as well as naturalistic, dyadic conversations. Preliminary results suggest that AAC provides strong lexical support for people with PNFA during conversation. We predict that training participants and their partners how to use personalized, low-tech communication boards will lead to reduced conversational scaffolding by partners and prolonged effective communication as the disease course progresses. Clinical implications and future directions of our research are discussed.


Aphasiology ◽  
2012 ◽  
Vol 26 (3-4) ◽  
pp. 404-427 ◽  
Author(s):  
Jamie Reilly ◽  
Joshua Troche ◽  
Alison Paris ◽  
Hyejin Park ◽  
Michelene Kalinyak-Fliszar ◽  
...  

2006 ◽  
Vol 17 (2) ◽  
pp. 77-87 ◽  
Author(s):  
Serena Amici ◽  
Maria Luisa Gorno-Tempini ◽  
Jennifer M. Ogar ◽  
Nina F. Dronkers ◽  
Bruce L. Miller

We present a review of the literature on Primary Progressive Aphasia (PPA) together with the analysis of neuropschychological and neuroradiologic profiles of 42 PPA patients. Mesulam originally defined PPA as a progressive degenerative disorder characterized by isolated language impairment for at least two years. The most common variants of PPA are: (1) Progressive nonfluent aphasia (PNFA), (2) semantic dementia (SD), (3) logopenic progressive aphasia (LPA). PNFA is characterized by labored speech, agrammatism in production, and/or comprehension. In some cases the syndrome begins with isolated deficits in speech. SD patients typically present with loss of word and object meaning and surface dyslexia. LPA patients have word-finding difficulties, syntactically simple but accurate language output and impaired sentence comprehension. The neuropsychological data demonstrated that SD patients show the most characteristic pattern of impairment, while PNFA and LPA overlap within many cognitive domains. The neuroimaging analysis showed left perisylvian region involvement. A comprehensive cognitive, neuroimaging and pathological approach is necessary to identify the clinical and pathogenetic features of different PPA variants.


Sign in / Sign up

Export Citation Format

Share Document