In this study, we report on an individual with a behavioral. Clinical, anatomical, and pathological features in the. Nonfluent, semantic, and logopenic are the currently recognized clinical variants. The logopenic variant of primary progressive aphasia, or logopenic aphasia, is a the most recently described. Henry ml, gornotempini ml 2010 the logopenic variant of primary progressive aphasia.
Barbara costa beber1,2,3, renata kochhann1,4,5, bruna matias da silva1, marcia l. The recommendations for the classification of primaryprogressive aphasia have advocated variantspecific patterns of atrophy. All underwent f18fdg metabolic and c11pib amyloid pet brain imaging. Thus, these individuals have difficulty repeating brief phrases, and have difficulty repeating a short sequence of numbers. Little is known regarding the overall life expectancy of individuals with logopenic progressive aphasia. Progranulinassociated pibnegative logopenic primary. I clinical, ii imagingsupported, iii definite pathologic diagnosis. Neuroimaging in the differential diagnosis of primary. Neuropsychological assessment of primary progressive.
Phonological processing in primary progressive aphasia. Primaryprogressive aphasia ppa is a clinically and pathologically. Scans were outlined using a rapid semiautomated technique which involves interactive selection of thresholds, followed by a series of erosions and dilations. Fdgpet helps detect alzheimers disease diagnostic imaging. Logopenic primary progressive aphasia is an atypical variant of alzheimers disease characterized by decreased rate of spontaneous language production. Mri scans were performed with a standardized imaging protocol at 3t. Current ppa criteria divide ppa into three variants.
Aphasia is an inability to comprehend or formulate language because of damage to specific brain regions. Logopenic variant of primary progressive aphasia logopenic. As with the other variants of ppa, the logopenic variant is considered an early onset form of dementia 2. Hodges2,3,4 1 faculty of health science, the university of sydney, sydney, new south wales, australia, 2 neuroscience research australia. Splitting the two groups showed that logopenic aphasia due to probable alzheimer pathology is a more aggressive variant characterized by more extensive languagecognitive disorders affecting, in addition to lexical processes. Logopenic and nonfluent variants of primary progressive aphasia are differentiated by acoustic measures of speech production kirrie j. The logopenic variant of primary progressive aphasia is an atypical clinical variant of alzheimers disease which is typically characterized by left temporoparietal atrophy on magnetic resonance imaging and hypometabolism on f18 fluorodeoxyglucose positron emission tomography. Logopenic progressive aphasia lpa, or the logopenic variant of primary progressive aphasia ppa, is a language impairment that is one of the known variant presentations of earlyonset ad 1.
Logopenic primary progressive aphasia springerlink. The logopenic variant of primary progressive aphasia needs to be differentiated from the. Affected individuals have slow, hesitant speech due to difficulty retrieving the correct words, names, or numbers. In 2011, a group of experts 2 developed consen sus criteria that recognized three dis. Frontotemporal dementias are neurodegenerative diseases in which symptoms of frontal andor temporal lobe disease are the first signs of the illness, and as the diseases. Initial image analysis was performed using the midas software freeborough. I clinical, ii imaging supported, iii definite pathologic diagnosis. This dementia is associated with alzheimers disease in the majority of cases.
It is similar to conduction aphasia and is associated with atrophy to the left posterior temporal cortex and inferior parietal lobule. The classification of ppa into one of the three variants may be performed at 3 levels. Primary progressive aphasia radiology reference article. To be diagnosed with aphasia, a persons speech or language must be significantly impaired in one or several of the four communication. Leyton ce, piguet o, savage s, burrell j, hodges jr. Phonological processing in primary progressive aphasia introduction primary progressive aphasia ppa is a debilitating condition wherein speech and language deteriorate as a result of neurodegenerative disease. Several primary features characterize the logopenic variant of primary progressive aphasia ppa. Logopenic variant ppa primary progressive aphasia aftd. Petmr imaging demonstrates cortical atrophy and corresponding hypometabolism, typically most severe in the left lateral temporoparietal region. This yields a region which separates brain from surrounding csf, skull and dura. Aug 08, 2014 primary progressive aphasia ppa is a progressive language disorder associated with atrophy of the dominant language hemisphere, typically left. It is an insidious disorder characterized by gradual impairment of speech and language that predates, usually by many years, the onset of more global cognitive. Speech is fluent but does not meet criteria for logopenic progressive aphasia or semantic dementia.
Generally logopenic dementia is considered one of three subtypes of primary progressive aphasia, along with semantic dementia and progressive nonfluent aphasia clinical presentation. Can mri visual assessment differentiate the variants of. Probabilistic tracto graphy was used to identify bilateral inferior longitudinal anterior, middle, posterior and uncinate fasciculi referred to as the. Jan 18, 20 little is known regarding the overall life expectancy of individuals with logopenic progressive aphasia. We aimed to characterize and compare patterns of atrophy and hypometabolism in logopenic primary. Microbleeds in the logopenic variant of primary progressive. Fdg pet and mri in logopenic primary progressive aphasia. Due to the nature of neurodegenerative disorders, patients with primary progressive aphasia develop cognitive impairment other than aphasia as the disorder progresses.
Within primary progressive aphasia the logopenic variant remains less. Logopenic aphasia or alzheimers disease dementia e. Patients with logopenic variant display impaired singleword retrieval both on confrontation naming tasks as well as in speech samples e. Aphasia is present, and core criteria for ppa are met gornotempini et al. Positive effects of language treatment for the logopenic. Working memory and language network dysfunctions in logopenic. Logopenic progressive aphasia lpa is a type of dementia characterized by language disturbance, including difficulty making or understanding speech. A healthcare providers guide to the logopenic variant of. Differentiation between subtypes of primary progressive. To fit our multivariate models we used the glmnet software package and. Hodges2,3,4 1 faculty of health science, the university of sydney, sydney, new south wales, australia, 2 neuroscience research australia, randwick, new south.
In particular, praxic disorders and semantic memory deficits have rarely been reported. In this study, we report on an individual with a behavioral profile and cortical atrophy. Clinical and neuroimaging biomarkers of amyloidnegative. Verbal and visuospatial span in logopenic progressive aphasia and alzheimers disease. Primary progressive aphasia is a neurodegenerative disorder that was recently classified into three types. Sincere thanks for this ongoing support for our members shibley. The logopenic variant of primary progressive aphasia, or logopenic aphasia, is a.
Progression of logopenic variant primary progressive aphasia. There are few studies examining praxis in subjects with primary progressive aphasia. To capture patients with progressive aphasia or aos, the following search terms were used. The progression of logopenic variant primary progressive aphasia lvppa, however, has not been well described. As such, grammar, syntax, average phrase length, and prosody are normal. A middleaged woman with logopenic progressive aphasia as a. The logopenic variant of primary progressive aphasia ppa is characterised by. The criteria for lpa include the presence of both impaired singleword retrieval in spontaneous speech and naming and impaired repetition of sentences. Patterns of longitudinal brain atrophy in the logopenic variant of. It is suspected that an atypical form of alzheimers disease is the most common cause of logopenic. It is important to note, that the terminology of, and classification, of these related conditions is evolving and thus variable across the literature 2,3. The aims of the present study were to evaluate the sensitivity and. Hippocampal atrophy is the most wellestablished imaging biomarker for ad.
Taskfree functional magnetic resonance imaging fmri results for the left working memory network. Clinical amyloid imaging in logopenic progressive aphasia article in alzheimer disease and associated disorders 291 august 20 with 12 reads how we measure reads. We aimed to characterize and compare patterns of atrophy and hypometabolism. Teichmann m1, kas a, boutet c, ferrieux s, nogues m, samri d, rogan c, dormont d, dubois b, migliaccio r. Image analysis was performed using the midas software package freeborough et al. Primary progressive aphasia was first described in 1982 and is the umbrella term for the languagevariant frontotemporal dementias associated with frontotemporal lobar degeneration ftld 3 clinical presentation. However, a third clinical entity logopaenic dementia has since been characterized, which actually represents an uncommon clinical variant of alzheimer disease 1,2,4. Feb 21, 2020 aphasia is present, and core criteria for ppa are met gornotempini et al. Logopenic progressive aphasia lpa is a form of primary progressive aphasia. The logopenic variant of primary progressive aphasia lvppa is a progressive language disorder in which patients have anomia, difficulty retrieving words and repeating complex sentences and phonological errors in their spoken speech gornotempini et al. Can mri visual assessment differentiate the variants of primary. The logopenic variant is the least common one and is closely related to alzheimers disease in comparison to the other two variants that are closely related to frontotemporal dementia.
From the department of neurology, department of nuclear medicine, neurosurgical research institute, school of medicine, and department of radiology. Mr imaging was least sensitive for the diagnosis of non. Logopenic aphasia, 19 subjects nonfluent aphasia, 16 subjects semantic aphasia, subjects corticobasal syndrome, 14 subjects alzheimers disease, 24 subjects. In contrast to patients with the agrammatic and semantic variants, the majority of subjects with the. Microbleeds have been associated with alzheimers disease ad, although it is unclear whether they occur in atypical presentations of ad, such as the logopenic variant of primary progressive aphasia lvppa. It is defined clinically by impairments in naming and sentence repetition.
Logopenic aphasia or alzheimers disease different phases of the same disease. Objectives the logopenic variant of primary progressive aphasia is an atypical clinical variant of alzheimers disease which is typically characterized by left temporoparietal atrophy on magnetic resonance imaging and hypometabolism on f18 fluorodeoxyglucose positron emission tomography. Deficits are the principal cause of impaired daily living activities c. Since the modern description of primary progressive aphasia ppa more than 30 years ago, the interest in neurodegenerative conditions that selectively target the language network has. Clinical amyloid imaging in logopenic progressive aphasia. Three variants of ppa are now recognized, each of which shows a unique constellation of speechlanguage deficits and pattern. Logopenic progressive aphasia genetic and rare diseases. Primary progressive aphasia syndromes were originally classified as either fluent semantic dementia or nonfluent progressive nonfluent aphasia.
Fluorodeoxyglucose f18 positron emission tomography in. This presenting syndrome has been referred to as the logopenic variant of primary progressive aphasia lvppa, or logopenic progressive aphasia 10,11. Jan 01, 2014 background microbleeds have been associated with alzheimers disease ad, although it is unclear whether they occur in atypical presentations of ad, such as the logopenic variant of primary progressive aphasia lvppa. In terms of neuroimaging, techniques such as diffusion imaging and.
Mr imaging was least sensitive for the diagnosis of nonfluentvariant. Despite considerable recent progress in understanding the underlying neurobiology of primary progressive aphasia ppa syndromes, relatively little attention has been directed toward the examination of behavioral interventions that may lessen the pervasive communication problems associated with ppa. Most prominent clinical feature is difficulty with language b. This is the short version of a video in week 2 of the online course the many faces of dementia. Frontotemporal dementia and primary progressive aphasia, a. The sample included 122 fdgpet imaging studies from 91 patients with ppa. Sep 16, 2015 logopenic aphasia, 19 subjects nonfluent aphasia, 16 subjects semantic aphasia, subjects corticobasal syndrome, 14 subjects alzheimers disease, 24 subjects. Ftd syndromes characterized predominantly by the gradual loss of the ability to speak, read, write and understand what others are saying fall under the category of primary progressive aphasia, or ppa. Regional cerebral blood flow spect and mri in logopenic vari.
Madhavan a, whitwell jl, weigand sd, duffy jr, strand ea, et al. A healthcare providers guide to the logopenic variant of primary. Jun 28, 2011 despite considerable recent progress in understanding the underlying neurobiology of primary progressive aphasia ppa syndromes, relatively little attention has been directed toward the examination of behavioral interventions that may lessen the pervasive communication problems associated with ppa. The new classification of primary progressive aphasia into. Working memory and language network dysfunctions in. A middleaged woman with logopenic progressive aphasia as. Regions showing decreased connectivity in logopenic primary progressive aphasia lvppa compared with the controls and dementia of the alzheimers type dat compared with the controls are shown corrected for multiple comparisons at p feb 28, 2016 jenny describes her experience of living with a diagnosis of logopenic aphasia. Regional cerebral blood flow spect and mri in logopenic. It is suspected that an atypical form of alzheimers disease is the most common cause of. We aimed to assess the presence and clinical correlates of microbleeds in lvppa. Many of our members have ppa or lppa, and this particular article will be particularly continue reading february research wrap. Regions showing decreased connectivity in logopenic primary progressive aphasia lvppa compared with the controls and dementia of the alzheimers type dat compared with the controls are shown corrected for multiple comparisons at p wrap. Positive effects of language treatment for the logopenic variant of primary progressive aphasia. This damage is typically caused by a cerebral vascular accident, or head trauma.
After taking into consideration the clinical presentation and imaging findings on mri and spect, the diagnosis of the logopenic variant of primary progressive aphasia was finally reached. The logopenic variant of primary progressive aphasia is an atypical clinical. We report the case of a middleaged woman who presented. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. The aim of this study was to examine the pattern and severity of ideomotor apraxia in subjects with logopenic and agrammatic variants of primary progressive aphasia and to determine if the presence of ideomotor apraxia correlated with specific neuroanatomical structural abnormalities. Objectives to determine patterns of hypometabolism on fluorodeoxyglucose f18 positron emission tomography fdgpet in patients with progressive apraxia of speech pas and primary progressive aphasia ppa variants and to use these patterns to further refine current classification design we identified all patients who had fdgpet and pas or ppa who were.
A high proportion of lvppa subjects have ad pathology 12. There is a gradual impairment of language not just speech. Logopenic and nonfluent variants of primary progressive. Western aphasia batteryrevised profiles in primary. The new classification of primary progressive aphasia into semantic, logopenic, or nonfluentagrammatic variants michael f. Primary progressive aphasia ppa is a language disorder that is characterized by deficits in functions such as object naming, syntax, and wordprocessing. Objectivesthe logopenic variant of primary progressive aphasia is an atypical clinical variant of alzheimers disease which is typically characterized by left temporoparietal atrophy on magnetic resonance imaging and hypometabolism on f18 fluorodeoxyglucose positron emission tomography.
Neuropsychological findings and the logopenic variant of. This month, we have just made it to post our research wrap for february, but with a mega effort by dr shibley rahman in writing this today, we have made it. One characteristic feature is profound difficulty with repetition and very shortterm memory. White matter damage in primary progressive aphasias. Primaryprogressive aphasia is a clinically and pathologically heterogeneous condition. Ideomotor apraxia in agrammatic and logopenic variants of. Axial flair brain mri image reveals bilateral hippocampal re. Differentiation between subtypes of primary progressive aphasia agosta et al p rimary progressive aphasia ppa is a relatively rare neurodegenerative syndrome in which language impairment is the presenting and most salient feature 1.