Progressive Apraxia Of Speech Research Articles

Primary Progressive Apraxia of Speech (PAS): A Distinct Neurodegenerative Syndrome (P07.167)

Primary Progressive Apraxia of Speech (PAS): A Distinct Neurodegenerative Syndrome (P07.167)

Progressive Crossed-Apraxia of Speech as a First Manifestation of a Probable Corticobasal Degeneration

We present the longitudinal neurolinguistic, neuropsychological and neurologic follow-up of a 64 y.o. right-handed woman, who developed progressive apraxia of speech (PAOS), followed by peripheral agraphia then a left corticobasal syndrome (CBS). Neuroimaging (CT, MRI and FDG-PET) unequivocally showed progressive right hemispheric atrophy and hypometabolism. This particular evolution first confirms that PAOS is a phenotype of probable corticobasal degeneration (CBD). More importantly, this case underpins the neural organisation of motor planning processing in relation with speech, as well as graphic and limb praxis impairments, and constitutes a rare example of crossed-PAOS.

Progressive crossed-apraxia of speech as a first manifestation of a probable corticobasal degeneration.

We present the longitudinal neurolinguistic, neuropsychological and neurologic follow-up of a 64 y.o. right-handed woman, who developed progressive apraxia of speech (PAOS), followed by peripheral agraphia then a left corticobasal syndrome (CBS). Neuroimaging (CT, MRI and FDG-PET) unequivocally showed progressive right hemispheric atrophy and hypometabolism. This particular evolution first confirms that PAOS is a phenotype of probable corticobasal degeneration (CBD). More importantly, this case underpins the neural organisation of motor planning processing in relation with speech, as well as graphic and limb praxis impairments, and constitutes a rare example of crossed-PAOS.

Progressive apraxia of speech as a window into the study of speech planning processes

We present a 3-year follow-up study of a patient with progressive apraxia of speech (PAoS), aimed at investigating whether the theoretical organization of phonetic encoding is reflected in the progressive disruption of speech. As decreased speech rate was the most striking pattern of disruption during the first 2 years, durational analyses were carried out longitudinally on syllables excised from spontaneous, repetition and reading speech samples. The crucial result of the present study is the demonstration of an effect of syllable frequency on duration: the progressive disruption of articulation rate did not affect all syllables in the same way, but followed a gradient that was function of the frequency of use of syllable-sized motor programs. The combination of data from this case of PAoS with previous psycholinguistic and neurolinguistic data, points to a frequency organization of syllable-sized speech-motor plans. In this study we also illustrate how studying PAoS can be exploited in theoretical and clinical investigations of phonetic encoding as it represents a unique opportunity to investigate speech while it progressively disrupts.

Corticobasal Ganglionic Degeneration and Progressive Supranuclear Palsy: Clinical and Speech-Language Characteristics

AbstractPurpose: The purpose of this article is to present a brief description of the neuroanatomical correlates, pathophysiology, and clinical presentation of corticobasal ganglionic degeneration, (often referred to as corticobasal degeneration, or CBD) and progressive supranuclear palsy (PSP).Methods: Descriptions of the clinical characteristics of the two degenerative syndromes are presented, including a discussion of the similarities and differences. The speech and language disorders associated with each are described, including progressive aphasia, progressive non-fluent aphasia (PNFA), progressive apraxia of speech (AOS), and dysarthria.Results and Conclusions: CBD and PSP have a number of commonalities in clinical characteristics and in pathology. Both progressive aphasia and apraxia of speech have been associated with CBD and PSP. Clinicians who evaluate patients exhibiting a progressive neurologic disease and who identify either progressive aphasia, PNFA and/or progressive AOS should consider CBD or PSP as a possible medical diagnosis.

Fluorodeoxyglucose F18 Positron Emission Tomography in Progressive Apraxia of Speech and Primary Progressive Aphasia Variants

To determine patterns of hypometabolism on fluorodeoxyglucose F18 positron emission tomography (FDG-PET) in patients with progressive apraxia of speech (PAS) and primary progressive aphasia (PPA) variants and to use these patterns to further refine current classification. We identified all patients who had FDG-PET and PAS or PPA who were evaluated by an expert speech-language pathologist. Patterns of hypometabolism were independently classified by 2 raters blinded to clinical data. Three speech-language pathologists reclassified all patients into 1 of 7 operationally defined categories of PAS and PPA blinded to FDG-PET data. Tertiary care medical center. Twenty-four patients with PAS or PPA and FDG-PET. Fluorodeoxyglucose F18 PET hypometabolic pattern. Of the 24 patients in the study, 9 had nonfluent speech output; 14, fluent speech; and 1 was unclassifiable. Twenty-one patients showed FDG hypometabolism; the remaining 3 did not. Among the patients showing hypometabolism, 8 had a prerolandic pattern of which 7 had nonfluent speech including progressive nonfluent aphasia (n = 3), PAS (n = 1), and mixed nonfluent aphasia/apraxia of speech (n = 3); the other patient had PPA unclassifiable. The remaining 13 had a postrolandic pattern, all with fluent speech (P < .001), including logopenic progressive aphasia (n = 6), progressive fluent aphasia (n = 6), and semantic dementia (n = 1). Patterns of hypometabolism differed between the nonfluent variants and between the fluent variants, including progressive fluent aphasia. Patterns of FDG-PET hypometabolism support the clinical categorizations of fluency, the distinction of apraxia of speech from progressive nonfluent aphasia, and the designation of a progressive fluent aphasia category.

Making sense of progressive non-fluent aphasia: an analysis of conversational speech

The speech of patients with progressive non-fluent aphasia (PNFA) has often been described clinically, but these descriptions lack support from quantitative data. The clinical classification of the progressive aphasic syndromes is also debated. This study selected 15 patients with progressive aphasia on broad criteria, excluding only those with clear semantic dementia. It aimed to provide a detailed quantitative description of their conversational speech, along with cognitive testing and visual rating of structural brain imaging, and to examine which, if any features were consistently present throughout the group; as well as looking for sub-syndromic associations between these features. A consistent increase in grammatical and speech sound errors and a simplification of spoken syntax relative to age-matched controls were observed, though telegraphic speech was rare; slow speech was common but not universal. Almost all patients showed impairments in picture naming, syntactic comprehension and executive function. The degree to which speech was affected was independent of the severity of the other cognitive deficits. A partial dissociation was also observed between slow speech with simplified grammar on the one hand, and grammatical and speech sound errors on the other. Overlap between these sets of impairments was however, the rule rather than the exception, producing continuous variation within a single consistent syndrome. The distribution of atrophy was remarkably variable, with frontal, temporal and medial temporal areas affected, either symmetrically or asymmetrically. The study suggests that PNFA is a coherent, well-defined syndrome and that varieties such as logopaenic progressive aphasia and progressive apraxia of speech may be seen as points in a space of continuous variation within progressive non-fluent aphasia.

Apraxia of speech and nonfluent aphasia: a new clinical marker for corticobasal degeneration and progressive supranuclear palsy

To highlight the fact that patients with corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) can sometimes present with a progressive apraxia of speech, nonfluent aphasia, or a combination of the two disorders. Corticobasal degeneration and PSP are neurodegenerative diseases characterized by neuronal loss and gliosis in cardinal brain regions, as well as the abnormal deposition of the microtubule associated protein tau in cell bodies and cell processes. The typical presenting features of CBD and PSP are akinesia and rigidity that are levodopa unresponsive, although there has been evidence that both diseases, moreso CBD, can present with a dementia syndrome. Recent clinicopathological studies have now also demonstrated that a subset of patients with CBD and PSP present with a progressive apraxia of speech, nonfluent aphasia, or a combination of the two disorders. Presenting features of progressive apraxia of speech or nonfluent aphasia are strongly associated with a diagnosis of CBD, PSP, or both.

Motor Speech Disorders and the Diagnosis of Neurologic Disease

Motor Speech Disorders and the Diagnosis of Neurologic Disease

Progressive Apraxia of Speech as a Sign of Motor Neuron Disease

To document and describe in detail the occurrence of apraxia of speech (AOS) in a group of individuals with a diagnosis of motor neuron disease (MND). Seven individuals with MND and AOS were identified from among 80 patients with a variety of neurodegenerative diseases and AOS (J. R. Duffy, 2006). The history, presenting complaints, neurological findings, and speech-language findings were documented for each case. Spastic or mixed spastic-flaccid dysarthria was present in all 7 cases. The AOS was judged as worse than the dysarthria in 4 cases. Nonverbal oral apraxia was eventually present in all cases. Aphasia was present in 2 cases and equivocally present in another 2. Dementia was present in 1 case and equivocally present in 2. AOS can occur in MND, typically also with dysarthria, but not invariably with aphasia or other cognitive deficits. Thus, a diagnosis of MND does not preclude the presence of AOS. More importantly, MND should be a diagnostic consideration when AOS is a prominent sign of degenerative disease.