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PubMed1994

The pathology and nosology of primary progressive aphasia.

We present three cases of primary progressive aphasia (PPA) with Pick-variant pathology to support a hypothesis of an underlying nosologic relatedness. Neuropathologic examination demonstrated focal brain atrophy with corresponding neuronal loss and gliosis, accompanied by superficial spongiosis. Specific histologic findings were ballooned neurons (Pick cells) in the atrophic areas, and in two of the cases, Pick bodies. They were immunoreactive for tau. In contrast to classic Pick's disease, there were no Pick bodies in the hippocampus. The intense neurofilament immunoreactivity of the perikarya of the ballooned neurons greatly facilitated their recognition. Based on our cases and a critical review of the literature, we hypothesize that the common underlying pathology of PPA is a variant of Pick's disease. Furthermore, we propose the concept of "Pick complex" to include other neurodegenerative diseases characterized by focal cortical degeneration, such as PPA, frontal lobe dementia, ALS with PPA, and corticonigral and corticobasal ganglionic degenerations.

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PubMed1996

Clinical and pathological characteristics of primary progressive aphasia and frontal dementia.

The recently described conditions of primary progressive aphasia and frontal lobe dementia overlap clinically and pathologically to a considerable extent with each other and with clinical and pathological descriptions of Pick's disease. Our clinical and neuropathological experience is summarized, leading us to the conclusion that the degree of overlap justifies the concept of "Pick complex" to include, in addition to the above, corticobasal ganglionic degeneration and some instances motor neuron disease with dementia.

Aphasia, Primary Progressive↗
PubMed2003

Primary progressive aphasia as the initial manifestation of corticobasal degeneration and unusual tauopathies.

The clinical, neuroradiological, neuropathological and biochemical findings in four patients with primary progressive aphasia and tauopathy are described. The aphasic syndrome preceded by several years the appearance of other symptoms in every case. Asymmetrical apraxia with alien hand phenomenon occurred in one case. Frontotemporal symptoms occurred in three cases, but progressed to dramatic cognitive devastation in only one of these. Generalized dementia consistent with probable Alzheimer's disease (AD) developed with time in another. Cerebral computer tomography scans, magnetic resonance imaging and SPECT studies revealed marked asymmetries in one case, and showed nonspecific cerebral atrophy in the remaining ones. The neuropathological examination revealed typical corticobasal degeneration (CBD) in one case; CBD and AD in another; and atypical CBD, argyrophilic grain disease (AGD) and alpha-synucleinopathy consistent with Parkinson's disease in a third. Unique neuropathological findings were found in the remaining case. This was characterized by severe cerebral atrophy, marked neuronal loss in the cerebral cortex and abnormal tau deposition in neurons of the cerebral cortex, diencephalon and brain stem. Ballooned neurons, Pick bodies, generalized cortical neurofibrillary tangles and astrocytic plaques were absent. However, massive globular inclusions, containing phospho-tau, occurred in glial cells, mainly oligodendrocytes, in the white matter. Biochemical studies of frontal homogenates revealed four bands of 73/74, 68, 64 and 60 kDa of phosphorylated tau (using antibodies recognizing phospho-tau Thr181, Ser262 and Ser422) in the patient with AD and CBD, suggesting a predominant AD pattern in this case. Two bands of 68 and 64 kDa of phospho-tau were recovered in the sarkosyl-insoluble fraction in the other three cases. This pattern is similar to that found in CBD, progressive supranuclear palsy and AGD. Taken together, the present series further supports pure and combined CBD as causes of primary progressive aphasia, and they extend the hypothesis that primary progressive aphasia may be the initial symptom of distinct tauopathies.

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PubMed2005

Paradoxical features of word finding difficulty in primary progressive aphasia.

Impaired word retrieval is a main symptom of primary progressive aphasia (PPA). The cognitive features of this impairment in PPA are poorly understood. We studied 12 patients with PPA (6 English-speaking and 6 Dutch-speaking), 7 patients with early-stage clinically probable Alzheimer's disease (PRAD), 5 patients with mild cognitive impairment (MCI), and 15 age-matched, cognitively intact, control subjects. Subjects had to name a picture (the probe), which was preceded by a written word (the prime) that could be the correct name of the picture, a noun belonging to the same semantic subcategory (related prime), a semantically unrelated noun (unrelated prime), or a pseudoword (neutral control). Naming latencies were longer in PPA and PRAD patients than in control subjects. Critically, the interaction between group and prime type was highly significant. PPA patients named the probe more slowly after a related compared with an unrelated prime. In contrast, PRAD patients, mild cognitive impairment patients, and healthy control subjects tended to name the probe faster when it was preceded by a related prime. The semantic interference effect in PPA generalized across languages and PPA subtypes. Selection among competing word forms sharing a same semantic field is abnormal in PPA. The semantic interference effect constitutes a positive distinguishing feature between PPA and PRAD.

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PubMed1990

Primary progressive aphasia. Longitudinal course, neuropsychological profile, and language features.

Four patients with the clinical syndrome of primary progressive aphasia and a nonfluent aphasia profile were followed up over a period of 3 to 5 years. Extensive neuropsychological data for three patients revealed a progressive, quantitative decline of language with relative stability of memory, visuospatial skills, and reasoning. Comportment and most activities of daily living were preserved even when speech was unintelligible. Although several aphasia types may be associated with primary progressive aphasia, a nonfluent aphasia profile and phonemic paraphasic errors are most useful in differentiating it from the much more common clinical syndrome, "probable Alzheimer's disease." The clinicopathological correlates of probable Alzheimer's disease differ from those associated with primary progressive aphasia. Therefore, the clinical distinction between the two syndromes may be important for predicting the underlying pathophysiologic changes during the life of the patient.

Adult↗
PubMedDate not supplied

Semantic dementia and primary progressive aphasia: a problem of categorization?

The relationship between semantic dementia (SD) and primary progressive aphasia (PPA) has been the subject of debate ever since the syndromes were first described, in converging streams of research from the neuropsychological and neurologic communities. The most salient clinical features of SD are anomia with circumlocution and semantic paraphasia, single-word comprehension deficit, and reduced category fluency. Of critical importance is the fact that patients also show deficits on non-verbal tasks using visual, auditory, and other modalities, suggesting that the key impairment in SD is a breakdown in conceptual knowledge rather than a specific problem with language. The finding of item consistency between the various tests supports this view. The order in which the features appear can be explained by the variable degree of redundancy in access to semantic knowledge from the different perceptual modalities. Atrophy is seen in the anterior and inferior temporal lobe rather than in classic language areas, further distancing SD from aphasic syndromes. Semantic dementia and progressive non-fluent aphasia (PNFA) share some clinical and pathologic characteristics with frontal variant frontotemporal dementia, but there are also clear differences between the three syndromes. We believe that many patients described as having fluent primary progressive aphasia in fact have early SD. Semantic dementia is a well-defined syndrome, distinct from PNFA but related to it within the spectrum of frontotemporal lobar degeneration syndromes.

Aphasia, Primary Progressive↗
PubMed2005

Primary progressive aphasia: reversed asymmetry of atrophy and right hemisphere language dominance.

Primary progressive aphasia (PPA) is almost always associated with asymmetrical left hemisphere degeneration. The authors report that right hemisphere atrophy in a patient with PPA was associated with atypical right hemisphere dominance for language. This suggests that neuronal damage in PPA is therefore tightly linked to the underlying anatomy of the language network.

Aphasia, Primary Progressive↗
PubMed1999

Primary progressive aphasia presenting as conduction aphasia.

We report a case of a woman with primary progressive aphasia (PPA) who presented with conduction aphasia. A 60-year-old, right-handed, Japanese female suffering from progressive aphasia had difficulty in repeating words and phrases. She displayed phonemic paraphasias but had preserved comprehension and had no cognitive or behavior disorder for more than 6 years after the onset of the condition. She was able to continue to work successfully and to perform all her normal daily activities. T1-weighted magnetic resonance imaging revealed minute dilatation of the left inferior horn and sulci in the left hemisphere, and positron emission tomography revealed mild hypometabolism in the left supramarginal gyrus and its surrounding areas. Therefore, she was diagnosed as suffering from PPA presenting as conduction aphasia. We believe that the progressive conduction aphasia of the patient belongs to one of the fluent forms of PPA, and the ability to continue normal work along with the clinical portrayal of preserved memory and cognition skills may be features of a form of PPA presenting as conduction aphasia.

Aphasia, Conduction↗
PubMedDate not supplied

[Primary progressive aphasia: its clinical variability: an analysis of 15 cases].

INTRODUCTION: Primary progressive aphasia (PPA) represents a clinical syndrome linked to multiple degenerative diseases. The diagnosis of PPA is made when language is the only area of salient and progressive dysfunction for at least the first two years of the disease. AIM: To evaluate the neuropsychological, neuropsychiatric and language characteristics of the PPA. PATIENTS AND METHODS: 15 patients with PPA underwent language, neuropsychological and neuropsychiatric evaluation, magnetic resonance imaging, computerized tomography and single photon emission computerized tomography. RESULTS: We observed a clear distinction between the oral expression patterns; the patients were classificated by type of aphasia. The most common sign of PPA was a word finding deficit, also known as anomia. Seven aphasia type Broca, four sensorial transcortical aphasia, two aphasia type Wernicke and two anomic aphasia were found in our patients. Depression, apathy, anxiety and irritability were the most prevalent neuropsychiatric sign. CONCLUSIONS: PPA is a language-based syndrome, that include fluent (normal articulation, flow and number of words per utterance) and nonfluent form of aphasia. It has been considered a cognitive term, however, PPA is associated with high prevalence of psychiatric manifestations. More research it will be necessary to evaluate the prognostic value of them. The slow and progressive deterioration of language provides an interesting model to understand the mechanisms and biological bases involved in the linguistic process.

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PubMed1996

Neuroradiological findings in primary progressive aphasia: CT MRI and cerebral perfusion SPECT.

Primary progressive aphasia (PPA) is defined as progressive decline in language for 2 or more years with preservation of activities of daily living and general cognitive functions. Whereas the clinical features of this syndrome have been well documented, the neuroradiological findings have not been studied systematically. We studied 13 patients with PPA retrospectively: 10 underwent CT, 12 MRI and 12 cerebral perfusion studies using 99mTc-HMPAO SPECT.CT and MR images were scored for focal atrophy by two independent assessors. Initial qualitative assessment of SPECT images was confirmed by quantitative analysis. CT was normal in 5 patients. Focal atrophy, affecting predominantly the left temporal lobe, was seen in 4 of 10 patients on CT, and 10 of 12 on MRI. Atrophy was localised primarily to the superior and middle temporal gyri on MRI. All 12 patients who underwent SPECT had unilateral temporal lobe perfusion defects, in 2 patients of whom MRI was normal. CT is relatively insensitive to focal abnormalities in PPA; MRI and SPECT are the imaging modalities of choice. MRI allows accurate, specific localisation of atrophy within the temporal neocortex. SPECT may reveal a functional decrease in cerebral perfusion prior to establishment of structural change.

Adult↗
PubMedDate not supplied

Primary progressive aphasia. An uncommon masquerader of psychiatric disorders.

Primary progressive aphasia is a recently described, uncommon language disorder with unclear etiological and clinical boundaries. The infrequency and ambiguity of the syndrome may prompt psychiatric consultation. The authors review the pertinent features of one such referral, including a brief literature review of the salient aspects of the differential diagnosis, and note the implications for appropriate treatment.

Agnosia↗
PubMed2006

Vasectomy in men with primary progressive aphasia.

OBJECTIVE: To study the frequency of vasectomy in men with primary progressive aphasia (PPA). BACKGROUND: PPA is a dementia syndrome in which aphasia emerges in relative isolation during the initial stages of illness. On the basis of a clinical observation in a patient who dated the onset of symptoms to the period after a vasectomy, and because of the curious sharing of the tau protein exclusively by brain and sperm, vasectomy rates were examined in men with PPA. METHOD: This study used a case control design. Forty-seven men with PPA and 57 men with no cognitive impairment (NC) between 55 and 80 years of age were surveyed about a history of vasectomy. RESULTS: The age-adjusted rate of vasectomy in PPA patients (40%) was higher than in NC (16%, P=0.02). There was a younger age at onset for the patients with vasectomy (58.8 vs. 62.9 y, P=0.03). CONCLUSIONS: Vasectomy may constitute one risk factor for PPA in men. Potential mechanisms mediating risk include vasectomy-induced immune responses to sperm, which shares antigenic epitopes with brain. Antisperm antibodies can also develop in women and become risk factors for PPA.

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PubMed2004

Primary progressive aphasia: a review.

This review summarizes clinical and imaging features associated with primary progressive aphasia (PPA). We investigate the hypothesis that these patients can be divided into subgroups of progressive non-fluent aphasia (PNFA) and semantic dementia (SD), based on their linguistic profiles and related imaging studies, and examine whether each of these major subgroups can be further subdivided. We focus on several critical features within each progressive aphasic subgroup. In PNFA, we examine agrammatism, phonologic disorder, and impaired verb processing to determine whether this syndrome is related to a modality-specific impairment in word formation and articulation, or a conceptual deficit that interferes with grammatical processing. In SD, we examine impaired semantic memory, limited remote memory, and anomia to assess whether this syndrome is due to a modality-neutral interruption of semantic memory, or the degradation of various material-specific representations of object features and words. We conclude that there is sufficiently consistent and converging evidence from clinical and imaging studies to support the claim that PNFA and SD are distinct subgroups of PPA. However, there does not appear to be sufficient evidence at this point to support further discrimination within these progressive aphasic subgroups. Testing hypotheses about finer-grained syndromes such as progressive dysarthria or progressive anomia has important consequences for improving our understanding of language organization and the neural basis for language.

Aphasia, Primary Progressive↗
PubMedDate not supplied

Patterns of cerebral atrophy in primary progressive aphasia.

The authors illustrate the spectrum of clinical and imaging patterns in primary progressive aphasia (PPA), a syndrome of slowly progressive speech and language impairment occurring with neurodegenerative disease. Although PPA presents with relatively isolated impairment in language, many patients progress to global cognitive or behavioral dysfunction. The syndrome may be associated with frontotemporal dementia (FTD)- or Alzheimer disease (AD)-type changes. Authors describe the clinical presentation in three cases of PPA and analyze the pattern of cerebral atrophy in each case with voxel-based morphometry. Two patients presented with nonfluent progressive aphasia. Subtle differences in the clinical features were suggestive of FTD in one case and AD in the other. Neuroimaging revealed a predominance of frontal atrophy in the first case and temporo-parietal atrophy in the second. The third case presented with the syndrome of semantic dementia and showed the typical behavioral problems associated with FTD and a pattern of left-greater-than-right temporal atrophy. Different clinical syndromes in PPA are associated with different patterns of atrophy. In the future, combined analysis of imaging and clinical characteristics may allow more accurate etiologic diagnosis.

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PubMed2006

An overview on Primary Progressive Aphasia and its variants.

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.

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PubMedDate not supplied

The use of reaction time measures to evaluate nonword reading in primary progressive aphasia.

We reported on a subject with nonfluent primary progressive aphasia (PPA), NL, who demonstrated an impaired ability to make rhyme judgments (Dowhaniuk, Dixon, Roy, Black, & Square, in press). Our hypothesis was that these deficits represent a precursor to phonological alexia. However, no definitive evidence supported the existence of a phonological reading impairment as NL made relatively few errors reading nonwords. To further evaluate NL's nonword reading, nonword and real word reaction times were compared. NL's reaction times were significantly longer for only nonwords compared to the slowest control subject. We then assessed the first two stages of processing involved in nonword reading (Coltheart, 1996). NL did not demonstrate deficits with graphemic parsing or phoneme assignment. His continuing problems with auditory rhyme judgments support the presence of a phonological processing deficit not specific to reading. We conclude that reaction time measures allow for the detection of subtle nonword reading deficits.

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PubMedDate not supplied

Primary progressive aphasia: a review of the neurobiology of a common presentation of Pick complex.

Primary progressive aphasia (PPA) is an identifiable and distinct form of clinical presentation of focal degenerative disease. The underlying pathology is often considered heterogeneous, but we propose that, in fact, this syndrome has a characteristic underlying neurobiology related to Pick's disease and frontotemporal dementia. The authors reviewed the literature of PPA andfound 58 cases with autopsy. The majority of the cases had related underlying pathology, termed "Pick complex," a form of non-Alzheimer's dementia. This includes Pick's disease, corticobasal degeneration, dementia lacking distinctive histology, and motor neuron disease inclusions. Several of the cases described with focal Alzheimer's pathology do not fulfill the criteria of PPA, but have Alzheimer's disease with a major aphasic component.

Aphasia, Primary Progressive↗