Primary progressive apraxia in Pick's disease

Fukui, Toshiya; Sugita, Koujiro; Kawamura, Mitsuru; Shiota, Jun; Nakano, Imaharu

doi:10.1212/wnl.47.2.467

Cited by 73 publications

(53 citation statements)

References 17 publications

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“…Most noteworthy, 11% of patients had AOS without any accompanying aphasia or dysarthria, a finding that supports those of single case or case series reports which suggest that AOS can be the only communication problem in neurodegenerative disease, at least early in its evolution (e.g., Broussolle et al, 1996;Cohen et al, 1993;Didic et al, 1998;Fukui et al, 1996;Sakurai et al, 1998;Tebartz Van Elst et al, 2002). When this is the case, a diagnostic designation of primary progressive AOS is appropriate and useful, both clinically and for research purposes.…”

Section: Presenting Complaints and Temporal Relationshipssupporting

confidence: 57%

“…AOS (or aphemia) has been reported as the initial manifestation of pathologically confirmed Pick's disease (Fukui, Sugita, Kawamura, Shiota, & Nakano, 1996;Sakurai, Murayama, Fukusako, Bando, Iwata, & Inoue, 1998). It has been the initial symptom and predominant sign in a small number of people with PSP (Boeve, Dickson, Duffy, Bartleson, Trenerry, & Petersen, 2003;Josephs et al, 2005).…”

Section: Duffymentioning

confidence: 99%

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Apraxia of speech in degenerative neurologic disease

2006

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Background: Only a limited number of case reports or case series have provided unequivocal evidence that apraxia of speech (AOS) can be the first or predominant sign of neurodegenerative disease. This may reflect a very low frequency of occurrence but may also reflect a failure in many studies to identify AOS or distinguish it from the diagnosis of primary progressive aphasia (PPA). The distinction may be important to clinical neurological localisation and diagnosis, prognosis, management, and histopathology. Aims: To describe the demographic characteristics, presenting complaints, perceptual attributes, associated speech-language and related clinical findings, neuroimaging findings, and clinical neurological diagnoses for a large group of patients with AOS due to degenerative neurologic disease. Methods & Procedures:Retrospective review of patients seen between 1985 and 2004, who had AOS that was never less severe than any aphasia that might have been present and whose neurologic evaluation concluded that the cause was degenerative.Outcomes & Results: A total of 80 patients met inclusion criteria. Of these patients, 61% were men. Average age at initial evaluation was 69 years (range536-86 years). Speechlanguage difficulty was the first symptom to emerge in 80% and the only patient complaint at the time of initial evaluation in 56%. Average duration of symptoms at initial evaluation was 29 months. Aphasia and dysarthria were unequivocally present in 49% and 50% of the sample, respectively. Of the 80 patients with AOS, 11% had neither aphasia nor dysarthria. When abnormal, neuroimaging often identified left or left.right hemisphere abnormalities. Of these patients, 44% received purely descriptive clinical neurologic diagnoses that were largely determined by speech-language findings; an additional 18% received diagnoses related to focal/asymmetric cortical atrophy syndromes that localised to the left hemisphere; 29% received diagnoses of diseases with prominent motor manifestations, most often corticobasal degeneration, progressive supranuclear palsy, or motor neuron disease. Conclusions: AOS can be the first and most prominent manifestation of neurodegenerative disease. It often co-occurs with aphasia and/or dysarthria, but it can be the only speech-language disorder. It does not appear to be associated with any single neurologic disease but specific neurologic diagnoses, when they are possible, tend to reflect conditions with prominent motor manifestations. It is important to distinguish primary progressive AOS from PPA.

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Section: Presenting Complaints and Temporal Relationshipssupporting

confidence: 57%

Section: Duffymentioning

confidence: 99%

Apraxia of speech in degenerative neurologic disease

2006

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“…Today, this case would be diagnosed as Cortico-Basal Degeneration (CBD), a condition which regularly affects the parietal lobes. Fukui et al describe a further patient with progressive apraxia, speech disorder and motor impairment, in whom brain atrophy extended to the superior parietal lobes, although it was centered in more anterior regions [10]. Pathology showed numerous Pick bodies and ballooned neurons.…”

Section: ■ Nosology Of Posterior Cortical Atrophymentioning

confidence: 99%

Posterior cortical atrophy: variant of Alzheimer?s disease?

2005

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Nine patients with posterior cortical atrophy (PCA), a rare degenerative brain disease of unclear etiology and nosology, were followed over a mean time of 7.4 years. The mean age at onset was low (56.2 years). At onset, eight patients had visuo-spatial and eight had memory impairment. A minority showed early signs of occipital lobe involvement with visual agnosia or hemianopia. Eight patients developed dementia after a mean course of five years. 18F-FDG-PET data of six patients were analysed with statistical parametric mapping. They showed hypometabolism centred on the lateral and medial parietal associative cortex, with variable involvement of the adjacent temporal and occipital associative cortex. A minority showed involvement of the frontal lobes, possibly related to deafferenting of areas related to the control of eye movements. Atrophy and hypometabolism were markedly asymmetric in a subset of cases. Autopsy was performed in one patient. Presenile onset, location, and asymmetry of atrophy suggest that PCA represents a biologically separable variant of Alzheimer's disease.

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“…Unfortunately, we were not able to extend the investigation by measuring biomarkers, for instance, due to the lack of financial support. However, our hypothesis of a tau protein-related neuropathology should be taken into consideration in the light of previous evidence [1][2][3][4] .…”

Section: Casementioning

confidence: 99%

“…Among the dementias, primary progressive apraxia has an uncertain etiology, presenting as a variant of Pick's disease 1 or other nosological entities 2 . The syndrome usually manifests in the sixth decade of life, and its clinical diagnosis depends on the types of apraxia manifested initially.…”

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confidence: 99%