Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes

Broussolle, Emmanuel; Bakchine, Serge; M, Tommasi; Laurent, Bernard; Bazin, B.; Cinotti, L.; Cohen, Laurent; Carrault, Guy

doi:10.1016/s0022-510x(96)00096-2

Cited by 115 publications

(84 citation statements)

References 51 publications

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“…Recently, several cases of 'progressive loss of speech output and orofacial dyspraxia' or 'progressive anarthria' have been reported [1][2][3][4]. It has been suggested that this syndrome might represent a distinct entity within the focal cortical atrophies [1,4] or be thought of as a form of primary progressive aphasia (PPA) [2,3].…”

Section: Introductionmentioning

confidence: 99%

Progressive Loss of Speech: A Neuropsychological Profile of Premotor Dysfunction

Didic¹,

Ceccaldi

Poncet³

1998

Eur Neurol

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Several patients with ‘progressive loss of speech output’ or ‘progressive anarthria’ of degenerative origin have been reported in the literature. We report 5 clinical cases with slowly progressive loss of speech output and initially no deficit in other cognitive domains. The early clinical features were analysed in an attempt to identify the anatomo-functional systems implied in the degenerative process. The first phase of the disorder was characterised by impaired articulation consistent with speech apraxia, telegraphic style and a difficulty to elaborate a series of orofacial or hand movements. It is argued that these symptoms result from an impairment of complex motor processing due to dysfunction of the ventral premotor system. In the second phase, a decrease in spontaneous speech and self-initiated action was combined with exaggerated dependency on external stimuli, interpreted as dysfunction of the dorsal premotor system. We suggest that the neuropsychological profile of the disorder may result from progressive degeneration of the premotor cortex.

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Section: Introductionmentioning

confidence: 99%

Progressive Loss of Speech: A Neuropsychological Profile of Premotor Dysfunction

Didic¹,

Ceccaldi

Poncet³

1998

Eur Neurol

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“…Broussole et al [15 ]suggested a unique syndrome called ‘slowly progressive anarthria'. In this syndrome, apraxia of speech in the initial stage and dysarthria, aprosodia and oral-facial apraxia in the advanced stage are core clinical features.…”

Section: Discussionmentioning

confidence: 99%

Pseudobulbar Dysarthria in the Initial Stage of Motor Neuron Disease with Dementia: A Clinicopathological Report of Two Autopsied Cases

Ishihara

Araki²,

Ihori³

et al. 2013

Eur Neurol

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We retrospectively analyzed the clinical features of two cases of neurodegenerative disease, whose initial symptoms were motor speech disorder and dementia, brought to autopsy. We compared the distributions of pathological findings with the clinical features. The main symptom of speech disorder was dysarthria, involving low pitch, slow rate, hypernasality and hoarseness. Other than these findings, effortful speech, sound prolongation and initial difficulty were observed. Moreover, repetition of multisyllables was severely impaired compared to monosyllables. Repetition and comprehension of words and sentences were not impaired. Neither atrophy nor fasciculation of the tongue was observed. Both cases showed rapid progression to mutism within a few years. Neuropathologically, frontal lobe degeneration including the precentral gyrus was observed. The bilateral pyramidal tracts also showed severe degeneration. However, the nucleus of the hypoglossal nerve showed only mild degeneration. These findings suggest upper motor neuron dominant motor neuron disease with dementia. We believe the results indicate a subgroup of motor neuron disease with dementia whose initial symptoms involve pseudobulbar palsy and dementia, and which shows rapid progression to mutism.

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“…We suspect that this is due to the relatively loose criteria for the diagnosis of PNFA which often include cases of progressive anomia (non-semantic type) and progressive apraxia of speech. 40,41 We have recently published four of these five cases with PSP pathology and suggest that the presence of apraxia of speech, a motor planning or programming abnormality, as a dominant or co-dominant feature to the aphasia may be a clue to predicting PSP pathology. 42,43 Cases of CBD presenting as PNFA have also been reported.…”

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confidence: 99%

Trigeminal neuralgia due to pontine infarction

Iizuka

Hosokai²,

Mori³

2006

Neurology

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Abstract-Objective: To examine the relationship between early clinical features, pathologies, and biochemistry of the frontotemporal lobar degenerations (FTLDs), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). Methods: The authors conducted pathologic reexamination with the most recent immunohistochemistry of all cases diagnosed with FTLD, PSP, and CBD between 1970 and. The authors also reviewed the early clinical features for clinical diagnosis and application of published research criteria. Results: Of 127 cases analyzed, 57 had a pathologic diagnosis of FTLD, 49 PSP, and 21 CBD. Of these, 38 were clinically reclassified as frontal variant frontotemporal dementia (FTD), 13 as progressive non-fluent aphasia (PNFA), 21 as CBD-like, 33 as PSP-like, and 13 with frontotemporal dementia with coexisting motor neuron disease (FTD-MND). The authors were unable to classify nine cases. All cases of FTD-MND were tau-negative and had pathologic evidence of motor neuron degeneration. All cases classified as PSP-like or CBD-like had tau-positive pathology. Of the 13 cases with PNFA, PSP and CBD accounted for almost 70% of the cases, while FTD was almost equally divided between tau-positive and tau-negative diseases. Conclusion: Frontotemporal lobar degeneration, corticobasal degeneration (CBD), and progressive supranuclear palsy (PSP) have overlapping clinical features. The prediction of tau-positive pathology from a CBD or PSP-like presentation is good, while the frontotemporal dementia (FTD)-motor neuron disease syndrome almost certainly predicts motor neuron degeneration. Surprisingly, PSP and CBD accounted for most cases classified as progressive non-fluent aphasia. Frontal variant FTD is an unpredictable disease in terms of its biochemistry.

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Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes

Cited by 115 publications

References 51 publications

Progressive Loss of Speech: A Neuropsychological Profile of Premotor Dysfunction

Progressive Loss of Speech: A Neuropsychological Profile of Premotor Dysfunction

Pseudobulbar Dysarthria in the Initial Stage of Motor Neuron Disease with Dementia: A Clinicopathological Report of Two Autopsied Cases

Trigeminal neuralgia due to pontine infarction

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