Progressive Dysarthria

Santens, Patrick; J, Van Borsel; Foncke, E.M.J.; Meire,; Merkx, H; J, De Bleecker; J, De Reuck

doi:10.1159/000017125

Cited by 17 publications

(8 citation statements)

References 18 publications

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“…Descriptions of PLS suggest that bulbar symptoms rarely precede spinal symptoms(21–25). Moreover, reports describing progressive pseudobulbar syndromes have rarely characterized the nature of observed dysarthria, either with respect to the speech diagnosis (e.g., spastic dysarthria (23, 26, 27)) or specific speech features(24). …”

Section: Discussionmentioning

confidence: 99%

Clinical and imaging characterization of progressive spastic dysarthria

Clark

Duffy

Whitwell

et al. 2013

Euro J of Neurology

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Objective To describe speech, neurological and imaging characteristics of a series of patients presenting with progressive spastic dysarthria (PSD) as the first and predominant sign of a presumed neurodegenerative disease. Methods Participants were 25 patients with spastic dysarthria as the only or predominant speech disorder. Clinical features, pattern of MRI volume loss on voxel-based morphometry, and pattern of hypometabolism with F18-Fluorodeoxyglucose (FDG-PET) scan are described. Results All patients demonstrated speech characteristics consistent with spastic dysarthria, including strained voice quality, slow speaking rate, monopitch and monoloudness, and slow and regular speech alternating motion rates. Eight patients did not have additional neurological findings on examination. Pseudobulbar affect, upper motor neuron pattern limb weakness, spasticity, Hoffman sign and positive Babinski reflexes were noted in some of the remaining patients. Twenty-three patients had electromyographic assessment and none had diffuse motor neuron disease or met El Escorial criteria for ALS. Voxel-based morphometry revealed striking bilateral white matter volume loss, , affecting the motor cortex (BA 4), including the frontoparietal operculum (BA 43) with extension into the middle cerebral peduncle. FDG-PET showed subtle hypometabolism affecting the premotor and motor cortices in some patients, particularly in those who had a disease duration longer than two years. Conclusions We have characterized a neurodegenerative disorder that begins focally with spastic dysarthria due to involvement of the motor and premotor cortex and descending corticospinal and corticobulbar pathways. We propose the descriptive label “progressive spastic dysarthria” to best capture the dominant presenting feature of the syndrome.

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Section: Discussionmentioning

confidence: 99%

Clinical and imaging characterization of progressive spastic dysarthria

Clark

Duffy

Whitwell

et al. 2013

Euro J of Neurology

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“…There is a notion of heterogeneity and subgroup formation in FTD, depending on the presence of language and speech disorders, motor abnormalities and the patterns of behavioral alterations. Correlations of specific patterns of hypometabolism with specific clinical presentations have not been investigated in a satisfactory way [5]. …”

Section: Discussionmentioning

confidence: 99%

“…This necessarily results in variable degrees of diagnostic uncertainty [2, 3]. Dementing disorders are clinically heterogeneous in themselves, as a consequence of variable distributions of brain dysfunction within specific nosological entities [4, 5]. The inclusion of patients with large variations in age and dementia severity further clouds the interpretation of many PET studies [6, 7].…”

Section: Introductionmentioning

confidence: 99%

Differential Regional Cerebral Uptake of <sup>18</sup>F-Fluoro-2-Deoxy-<i>D</i>-Glucose in Alzheimer’s Disease and Frontotemporal Dementia at Initial Diagnosis

Santens¹,

Bleecker²,

Goethals³

et al. 2001

Eur Neurol

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In this paper, the cerebral uptake of ¹⁸F-fluoro-2-deoxy-D-glucose (FDG) was studied in a relatively small group of patients suffering from either clinically diagnosed Alzheimer’s disease (AD) or frontotemporal dementia during the initial differential diagnostic workup. The image analysis was done visually and semiquantitatively using three different reference regions. Visual analysis confirmed earlier literature findings on the distribution of decreased FDG uptake and demonstrated prevalent asymmetric patterns in both groups. Only semiquantitative analysis using the sensorimotor cortex as a reference region confirmed the visual findings. Moreover, there were no differences in medial temporal lobe activities between both groups, and there was no correlation of medial temporal lobe activity with dementia severity. In the AD group, a correlation of parietal FDG uptake with MMSE scores was found.

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“…De Koning et al [11 ]and Becker et al [12] reported cases of primary lateral sclerosis [11,12]. Santens et al [13 ]reported 2 cases of progressive dysarthria. Soliveri et al [14] reported 2 corticobasal degeneration cases, 1 FTD case, 1 progressive aphasia case, 1 ALS case, 1 motor neuron disease plus dementia case, and 1 ALS plus aphasia case.…”

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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Progressive Dysarthria

Cited by 17 publications

References 18 publications

Clinical and imaging characterization of progressive spastic dysarthria

Clinical and imaging characterization of progressive spastic dysarthria

Differential Regional Cerebral Uptake of <sup>18</sup>F-Fluoro-2-Deoxy-<i>D</i>-Glucose in Alzheimer’s Disease and Frontotemporal Dementia at Initial Diagnosis

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

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