Clinical and imaging characterization of progressive spastic dysarthria

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Background and purpose: To describe speech, neurological and imaging characteristics of a series of patients presenting with progressive spastic dysarthria 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 on F18-fluorodeoxyglucose positron emission tomography (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 amyotrophic lateral sclerosis. 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 2 years. Conclusions: A neurodegenerative disorder that begins focally with spastic dysarthria due to involvement of the motor and premotor cortex and descending corticospinal and corticobulbar pathways is characterized. The descriptive label 'progressive spastic dysarthria' to best capture the dominant presenting feature of the syndrome is proposed. Click here for the corresponding questions to this CME article.

Original languageEnglish (US)
Pages (from-to)368-376
Number of pages9
JournalEuropean Journal of Neurology
Volume21
Issue number3
DOIs
StatePublished - Mar 2014

Fingerprint

Dysarthria
Motor Cortex
Neurodegenerative Diseases
Positron-Emission Tomography
Babinski's Reflex
Voice Quality
Speech Disorders
Motor Neuron Disease
Fluorodeoxyglucose F18
Amyotrophic Lateral Sclerosis
Motor Neurons
Extremities

Keywords

  • Dysarthria
  • MRI
  • Neuromuscular disease
  • PET

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Clinical and imaging characterization of progressive spastic dysarthria. / Clark, Heather; Duffy, J. R.; Whitwell, Jennifer Lynn; Ahlskog, J. E.; Sorenson, Eric James; Josephs, Keith Anthony.

In: European Journal of Neurology, Vol. 21, No. 3, 03.2014, p. 368-376.

Research output: Contribution to journalArticle

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abstract = "Background and purpose: To describe speech, neurological and imaging characteristics of a series of patients presenting with progressive spastic dysarthria 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 on F18-fluorodeoxyglucose positron emission tomography (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 amyotrophic lateral sclerosis. 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 2 years. Conclusions: A neurodegenerative disorder that begins focally with spastic dysarthria due to involvement of the motor and premotor cortex and descending corticospinal and corticobulbar pathways is characterized. The descriptive label 'progressive spastic dysarthria' to best capture the dominant presenting feature of the syndrome is proposed. Click here for the corresponding questions to this CME article.",
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