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[Progressive anarthria: an individual entity].

N Limousin1, S Rimbaux, K Mondon

  • 1Service de neurologie et de neurophysiologie clinique, hôpital Bretonneau, CHRU de Tours, 2, boulevard Tonnellé, 37044 Tours cedex, France. nadege.limousin@tele2.fr

Revue Neurologique
|June 17, 2008
PubMed
Summary
This summary is machine-generated.

Primary progressive anarthria, a rare speech impairment, requires prompt diagnosis. Early intervention and specific tests are crucial for accurate diagnosis and management of this focal cortical atrophy.

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Area of Science:

  • Neurology
  • Neuroscience
  • Speech Pathology

Background:

  • Primary progressive anarthria is a rare focal cortical atrophy characterized by progressive speech impairment, orofacial apraxia, and mutism.
  • It presents with frontal lobe syndrome and was first described 15 years ago.

Purpose of the Study:

  • To analyze clinical, neuropsychological, and test data in patients with primary progressive anarthria.
  • To understand the diagnostic challenges and disease course.

Main Methods:

  • Retrospective study of five patients with primary progressive anarthria.
  • Analysis of onset age, diagnostic delay, clinical/neuropsychological data, electromyography, MRI, and SPECT.

Main Results:

  • Mean age at onset was 75.2 years; diagnostic delay averaged 11.2 months.
  • Anarthria was linked to dysexecutive syndrome and sometimes impaired comprehension.
  • MRI revealed frontal or temporal atrophy; SPECT showed reduced blood flow in these regions.

Conclusions:

  • Delayed diagnosis due to poor initial orientation leads to severe disability.
  • Complementary tests are essential for diagnosis and to exclude other conditions like amyotrophic lateral sclerosis.
  • Early stages may involve premotor cortex, with later extension to motor cortex.