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Journal of Neurology

, Volume 257, Issue 8, pp 1369–1372 | Cite as

Symptomatic paroxysmal dysarthria-ataxia in demyelinating diseases

  • Christophe Marcel
  • Mathieu AnheimEmail author
  • Constance Flamand-Rouvière
  • Françoise Heran
  • Pascal Masnou
  • Clotilde Boulay
  • Ivan Mari
  • Christine Tranchant
  • Emmanuel Roze
Original Communication

Abstract

Paroxysmal dysarthria-ataxia syndrome (PDA) is a rare neurological disorder that can be either primary or symptomatic of acute neurological dysfunction. Episodes of symptomatic PDA are poorly documented and there are no video reports. We describe the cases of two patients with symptomatic PDA related to demyelinating diseases. Detailed studies of the patients’ speech disorders showed that the dysarthria and gait disorders were of the ataxic type in both cases. Both patients had midbrain lesions at or below the level of the red nucleus, confirming that this area is critically involved in PDA. The best clinical signs for distinguishing between symptomatic and primary PDA are adult onset and short (<1 min) episodes in the former. If these signs are present, brain MRI should be used to identify a cause of symptomatic PDA.

Keywords

Episodic ataxia Multiple sclerosis Neuronal signaling Clinically isolated syndrome Cerebello-thalamo-cortical pathway Myelin 

Supplementary material

Video legend segment 1: ictal gait ataxia in patient #1, with normal gait between episodes; segment 2: ictal dysarthria in patient #2 when reading in French; segment 3: ictal dysarthria in patient #2 when reading in English; segment 4: ictal loudness variability and mild hoarseness during the prolonged-vowel test in patient #2; segment 5: ictal dysarthria when singing in patient #2; segment 6: ictal dysmetria in the finger-to-nose test in patient #2

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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Christophe Marcel
    • 1
  • Mathieu Anheim
    • 1
    • 2
    • 3
    Email author
  • Constance Flamand-Rouvière
    • 4
  • Françoise Heran
    • 5
  • Pascal Masnou
    • 4
  • Clotilde Boulay
    • 1
    • 6
  • Ivan Mari
    • 7
  • Christine Tranchant
    • 1
    • 3
  • Emmanuel Roze
    • 8
    • 9
    • 10
  1. 1.Département de NeurologieHôpital Civil de StrasbourgStrasbourgFrance
  2. 2.Service de Génétique, Groupe HospitalierHôpital Pitié-SalpêtrièreParis cedex 13France
  3. 3.Institut de Génétique et de Biologie Moléculaire et Cellulaire, CNRS, INSERMULPIllkirch GraffenstadenFrance
  4. 4.Service de NeurologieHôpital de BicêtreLe Kremlin BicêtreFrance
  5. 5.Service de RadiologieFondation Adolf de RothschildParisFrance
  6. 6.Département de NeurologieHôpital E. MullerMulhouseFrance
  7. 7.Service de NeurologieFondation Adolf de RothschildParisFrance
  8. 8.Service de NeurologieHôpital Pitié-SalpêtrièreParisFrance
  9. 9.CNRS, UMRS 952, INSERM, UMR 7224Université Pierre et Marie Curie-Paris-6ParisFrance
  10. 10.CIC 9503, Département de Neurologie, Hôpital Pitié-SalpêtrièreParis CedexFrance

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