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Symptomatic paroxysmal dysarthria-ataxia in demyelinating diseases

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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.

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Authors and Affiliations

  1. Département de Neurologie, Hôpital Civil de Strasbourg, 1 Place de l’Hôpital, BP426, 67091, Strasbourg, France

    Christophe Marcel, Mathieu Anheim, Clotilde Boulay & Christine Tranchant

  2. Service de Génétique, Groupe Hospitalier, Hôpital Pitié-Salpêtrière, 47-83 Boulevard de l’Hôpital, 75651, Paris cedex 13, France

    Mathieu Anheim

  3. Institut de Génétique et de Biologie Moléculaire et Cellulaire, CNRS, INSERM, ULP, Illkirch Graffenstaden, France

    Mathieu Anheim & Christine Tranchant

  4. Service de Neurologie, Hôpital de Bicêtre, Le Kremlin Bicêtre, France

    Constance Flamand-Rouvière & Pascal Masnou

  5. Service de Radiologie, Fondation Adolf de Rothschild, Paris, France

    Françoise Heran

  6. Département de Neurologie, Hôpital E. Muller, Mulhouse, France

    Clotilde Boulay

  7. Service de Neurologie, Fondation Adolf de Rothschild, Paris, France

    Ivan Mari

  8. Service de Neurologie, Hôpital Pitié-Salpêtrière, Paris, France

    Emmanuel Roze

  9. CNRS, UMRS 952, INSERM, UMR 7224, Université Pierre et Marie Curie-Paris-6, Paris, France

    Emmanuel Roze

  10. CIC 9503, Département de Neurologie, Hôpital Pitié-Salpêtrière, 47-83 Boulevard de l’Hôpital, 75651, Paris Cedex, France

    Emmanuel Roze

Authors
  1. Christophe Marcel
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  2. Mathieu Anheim
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  3. Constance Flamand-Rouvière
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  4. Françoise Heran
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  5. Pascal Masnou
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  6. Clotilde Boulay
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  7. Ivan Mari
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  8. Christine Tranchant
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  9. Emmanuel Roze
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Corresponding author

Correspondence to Mathieu Anheim.

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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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Marcel, C., Anheim, M., Flamand-Rouvière, C. et al. Symptomatic paroxysmal dysarthria-ataxia in demyelinating diseases. J Neurol 257, 1369–1372 (2010). https://doi.org/10.1007/s00415-010-5534-3

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  • DOI: https://doi.org/10.1007/s00415-010-5534-3

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