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


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.


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


  1. 1.
    Jen JC, Graves TD, Hess EJ, Hanna MG, Griggs RC, Baloh RW, CINCH investigators (2007) Primary episodic ataxias: diagnosis, pathogenesis and treatment. Brain 130:2484–2493CrossRefPubMedGoogle Scholar
  2. 2.
    Damak M, Riant F, Boukobza M, Tournier-Lasserve E, Bousser MG, Vahedi K (2009) Late onset hereditary episodic ataxia. J Neurol Neurosurg Psychiatry 80:566–568CrossRefPubMedGoogle Scholar
  3. 3.
    Andermann F, Cosgrove JB, Lloyd-Smith D, Walters AM (1959) Paroxysmal dysarthria and ataxia in multiple sclerosis; a report of 2 unusual cases. Neurology 9:211–215PubMedGoogle Scholar
  4. 4.
    Espir ML, Millac P (1970) Treatment of paroxysmal disorders in multiple sclerosis with carbamazepine (Tegretol). J Neurol Neurosurg Psychiatry 33:528–531CrossRefPubMedGoogle Scholar
  5. 5.
    Wolf P, Assmus H (1974) Paroxysmal dysarthria and ataxia. A pathognomonic seizure syndrome in multiple sclerosis. J Neurol 208:27–38CrossRefPubMedGoogle Scholar
  6. 6.
    Matthews WB (1975) Paroxysmal symptoms in multiple sclerosis. J Neurol Neurosurg Psychiatry 38:617–623CrossRefPubMedGoogle Scholar
  7. 7.
    Osterman PO, Westerberg CE (1975) Paroxysmal attacks in multiple sclerosis. Brain 98:189–202CrossRefGoogle Scholar
  8. 8.
    Twomey JA, Espir ML (1980) Paroxysmal symptoms as first manifestations of multiple sclerosis. J Neurol Neurosurg Psychiatry 43:296–304CrossRefPubMedGoogle Scholar
  9. 9.
    Gorard DA, Gibberd FB (1989) Paroxysmal dysarthria and ataxia: associated MRI abnormality. J Neurol Neurosurg Psychiatry 52:1444–1445CrossRefPubMedGoogle Scholar
  10. 10.
    Akman-Demir FG, Eraksoy M, Gürvit IH, Saruhan-Direskeneli G, Aral O (1995) Paroxysmal dysarthria and ataxia in a patient with Behcet’s disease. J Neurol 242:344–347CrossRefPubMedGoogle Scholar
  11. 11.
    Tünzün E, Akman-Demir G, Eraksoy M (2001) Paroxysmal attacks in multiple sclerosis. Mult Scler 7:402–404Google Scholar
  12. 12.
    Eriksson M, Ben-Menachem E, Andersen O (2002) Epileptic seizures, cranial neuralgias and paroxysmal symptoms in remitting and progressive multiple sclerosis. Mult Scler 8:495–499CrossRefPubMedGoogle Scholar
  13. 13.
    Matsui M, Tomimoto H, Sano K, Hashikawa K, Fukuyama H, Shibasaki H (2004) Paroxysmal dysarthria and ataxia after midbrain infarction. Neurology 63:345–347PubMedGoogle Scholar
  14. 14.
    Blanco Y, Compta Y, Graus F, Saiz A (2008) Midbrain lesions and paroxysmal dysarthria in multiple sclerosis. Mult Scler 14:694–697CrossRefPubMedGoogle Scholar
  15. 15.
    Tintoré M, Rovira A, Rio J, Tur C, Pelayo R, Nos C, Téllez N, Perkal H, Comabella M, Sastre-Garriga J, Montalban X (2008) Do oligoclonal bands add information to MRI in first attacks of multiple sclerosis? Neurology 70:1079–1083CrossRefPubMedGoogle Scholar
  16. 16.
    Miller DH, Weinshenker BG, Filippi M, Banwell BL, Cohen JA, Freedman MS, Galetta SL, Hutchinson M, Johnson RT, Kappos L, Kira J, Lublin FD, McFarland HF, Montalban X, Panitch H, Richert JR, Reingold SC, Polman CH (2008) Differential diagnosis of suspected multiple sclerosis: a consensus approach. Mult Scler 14:1157–1174CrossRefPubMedGoogle Scholar

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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