Advertisement

Journal of Neurology

, Volume 259, Issue 3, pp 557–558 | Cite as

Symptomatic paroxysmal kinesigenic choreoathetosis as primary manifestation of multiple sclerosis

  • Simone Zittel
  • Maxim Bester
  • Christian Gerloff
  • Alexander Münchau
  • Frank LeypoldtEmail author
Letter to the Editors

Dear Sirs,

Paroxysmal kinesigenic choreoathetosis (PKC) is a rare movement disorder characterized by episodes of uni- or bilateral choreoathetosis precipitated by sudden or rapid movements, startle or hyperventilation. It can be categorized as primary, e.g. idiopathic or hereditary, and secondary PKC [3]. Amongst the more frequent causes of the latter is multiple sclerosis (MS) [1]. Here we describe two patients presenting with symptomatic PKC as a clinically isolated syndrome.

Patient 1, a 34-year old woman, presented with a two-week history of episodes of right-sided numbness, tingling and spasms of her hand and leg occurring 2–15 times a day for 60 s per episode. These episodes were triggered by sudden movements, e.g. turning her head or moving in bed. Patient 2, a 27-year old woman, experienced multiple (>50) daily episodes of painful spasms of her right arm and leg as well as “pressed” speech for a period of about 1 min triggered by repetitive movements or hyperventilation. In...

Keywords

Multiple Sclerosis Dystonia Neurofibromatosis Muscle Soreness Spinal Cord Lesion 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgments

Dr. Zittel is supported by the Wegener Stiftung and has received research support by Merz Pharmaceuticals. Dr. Gerloff was supported by advisory boards of Boehringer Ingelheim and Silk Road Medical, and by grants from the DFG (Ge 844/2-1, Ge 844/4-1), the Bundesministerium für Bildung und Forschung (BMBF competence net stroke, BMBF Multicare), and UKE intramural grants. Dr. Münchau is supported by the Deutsche Forschungsgemeinschaft (MU1692/2-2). He received research support and honoraria for lectures by Pharm Allergan, Ipsen, Merz Pharmaceuticals. He is supported by the Dystonia Medical Research Foundation (USA), the Tourette Syndrome Association (Germany) and N.E.MO., charity supporting the research of paediatric movement disorders.

Conflict of interest

None.

Supplementary material

Segment 1 (Patient 1) When running on the spot, the patient experiences bradykinesia of right hand and arm after 30 s with associated dystonia and slight chorea. She describes a tingling sensation of right arm and leg. A similar but more intense right sided dystonia and chorea can be elicited by 90 s of hyperventilation and subsides 20 s later. During the attacks symptoms showed a crescendo-decrescendo dynamic. Segment 2 (Patient 2) While running on the spot for 30 s, generalized predominantly right-sided chorea, appendicular and axial dystonia develop accompanied by a “cramp-like” sensation and “pressed” dystonic dysarthria. Symptoms resolved after 60 s (MPG 35100 kb)

References

  1. 1.
    Blakeley J, Jankovic J (2002) Secondary paroxysmal dyskinesias. Mov Disord 17:726–734PubMedCrossRefGoogle Scholar
  2. 2.
    de Seze J, Stojkovic T, Destee M, Destee A, Vermersch P (2000) Paroxysmal kinesigenic choreoathetosis as a presenting symptom of multiple sclerosis. J Neurol 247:478–480PubMedCrossRefGoogle Scholar
  3. 3.
    Demirkiran M, Jankovic J (1995) Paroxysmal dyskinesias: clinical features and classification. Ann Neurol 38:571–579PubMedCrossRefGoogle Scholar
  4. 4.
    Fragoso YD, Araujo MG, Branco NL (2006) Kinesigenic paroxysmal hemidyskinesia as the initial presentation of multiple sclerosis. MedGenMed 8:3PubMedGoogle Scholar
  5. 5.
    Polman CH, Reingold SC, Edan G, Filippi M, Hartung HP, Kappos L, Lublin FD, Metz LM, McFarland HF, O’Connor PW, Sandberg-Wollheim M, Thompson AJ, Weinshenker BG, Wolinsky JS (2005) Diagnostic criteria for multiple sclerosis: 2005 revisions to the “McDonald Criteria”. Ann Neurol 58:840–846PubMedCrossRefGoogle Scholar
  6. 6.
    Roos RA, Wintzen AR, Vielvoye G, Polder TW (1991) Paroxysmal kinesigenic choreoathetosis as presenting symptom of multiple sclerosis. J Neurol Neurosurg Psychiatry 54:657–658PubMedCrossRefGoogle Scholar
  7. 7.
    Spinicci G, Cherchi MV, Murru R, Conti M, Marrosu MG (2010) A case of neurofibromatosis and multiple sclerosis. Neurol Sci 31:631–634PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Simone Zittel
    • 1
  • Maxim Bester
    • 2
  • Christian Gerloff
    • 1
  • Alexander Münchau
    • 1
  • Frank Leypoldt
    • 1
    Email author
  1. 1.Department of NeurologyUniversity Medical Center Hamburg EppendorfHamburgGermany
  2. 2.Department of NeuroradiologyUniversity Medical Center Hamburg EppendorfHamburgGermany

Personalised recommendations