Advertisement

Child's Nervous System

, Volume 3, Issue 1, pp 47–49 | Cite as

Paroxysmal kinesigenic choreoathetosis

  • Shaul Harel
  • U. Yurgenson
  • M. Kutai
Case Reports

Abstract

A healthy intelligent 13.5-year-old boy is reported who presented with paroxysmal kinesigenic choreoathetosis. He had had unilateral tonic spasms for 2 months. In a stressful situation, especially after a period of immobility, a sudden voluntary movement provoked a dystonic-choreoathetoic posturing of the left arm and leg. Attacks were brought on, for instance, by standing up quickly from a sitting position. They lasted only 5–10 s, without loss of consciousness and occurred up to ten times daily. Between the attacks there were no abnormalities. Family history was non-contributory. Neurological examination and laboratory findings, including repeated EEGs, were all negative. The child responded very well to phenytoin treatment and has now been completely free of dystonic attacks for a period of 9 months. The importance of early recognition of this syndrome and the differential diagnosis with reflex epilepsy and other forms of dystonic choreoathetosis is discussed.

Key words

Paroxysmal disorder Choreoathetosis Dystonia Movement disorder 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Boel M, Casaer P (1984) Paroxysmal kinesigenic choreoathetosis. Neuropediatrics 15:215–217Google Scholar
  2. 2.
    Burger LJ, Lopez RI, Elliot FA (1972) Tonic seizures induced by movement. Neurology 22:656–659Google Scholar
  3. 3.
    Busard HLSM, Renier WO, Gabreels FJM, Vos AJM, Declerck AC, Verhey FHM (1983) Autosomal dominant paroxysmal kinesigenic choreathetosis: an electroneurophysiologic study. EFCNS Symposium, Noordwijkerhout, June 1983, pp 16–37Google Scholar
  4. 4.
    Coulter DL, Donofrio P (1980) Haloperidol for nonkinesiogenic paroxysmal dyskinesia. Arch Neurol 37:325–326Google Scholar
  5. 5.
    Forssman H (1961) Hereditary disorder characterized by attacks of muscular contractions, induced by alcohol amongst other factors. Acta Med Scand 170:517–533Google Scholar
  6. 6.
    Fukuyama Y, Okada R (1968) Hereditary kinaesthetic reflex epilepsy. Report of five pedigrees with seizures induced by movement and review of literature. Proc Aust Assoc Neurol 5:583–587Google Scholar
  7. 7.
    Goodenough DJ, Fariello RG, Annis BL, Chun RWM (1978) Familial and acquired paroxysmal dyskinesias. A proposed classification with delineation of clinical features. Arch Neurol 35:827–831Google Scholar
  8. 8.
    Hishikawa Y, Furuya E, Yamamoto J, Nan'no H (1973) Dystonic seizures induced by movement. Arch Psychiatr Nervenkr 217:113–138Google Scholar
  9. 9.
    Homan RW, Vasko MR, Blaw M (1980) Phenytoin plasma concentrations in paroxysmal kinesigenic choreoathetosis. Neurology 30:673–676Google Scholar
  10. 10.
    Kato M, Araki S (1969) Paroxysmal kinesigenic choreoathetosis. Report of a case relieved by carbamazepine. Arch Neurol 20:508–513Google Scholar
  11. 11.
    Kertesz A (1967) Paroxysmal kinesigenic chloreoathetosis. An entity within the paroxysmal choreoathetosis syndrome. Description of 10 cases, including 1 autopsied. Neurology 17:680–690Google Scholar
  12. 12.
    Lance JW (1977) Familial paroxysmal dystonic choreoathetosis and its differentiation from related syndromes. Ann Neurol 2:285–298Google Scholar
  13. 13.
    Lishman WA, Symonds CP, Whitty CWM, Willison RG (1962) Seizures induced by movement. Brain 86:93–108Google Scholar
  14. 14.
    Loong SC, Ong YY (1973) Paroxysmal kinesigenic choreoathetosis. J Neurol Neurosurg Psychiatry 36:921–924Google Scholar
  15. 15.
    Mayex R, Fahn S (1982) Paroxysmal dystonic choreoathetosis in a patient with familial ataxia. Neurology (NY) 32:1184–1186Google Scholar
  16. 16.
    Mount LA, Reback S (1940) Familial paroxysmal choreoathetosis. Preliminary report on a hitherto undescribed clinical syndrome. Arch Neurol Psychiatry 44:841–847Google Scholar
  17. 17.
    Neophytides AN (1981) Choreoathetosis, paroxysmal kinesigenic. In: Vinken PJ, Bruyn GW (eds) Neurogenetic directory, part 1. (Handbook of clinical neurology, vol 42) North Holland, Amsterdam, pp 207–209Google Scholar
  18. 18.
    Perez-Borja C, Tassinari AC, Swanson AG (1967) Paroxysmal choreathetosis and seizures induced by movement (reflex epilepsy). Epilepsia 9:260–270Google Scholar
  19. 19.
    Pryles CB, Livingston S, Ford FR (1952) Familial paroxysmal choreoathetosis of Mount and Reback: study of a second family in which this condition is found in association with epilepsy. Pediatrics 9:44–47Google Scholar
  20. 20.
    Richards RN, Barnett HJM (1968) Paroxysmal dystonic choreoathetosis. A family study and review of the literature. Neurology 18:461–469Google Scholar
  21. 21.
    Rondot P (1983) Involuntary movements and neurotransmitters. Neuropediatrics 14:59–65Google Scholar
  22. 22.
    Stevens H (1966) Paroxysmal choreo-athetosis. A form of reflex epilepsy. Arch Neurol 14:415–420Google Scholar
  23. 23.
    Suber DA, Riley TL (1980) Valproic acid and normal computerized tomographic scan in kinesigenic familial paroxysmal choreathetosis. Arch Neurol 37:327Google Scholar
  24. 24.
    Tabaee-Zadeh MJ, Frame B, Kapphahn K (1972) Kinesiogenic choreoathetosis and idiopathic hypoparathyroidism. N Engl J Med 286:762–763Google Scholar
  25. 25.
    Tibbles JAR, Barnes SE (1980) Paroxysmal dystonic choreoathetosis of Mount and Reback. Pediatrics 65:149–151Google Scholar
  26. 26.
    Watson RT, Scott WR (1979) Paroxysmal kinesigenic choreoathetosis and brain stem atrophy. Arch Neurol 36:522Google Scholar

Copyright information

© Springer-Verlag 1987

Authors and Affiliations

  • Shaul Harel
    • 1
  • U. Yurgenson
    • 1
  • M. Kutai
    • 1
  1. 1.Pediatric Neurology Unit, Division of Pediatrics, Tel Aviv Medical Center, Sackler Medical SchoolUniversity of Tel AvivTel AvivIsrael

Personalised recommendations