Neurological sequelae of the dancing eye syndrome

Eur J Pediatr. 1996 Mar;155(3):237-44. doi: 10.1007/BF01953945.


Fifty-four patients with dancing eye syndrome (DES), presenting over a 25-year period, were reviewed retrospectively. One third of them were on active follow up at the time of the study. Malignancy was uncommon, diagnosed in only 4 patients, neuroblastoma in 3 cases and acute lymphoblastic leukaemia (ALL) in 1. An intercurrent illness preceded onset of DES in 51% of the children and was of equivocal significance. There was no clear temporal relationship to immunization. The acute phase of the illness was severe and caused total immobilization in 88% of patients. A favourable initial response to treatment with prednisolone or corticotrophin gel was observed in all patients. Although corticotrophin seemed to produce a more rapid response, overall improvement was similar with both treatments. Long-term neurological morbidity was a major problem with 91% of children suffering either persisting symptoms or repeated relapses. A persisting disability was found in 88% and was considered severe in 61% of patients. No features prognostic of neurological severity or outcome were identified.

Conclusion: Demonstrable malignancy is uncommon in the dancing eye syndrome. The neurological legacy of DES is often evident well into adult life.

MeSH terms

  • Brain Damage, Chronic / etiology*
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Disability Evaluation
  • Encephalomyelitis / diagnosis
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Male
  • Myoclonus / etiology*
  • Neuroblastoma / diagnosis
  • Neurologic Examination*
  • Ocular Motility Disorders / etiology*
  • Paraneoplastic Syndromes / etiology
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / diagnosis
  • Syndrome