Neurological sequelae of the dancing eye syndrome.

UNLABELLED: 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.