Multinational European Trial for Children With the Opsoclonus Myoclonus Syndrome / Dancing Eye Syndrome
- Children with newly diagnosed OMS/DES either NB-pos or NB-neg.
Three out of the following four components are necessary for the diagnosis of OMS/DES:
- Opsoclonus or ocular flutter (but not nystagmus)
- Ataxia and/or myoclonus
- Behavioural change and/or sleep disturbance
- Neuroblastoma The diagnosis of OMS/DES may be difficult in some patients. Opsoclonus,
in particular, may be intermittent or late in onset. A video example will be
available at www.dancingeyes.org.uk. If uncertain, please contact the national
coordinator for support in interpreting clinical features.
- Age 6 months or over up to less than 8 years (< 8th birthday) The date of
diagnosis of OMS/DES is the date on which a doctor confirms the condition to be
OMS/DES. The date of symptom onset needs also to be documented.
- Treatment start with the standard corticosteroid treatment with dexamethasone
pulses as proposed by the guidelines given in this trial protocol (see 11.10,
- In patients with presumed NB-neg OMS/DES, neuroblastoma must be excluded
according the guidelines of this trial (see chapter 18.104.22.168, page 30, and
appendix 11.9, page 70)
- Documented informed consent for treatment and enrolment in the trial by parents
/ legal representatives.
•Patients with opsoclonus, myoclonus or ataxia caused by other identified disease (e.g.
current active CNS infection, neurometabolic disorder or demyelination).
An identified viral precursor is not an exclusion criterion.
- prior or parallel use of chemotherapy (other than required for treatment of the
- Corticoid steroid for OMS/DES or other reasons lasting 14 days or more immediately
before treatment start according the standard treatment proposed (treatment with
corticosteroids for less than 14 days will be allowed)
- contre-indication of use of one of the experimental study drug (cf Summary of Product
Characteristics used in this study)