(Opsoclonic encephalopathy; opsoclonus-myoclonus syndrome, OMA syndrome, dancing eyes and legs syndrome)

Estimated annual incidence: 1/5.106. Association, in children less than 3 years of age of:

-        myoclonus: constant and intense, making coordinated movements impossible

-        opsoclonus: anarchic, asymmetric and multidirectional movements ofeyeballs.

-        ataxia

-        behavior disorders (sleep)

Association, in 50 to 80 % of cases, with abdominal, thoracic or pelvic neuroblastomas of small size: the excision of the tumor causes a net regression or disappearance of symptoms. More rarely associated with a ganglioneuroma or a hepatoblastoma. This pathology is found in 2 to 33 % of neuroblastomas the gravity of which  is generally moderate. In these cases, it consists of a paraneoplastic syndrome of probably autoimmune origin: anti-neuronal tissue antibodies (anti - Hu, anti-Ri etc) have been found in some cases. A genetic predisposition is possible in the presence of the  HLA II DRB1 * 013 antigen.

In the other cases, the etiology is most probably viral .

Treatment: ACTH or steroids associated with chemotherapy against the neuroblastoma plus IV immunoglobulins  or rituximab.

Anesthetic implications:

aggravation of the opsoclonus-myoclonus has been described in one case after administration of IM ketamine. When a neuroblastoma is present: management  of a tumor that can secrete catecholamines; otherwise, sequelae of corticosteroids (vascular fragility, obesity, hypertension, perioperative opotherapy).

References : 

-         Burrows FA, Seeman RG.
Ketamine and myoclonic encephalopathy of infants (Kinsbourne syndrome).
Anesth Analg 1982; 61: 873-5

-        Nisa N, Tabawar P, Vasudevan B.
Anaesthesia in a child with Kinsbourne syndrome: does anaesthesia technique matters?
Saudi J Anaesth 2016; 10: 468-70

-        Kushner BH.
Enigmatic entities: opsoclonus myoclonus ataxia syndrome linked to neuroblastoma.
Lancet Child & Adol Health 2018; 2: 3-5

-        Maranhão MVV, Cavalcanti Fulgino de Holanda A, Lira Tavares F.
Kinsbourne syndrome: case report.
Rev Bras Anestesiol 2013;63 :287-9

Updated November 2022