Two cases of non-epileptic opsoclonus presenting as status epilepticus are reported fom the John Radcliffe Hospital, Oxford, UK. Jerking of eyes and limbs were initially explained as suspected encephalitis, and the patients were treated for seizures with anticonvulsants and anesthetic intubation, but without benefit. EEGs showed no epileptic discharges. A diagnosis of opsoclonus myoclonus was made in both cases, and treatment with adrenocorticotropic hormone (40 IU/day) in one and prednisolone (4 mg/kg/day) in the other resulted in rapid resolution of symptoms. No neoplasm or infectious agent was identified, and neither patient has relapsed or shown developmental delay. Video footage of both patients showing florid jerking suggestive of status epilepticus is presented on line. [1]

COMMENT. The electroencephalogram is indispensable in the distinction of non-epileptic paroxysmal disorders from epileptic seizures and in the diagnosis of nonconvulsive status epilepticus and an encephalopathic process. [2]

Outcome of opsoclonus-myoclonus studied in 11 patients at Children’s Memorial Hospital, Chicago found that 9 of 10 treated with ACTH had recurrence of symptoms during a gradual withdrawal of ACTH; prednisone in one patient was ineffective in controlling opsoclonus-myoclonus. Eight had developmental delay with motor incoordination and speech delay (7 with neuroblastoma and 1 without). Tumor removal did not improve symptoms. One of 8 with tumor and 2 of 3 with no tumor had normal neurologic development. [3]