1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-23-93-a 10.15844/pedneurbriefs-23-12-6 Paroxysmal Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Opsoclonus Myoclonus Presenting as Status Epilepticus http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 12 2009 01 12 2015 23 12 93 93 Copyright: © 2009 The Author(s) 2009 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Opsoclonus myoclonus: a non-epileptic movement disorder that may present as status epilepticus

Two cases of non-epileptic opsoclonus presenting as status epilepticus are reported fom the John Radcliffe Hospital, Oxford, UK.

 Non-Epileptic Opsoclonus Suspected Encephalitis Electroencephalogram

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]

Haden SV McShane MA Holt CM Opsoclonus myoclonus: a non-epileptic movement disorder that may present as status epilepticus Arch Dis Child 2009 Nov 94 11 897 899 10.1136/adc.2009.160044 19847000 Markand ON Pearls, perils, and pitfalls in the use of the electroencephalogram Semin Neurol 2003 Mar 23 1 7 46 10.1055/s-2003-40750 12870104 Hammer MS Larsen MB Stack CV Outcome of children with opsoclonus-myoclonus regardless of etiology Pediatr Neurol 1995 Jul 13 1 21 24 10.1016/0887-8994(95)00083-R 7575843