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Título

MYOCLONIC STATUS EPILEPTICUS IN A PEDIATRIC PATIENT: CASE REPORT

Apresentação do caso

Female patient, 14 years old, healthy, with a history of ingestion of an unknown amount of rodenticide. A few hours after, she presented vomiting, diarrhea, bradycardia, myotic pupils and generalized tonic-clonic seizures, evolving with two cardiorespiratory arrests. It was performed the first cardiopulmonary resuscitation maneuvers, including sedation and intubation; atropinization and vasoactive drugs was administrated at the intensive care unit. During hospitalization, she developed generalized myoclonus. Electroencephalogram showed a myoclonic status epilepticus, which was reversed with the use of high doses of thiopental, having no response to other anticonvulsants. She progressed with the absence of some brainstem reflexes, but did not complete a brain death diagnosis, maintaining cerebral blood flow on Doppler; brain magnetic resonance revealed severe hypoxic-ischemic encephalopathy. After prolonged hospitalization, she required gastrostomy and tracheostomy for dehospitalization. Currently, bedridden and with important neurological sequelae, the patient maintains outpatient follow-up.

Discussão

Post-hypoxic myoclonus, particularly myoclonic status epilepticus (MSE), is uncommon in infants and a marker of poor prognosis.
Patients who survived long cardiorespiratory arrest, can develop severe neurological deficits, including post-hypoxic myoclonus. This status may be divided into: MSE and Lance-Adams Syndrome (LAS).
MSE is a condition that makes the patient have generalized myoclonus for more than 30 minutes. It occurs shortly after cardiopulmonary resuscitation, with an electroencephalogram showing epileptiform activity. On the contrary, LAS appears days, weeks or months after an ischemic event. The electroencephalogram usually does not show epileptiform activity, with a pattern of diffuse slowing – which is different from the case of the patient under discussion
The treatment of MSE is challenging and not well established. Administration of phenytoin, valproic acid, phenobarbital, and various benzodiazepines may be ineffective.

Comentários finais

Although there is no specific treatment, it is important that physicians pay attention to this diagnosis, after a long cardiorespiratory arrest. Early measures define survival and avoid limited prognosis, including brain injury.

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