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Título

WEST SYNDROME ASSOCIATED WITH HYPOXIC BRAIN INJURY CAUSED BY INTOXICATION: A CASE REPORT

Apresentação do caso

M.S.G, 1 year old, previously healthy, suffered intoxication by acaricide (organophosphate and pyrethroid) in November 2021. Patient presented seizures, vomiting, bronchoaspiration, pneumonia, severe respiratory distress and two cardiorespiratory arrests. The magnetic resonance imaging showed hemorrhagic laminar cortical necrosis and slight accentuation of cortical sulci and brain fissures. He was taken to the pediatric neurology using phenobarbital and baclofen. The electroencephalogram (EEG) presented an electrographic status epilepticus, and it was started levetiracetam and nitrazepam, once there wasn't the possibility of hospitalization. The second EEG presented an epileptic encephalopathy, with the persistence of the electrographic features, multifocal epileptiform activity and in burst-suppression occupying more than 80% of the record. Although the tracing was not typical of a hypsarrhythmia, due to the absence of slow high-voltage activity, the presence of semiology compatible with epileptic spasms led to the possibility that it was an evolution to West Syndrome. Therefore, it was decided to start corticosteroid (prednisone 3mg/kg/day). A new EEG presented abundant multifocal epileptiform activity in the tracing; no burst-suppression episodes were observed, nor was the pattern of electrographic status epilepticus on the record. The patient showed improvement in infantile spasms after treatment with corticosteroids for 3 months. However, after the withdrawal from prednisone, the patient started seizures again.

Discussão

West Syndrome is the combination of infantile spasms, hypsarrhythmia and developmental regression. It is caused sometimes by an injury to the brain. Other times, it is caused by developmental anomalies of brain structure, genetic mutations or metabolic disorders. In current practice, ACTH and vigabatrin are the main treatments. As the ACTH is not available in Brazil, high-dose oral of corticosteroids are used. Its use is as effective as ACTH, with fewer adverse effects and it can control between 33-63% of the infantile spasms.

Comentários finais

The prognosis of West Syndrome is usually poor. About 65-70% of children will have spasms fully controlled. Unfortunately, most children will have other kinds of seizures in later childhood including Lennox-Gastaut Syndrome. In this particular case, the patient has severe brain inury, which makes it even more difficult to control his seizures.

Referências (se houver)

1. Epilepsias e Síndromes Epilépticas. In: Rodrigues, Marcelo Masruha; Vilanova, Luiz Celso Pereira (eds). Tratado de neurologia infantil. RIO DE JANEIRO: Atheneu, 2017. p.991-1150.

2. Chellamuthu P, Sharma S, Jain P, Kaushik JS, Seth A, Aneja S. High dose (4 mg/kg/day) versus usual dose (2 mg/kg/day) oral
prednisolone for treatment of infantile spasms: an open-label, randomized controlled trial. Epilepsy Res. 2014;108(8):1378-84.

3. Hussain SA, Shinnar S, Kwong G, Lerner JT, Matsumoto JH, Wu JY, et al. Treatment of infantile spasms with very high dose prednisolone before high dose adrenocorticotropic hormone. Epilepsia. 2014;55(1):103-7.

Fonte de Fomento (se houver)

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Área

Epilepsias