Case presentation: 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.
Discussion: 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.
Final comments: 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 injury, which makes it even more difficult to control his
seizures.
