Seizure is clinical manifestation of sudden disruption of the normal electrical activity of cortical neurons. The brain electrical activity is periodically disturbed, alteration in neural cell integrity, increase in firing impulses and spread to adjacent normal neurons result in temporary brain dysfunction with alterations in consciousness, behavior or motor function. It may be triggered by illness, infection, stress, stroke, brain tumor, or the underlying cause may not completely understand. Status epilepticus (SE) is a medical emergency and requires prompt diagnosis and treatment. Treatment includes general support measures, drugs to suppress epileptic activity and relieving the underlying condition. Refractory SE requires admission to an intensive care unit (ICU) to allow adequate monitoring and support of respiratory, metabolic and hemodynamic functions and cerebral electrical activity. For SE treatment, benzodiazepines are the first line antiepileptic agents, and if benzodiazepines fail to control seizures, Phenytoin is usually indicated; Phenobarbital or Valproate may also be considered. For refractory SE, Propofol and Thiopental represent first line agents after careful assessment of potential risks. In refractory SE, general anesthesia may be required. There is currently no unique consensus for definite treatment option of RSE. In this review, the management protocol of seizure, assessment, monitoring, and different alternative therapy would be discussed.
Purpose: Children with autism spectrum disorder are at an increased risk for developing seizures, which can be triggered by classical antipsychotics. Aripiprazole is an atypical antipsychotic that has a safer drug profile. The objective is to present the experience with seizure control in autistic children who are placed on Aripiprazole.
Methods: Series of consecutive autistic children with comorbid epilepsy treated with Aripiprazole were identified prospectively over a 3-year period. Monthly follow up by one pediatric neurologist was performed to document seizure control.
Results: 56 autistic children with comorbid epilepsy were placed on Aripiprazole. Most children (59%) were seizure free for at least 6 months. The initial Aripiprazole dose was 5 mg in all patients. Follow up ranged between 5-8 months (mean 6.9). A total of 5 (9%) children developed seizure provocation (3/5) or worsening seizure control (2/5). There were 3 males and 2 females with ages ranging between 6-11.5 years (mean 8.5). Three of these children had a previous history of seizure worsening with other antipsychotic drugs (respiridone in 2 and haloperidol in 1). One child with seizure provocation developed status epilepticus 5 days after introducing Aripiprazole that required intensive care admission. The drug was stopped in all 5 children with no long-term effects.
Conclusion: Seizure provocation or worsening seizure control is not uncommon following the introduction of Aripiprazole in autistic children with controlled epilepsy. Although the risk is low, parents should be warned and advised on what to do, particularly in the first month of therapy.
Mariana Catalina Garcini Sandoval*, Enrique Espinosa Zúñiga and Martha Guadalupe García Toribio
Published on: 24th August, 2023
Sturge-Weber Syndrome (SWS) is a congenital, vascular, neurocutaneous, uncommon disease associated with facial angiomas port wine birthmark (PWB) or “nevus flammeus”, cerebral vascularity alterations (leptomeningeal vascular malformation), and ocular disorders. It is the third most common neurocutaneous syndrome after neurofibromatosis and tuberous sclerosis. GNAQ R183Q is the most frequent related mutation, caused by a postzygotic, somatic, gain-of-function. 75% of patients present seizures during the first year of life, mainly focal motor seizures, with or without consciousness impairment.We present the case of a 33-year-old female with a diagnosis of SWS, with refractory seizures that started at 4 months of age. In this admission, she presented upper and lower respiratory tract infections that culminated in a convulsive status epilepticus (CSE), the reason for which she required sedation and advanced airway management with adjustment of the anti-seizure medication (ASM). An electroencephalogram (EEG) was performed that reported epileptic activity, as well as an imaging study with data suggestive of calcification in the frontal and right parietal region, compatible with vascular malformation.
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