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Infantile Spasms: Clinical profile and treatment outcomes
Shazia Kulsoom1, Shahnaz H Ibrahim2, Sidra Kaleem Jafri3
1Dr. Shazia Kulsoom, MBBS, FCPS, Department of Pediatrics and Child Health, Aga Khan University Hospital, Karachi, Pakistan.
Insights
Infantile spasms (IS) are severe childhood epilepsy. Most cases were symptomatic, often due to hypoxic ischemic insult, and required multiple anti-epileptic drugs like vigabatrin for seizure control. Treatment outcomes did not differ between idiopathic and symptomatic groups.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Pediatrics
Background:
- Infantile spasm (IS) is a severe epileptic encephalopathy affecting children under two years old.
- Understanding the clinical profile, etiology, and treatment outcomes is crucial for managing IS.
- Tertiary care centers play a vital role in diagnosing and treating complex pediatric neurological conditions.
Purpose of the Study:
- To determine the clinical characteristics, etiological factors, and treatment responses in children with infantile spasms.
- To analyze the diagnostic utility of EEG and MRI in infantile spasms.
- To evaluate the effectiveness of various anti-epileptic drugs (AEDs) in managing infantile spasms.
Main Methods:
- Retrospective study of 36 patients diagnosed with infantile spasms (IS) aged 3 months to 2 years.
- Data collected from case records at Aga Khan University Hospital, Karachi (2010-2015).
- Analysis of clinical observations, investigations, AEDs, and treatment outcomes (complete, partial, no response) after six weeks of treatment.
Main Results:
- Symptomatic etiology predominated (61%), with hypoxic ischemic insult being the most common cause (32%).
- Consanguinity (66.6%) and developmental motor delay (89%) were frequently observed.
- Vigabatrin was the most commonly used AED (88%), and EEG/MRI were key diagnostic tools.
Conclusions:
- The majority of infantile spasm cases in this cohort were symptomatic, characterized by generalized tonic-clonic and myoclonic seizures.
- EEG and MRI were effective diagnostic modalities.
- Multiple AEDs were often necessary, with vigabatrin being the preferred choice; treatment outcomes were similar for idiopathic and symptomatic IS.
Background And Objective:
Infantile spasm (IS) is one of the severe epileptic encephalopathies which affect children in early two years of life. Our objective was to determine the clinical profile, etiology and outcome of treatment in children with infantile spasms attending tertiary care hospital at Karachi, Pakistan.
Methods:
This is retrospective study of 36 patients out of 94 registered as IS, aged three months to two years, managed and followed up at Aga Khan University Hospital, Karachi, from 2010 to 2015. Data of all children with IS was collected from case record. Details including clinical observations, lab investigations, anti-epileptic medications and treatment outcome was collected and analyzed. Patients who received treatment for six weeks to document response were included. The treatment response was categorized as complete response, partial response (>50% improvement) and no response. Data was analyzed on SPSS using descriptive statistics.
Results:
Thirty- six patients (38.29%) with IS fulfilled eligibility criteria. The mean ± SD age at presentation was 4.6±2.1 months. Male to female ratio was 2:1. Consanguinity and developmental motor delay was observed in 66.6% and 89% respectively. Symptomatic etiology was predominant (61%) and hypoxic ischemic insult (32%) was the commonest underlying cause. EEG and MRI were diagnostic tools whereas metabolic studies were not helpful. Multiple antiepileptic drugs were used for seizure control and vigabatrin was the most frequently used (88%) drug. Short term treatment response was not different in idiopathic or symptomatic infantile spasms.
Conclusion:
Majority of patients had symptomatic infantile spasms and generalized tonic clonic along with myoclonic jerks were predominant seizure types. EEG and MRI were diagnostic in most of cases. Multiple AEDs were required to control seizures and VGB was most common drug (88%) used. Treatment outcome was not different in idiopathic and symptomatic groups.
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