Description
Infantile epilepsy, also known as early onset epilepsy, is a type of epilepsy that starts in the first two years of life. It is characterized by seizures that may be subtle, such as staring spells, or more obvious, such as convulsions. The causes of infantile epilepsy are varied and may be due to genetic factors, brain malformations, or brain injuries. The management of infantile epilepsy involves a comprehensive approach that focuses on controlling seizures, minimizing side effects of medications, and improving the quality of life of the child and their family. Treatment typically involves the use of antiepileptic drugs, which are selected based on the type of epilepsy and the age of the child.
Summary Listen
- Infantile spasms, also known as West syndrome, are a type of epilepsy common in infants and toddlers, significantly contributing to neurodevelopmental morbidity. Co-morbidities often include developmental delay, intellectual disability, autism, cerebral palsy, and sensory impairments. It's an age-dependent epileptic encephalopathy resulting from brain injury before, during, or after birth.
- The W syndrome is characterized by a triad of spasms (often in clusters upon awakening), developmental delay, and hypsarrhythmia on EEG. Flexor, extensor, or mixed spasms are possible, and they are sometimes triggered by external stimuli. Most affected children have underlying neurodevelopmental impairments, though some regress after onset.
- Diagnosis involves detailed history, neurodevelopmental assessment, and skin examination. Neuroimaging, particularly MRI, is crucial to identify underlying causes like cerebral dysgenesis or tuberous sclerosis. Metabolic workup and genetic testing are also indicated when no specific etiology is found. EEG shows hypsarrhythmia, but variations exist.
- Treatment includes hormonal therapy (ACTH or oral steroids) and vigabatrin. ACTH has parental administration drawbacks and costs, while steroids are easy to administer orally. Hormonal therapy is generally preferred, except in cases of tuberous sclerosis. Vigabatrin may be considered when hormonal therapy is contraindicated or fails, but visual side effects need to be monitored.
- Second-line treatments after failure of hormonal therapy and vigabatrin include benzodiazepines, sodium valproate, topiramate, and zonisamide. Dietary therapy, specifically ketogenic diet, can be considered if other treatments fail. Epilepsy surgery may be an option for cases with surgically resectable lesions.
- Early diagnosis is vital. Healthcare professionals dealing with high-risk infants should be trained to recognize spasms. Supportive care focuses on anticipatory guidance and managing co-morbidities. Prognosis is variable, with mortality ranging from 25-30% and significant psycho-motor retardation in most survivors.
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About the Speakers
Dr.Bharat Parmar
PROFESSOR AND HEAD OF PAEDIATRICS at ZYDUS MEDICAL College, Civil Hospital.
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