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Encephalopathy in an infant with infantile spasms: possible role of valproate toxicity
Shobhana Sivathanu1, Sowmya Sampath, Madhubala Veerasamy
1Department of Pediatrics, ESI-PGIMSR, Chennai, Tamil Nadu, India.
Insights
This case report details a rare instance of valproic acid toxicity in an infant, presenting as encephalopathy despite normal liver function and ammonia levels. Early recognition and drug withdrawal are crucial for recovery.
Area of Science:
- Pediatric Neurology
- Clinical Pharmacology
Background:
- Infantile spasms and global developmental delay are serious pediatric neurological conditions.
- Sodium valproate is a common antiepileptic drug used in pediatric practice.
Observation:
- An infant with infantile spasms developed encephalopathy, including vomiting and altered sensorium, after initiating sodium valproate therapy.
- Initial investigations for hyperammonaemia and hepatic encephalopathy were normal.
- Seizures increased despite treatment, and symptoms resolved dramatically upon valproate withdrawal.
Findings:
- The adverse drug reaction (ADR) of valproate-induced encephalopathy was confirmed using the Naranjo ADR probability score.
- This case is the youngest reported instance of valproate-induced encephalopathy with normal liver function and ammonia levels.
- Marginal elevation in serum valproic acid levels was noted.
Implications:
- Valproate toxicity can manifest as encephalopathy even with normal liver function tests and serum ammonia.
- Clinicians should consider valproate-induced encephalopathy in infants presenting with neurological deterioration on the medication.
- This highlights the importance of careful drug monitoring and considering rare ADRs in pediatric epilepsy management.
Abstract:
An infant presented with global developmental delay and infantile spasms. EEG was suggestive of hypsarrhythmia. She was started on sodium valproate, clonazepam and adrenocorticotropic hormone injection. After an initial improvement the child developed vomiting, altered sensorium and increase in frequency of seizures suggestive of encephalopathy. Valproate-induced hyperammonaemia or hepatic encephalopathy was considered and the drug was withheld following which there was a dramatic improvement. Paradoxically, the liver function tests and serum ammonia were normal. However, a complete reversal of encephalopathy, on withdrawal of the drug, strongly suggested an adverse drug reaction (ADR) due to valproic acid. Marginal elevation of serum valproic acid prompted us to use the Naranjo ADR probability score to confirm the diagnosis. This case highlights the fact that valproate toxicity can manifest with normal liver function and serum ammonia levels. This is the youngest reported case with this rare form of valproate-induced encephalopathy.
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