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J Pediatr Neurosci. 2019 Jan-Mar;14(1):36-37. doi: 10.4103/jpn.JPN_12_18.

Late-Onset Citrullinemia Type I: A Radiological Mimic of Herpes Encephalitis.

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Department of Pediatric Neurology, Superspeciality Pediatric Hospital and Post Graduate Teaching Institute, Uttar Pradesh, India.
Department of Pediatrics, Advanced Pediatric Center, Division of Pediatric Neurology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Radio diagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Pediatric Neurology and Neurodevelopment, Medanta-The Medicity, Gurugram, Haryana, India.


We describe a 10 year old boy who presented with acute onset rapidly progressing encephalopathy. MRI revealed bilateral insular cortex and basifrontal involvement suggesting Herpes encephalitis.He was treated with acyclovir and his symptoms improved. Six months after the first hospitalization he reported back with two episodes of partial seizures. He was started on Valproate. A week after starting valproate he was readmitted with hyperammonemic encephalpathy, on further investigations Citrullinemia Type 1 was diagnosed. This case highlights a metabolic disorder which radiologically mimics herpes encephalitis. The management of the disorder differs remarkably from herpes encephalitis and hence its recognition and suspicion based on radiology is critical.


Citrullinemia; herpes encephalitis; hyperammonemia

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