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A case report of infantile striatal necrosis with an acute onset
Insights
This study describes an 8-year-old boy with infantile striatal necrosis who improved after TRH-T therapy. The condition involves acute encephalopathy and striatal necrosis, potentially linked to heredogenous disorders.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Infantile striatal necrosis is a rare neurological disorder.
- It presents with acute encephalopathy, seizures, and dystonia.
- A specific subgroup of heredogenous disorders causes putaminal necrosis in children.
Abstract:
We report here an autopsy case, an 8-year-old boy diagnosed as having infantile striatal necrosis, characterized by a preceding febrile illness followed by acute encephalopathy with abrupt obtundation, seizures and dystonia, with remarkable improvement of the disturbed consciousness and intelligence after TRH-T therapy. These clinical symptoms were linked with bilateral necrosis of the striata on CT scanning. The presented case belonged to a newly described subgroup of the heredogenous disorders that produce necrosis of the putamina in children.