Exceptional verbal intelligence after hemispherotomy in a child with Rasmussen encephalitis

Area of Science:

• Pediatric Neurology
• Neurosurgery
• Cognitive Neuroscience

Background:

• Rasmussen encephalitis (RE) is a rare, progressive neurological disorder causing severe epilepsy and cognitive decline.
• Hemispherotomy is a surgical option for intractable epilepsy, involving disconnection of one cerebral hemisphere.
• Presurgical evaluation is crucial for assessing cognitive function and language lateralization.

Observation:

• A left-handed 7-year-old girl with RE underwent left hemispherotomy.
• Presurgical deficits included mild hemiparesis, no visual impairment, and mild cognitive impairment with short-term memory deficits.
• Language functional MRI (fMRI) indicated right-hemisphere language dominance.

Findings:

• Post-surgery, the patient developed right hemiplegia and hemianopsia but maintained intellectual capacity.
• The patient achieved seizure freedom, allowing discontinuation of antiepileptic drugs.
• Long-term follow-up revealed exceptional verbal intelligence (VCI 155) and improved working memory, language, and reading skills.
• A notable visuoverbal discrepancy emerged, with high verbal and low spatial reasoning.

Implications:

• Early hemispherotomy can prevent the global cognitive deterioration typically seen in Rasmussen encephalitis.
• This case highlights the potential for significant cognitive recovery and high verbal functioning post-hemispherotomy.
• The pronounced visuoverbal discrepancy offers insights into the neurocognitive profile of RE patients after hemispherectomy.