Child Neurology: A Case of Rasmussen Syndrome Without Seizures.

Area of Science:

• Neuroimmunology
• Neurology
• Neurodegeneration

Background:

• Rasmussen syndrome (RS) is a rare neuroimmune disease.
• It typically presents with refractory epilepsy, focal neurological deficits, and cognitive decline.
• Diagnosis can occur without seizures if specific criteria are met.

Purpose of the Study:

• To describe a case of Rasmussen syndrome diagnosed without seizures.
• To illustrate the diagnostic criteria and pathophysiology of RS.
• To emphasize RS as a progressive neurodegenerative disorder.

Main Methods:

• Case report of a patient with hemiparesis and unihemispheric atrophy.
• Evaluation based on Bien criteria, including clinical progression and brain biopsy.
• Long-term clinical monitoring for seizure development.

Main Results:

• The patient met diagnostic criteria for RS through progressive clinical changes and histopathology, despite no initial imaging evidence of cortical atrophy progression.
• The patient remained seizure-free for 4.5 years post-symptom onset.
• Brain biopsy confirmed consistent histopathologic findings.

Conclusions:

• Rasmussen syndrome is an immune-driven, progressive neurodegenerative disorder.
• Seizures are a common but not essential feature for RS diagnosis.
• The case broadens the understanding of RS presentation and diagnostic possibilities.