Chronic T Cell Encephalitis Responsive to Immunotherapy

Area of Science:

• Neurology
• Neuroimmunology

Background:

• Rasmussen's encephalitis is a rare chronic inflammatory neurological disease causing progressive unilateral brain atrophy, typically in children.
• It often presents with intractable focal epilepsy, hemiparesis, and cognitive decline.

Observation:

• An adult patient presented with pseudobulbar affect and frontal lobe dysfunction.
• The patient developed multifocal myoclonic jerks, focal motor seizures, and hemiparesis with bilateral cerebellar signs.
• Imaging revealed progressive hemispheric atrophy with bilateral involvement.

Findings:

• Brain biopsy confirmed chronic T-cell infiltrate, consistent with inflammation.
• The patient's presentation included atypical features such as bilateral symptoms and specific neurological deficits not typically seen in Rasmussen's encephalitis.
• Positron emission tomography-computed tomography (PET CT) demonstrated bilateral features.

Implications:

• This case expands the understanding of the clinical spectrum of Rasmussen's encephalitis, particularly in adult-onset and bilateral presentations.
• It underscores the importance of considering atypical presentations and utilizing advanced imaging and biopsy for diagnosis.
• Further research is needed to elucidate the pathogenesis and optimize treatment strategies for such complex cases.