Treatment of Rasmussen's syndrome with ganciclovir

Area of Science:

• Neurology
• Virology
• Pediatrics

Background:

• Rasmussen's syndrome is a rare, chronic inflammatory neurological disease affecting one hemisphere of the brain, often leading to intractable epilepsy and progressive neurological deficits.
• Cytomegalovirus (CMV) has been investigated as a potential causative agent in the pathogenesis of Rasmussen's syndrome.

Observation:

• Four patients with suspected Rasmussen's syndrome were treated with ganciclovir, a potent antiviral medication against CMV.
• One pediatric patient experienced complete seizure remission within days of treatment initiation, with sustained recovery of neurological and cognitive functions.
• Other patients treated later in the disease course or post-surgery showed varied responses, including seizure reduction in one case.

Findings:

• The CMV genome was detected in brain tissue samples from two out of three assessed patients, supporting a viral etiology.
• Prompt administration of ganciclovir was associated with a significant positive clinical response, including seizure cessation and functional recovery.
• Delayed treatment or combination with surgery yielded less consistent outcomes.

Implications:

• These findings suggest that cytomegalovirus may play a role in Rasmussen's syndrome, offering a potential target for therapeutic intervention.
• Early diagnosis and initiation of antiviral treatment with ganciclovir should be strongly considered in suspected cases of Rasmussen's syndrome.
• Further research is warranted to elucidate the precise role of CMV and optimize antiviral treatment strategies for this debilitating condition.