Rasmussen encephalitis associated with segmental vitiligo of the scalp: clinicopathologic report

Area of Science:

• Neurology
• Immunology
• Pediatrics

Background:

• Rasmussen syndrome is a rare, chronic inflammatory neurological disease affecting one cerebral hemisphere.
• Autoimmune mechanisms are increasingly implicated in the pathogenesis of Rasmussen syndrome.

Observation:

• A 3-year-old boy presented with segmental vitiligo, poliosis, alopecia, and subsequent epilepsia partialis continua.
• Neurological examination revealed progressive right cerebral hemisphere atrophy, dysacusia, and iris depigmentation.
• Cerebrospinal fluid analysis showed pleocytosis and hypergammaglobulin.

Findings:

• Despite extensive medical treatment, the patient underwent a right hemispherectomy.
• Neuropathology confirmed a widespread inflammatory process with neuronal loss and tissue collapse.
• The findings support an autoimmune etiology for Rasmussen syndrome, potentially linked to Vogt-Koyanagi-Harada syndrome.

Implications:

• This case underscores the importance of considering autoimmune conditions in pediatric neurological disorders.
• The study reinforces the autoimmune theory in Rasmussen syndrome pathogenesis.
• Further research into autoimmune markers may improve diagnosis and treatment of this debilitating condition.