TLR3 deficiency

Onset may occur at any age but is most common in adults. It presents as an acute necrosing temporal encephalitis, after a primary or recurrent infection. Onset is rapid (less than 48 hours) with a fever of 40 oC, headaches, and behavioural, language and memory problems. These initial manifestations are followed by numbness and coma, which may be accompanied by convulsions and paralysis.

Therapeutic options