CNS aquaphorin 4 autoimmunity in children

McKeon A, Lennon VA, Lotze T et al. Neurology 2008; 71:93-100.

Spectrum is such that patients with ON and / pr LETM with positive antibodies are more likely to have recurrence, but also are more likely to have episodic symptoms especially encephalopathy, seizures, nausea, diplopia, vertigo and hiccups. Brain lesions, possibly symptomatic, are seen in 60 % of patients, contrary to prior teaching. 42 % had other coexisting autoimmune diseases including SLE, JRA, Graves, and Sjogren's . More than half had residual disability. 84 % improved with iv methylprednisolone which was usually used as first therapy.