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Childhood Inflammatory Brain Disease

Childhood Inflammatory Brain Disease (IBD) Is an umbrella term that is increasingly being used to describe primary and secondary inflammatory disorders of the brain. IBD is a leading cause of devastating neuropsychiatric syndromes in previously healthy children.

As our knowledge of the rapidly expanding spectrum of new disease entities have been discovered in the last decade, treatment protocols have been initiated employing immune modulatory therapies. Such treatable disorders which manifest as acute neuropsychiatric disturbances include antibody-mediated encephalopathies associated with circulating antibodies against extracellular and intracellular neuronal antigens directed present in the N-methyl-D-aspartate (NMDA) receptor, voltage-gated potassium channel (VGKC)-complex, glutamic acid decarboxylase 65(GAD65), gamma-aminobutyric acid (GABA), and aquaporin-4 antibodies (NMO) receptors in the brain, streptococcal (PANDAS/PANS) and other infectious-mediated vectors; as well as, encephalopathy associated with thyroid endocrinopathy (Hashimoto type) and dietary sources of gluten  (Celiac disease).  

One particular immune modulatory treatment, intravenous immune globulin (IVIG) therapy, is first-line therapy both in the acute phase of disease, and as a part of long-term management. The typical dose is 2 grams per kilogram per month administered over 2 to 4 days per month with careful monitoring for any observed side-effects.  Read more and share in this important group of disorders that can be found in Chapter 23, Volume 2 of The Vasculitides, edited by David S. Younger MD MPH MS.