The following are the proposed treatment protocols for childhood CNS vasculitis and inflammatory brain diseases which may be useful for physicians.

  • Angiography-positive non-progressive CNS Vasculitis (NP-cPACNS) affects medium-large sized vessels and the diagnosis is confirmed on angiography. It is a monophasic disease. Children with NP-cPACNS typically present arterial ischemic stroke.  NP-cPACNS suggested treatment protocol

 

  • Angiography-positive progressive CNS Vasculitis (P-cPACNS) affects medium-large sized vessels and the diagnosis is confirmed on angiography. When untreated, children with P-cPACNS will acquire new neurological deficits and new areas of stenosis on angiography after three months. P-cPACNS suggested treatment protocol  

 

  • Angiography-negative CNS Vasculitis (SV-cPACNS) affects small sized vessels, and the diagnosis is confirmed on brain biopsy. Children with SV-cPACNS present with predominantly diffuse neurological deficits. SV-cPACNS suggested treatment protocol 

 

  • Antibody Mediated Inflammatory Brain Diseases (IBrainD) represent a novel group of diseases in which inflammation targets the brain structure in previously healthy children and causes devastating injury to the brain. Antibody mediated IBrainD suggested treatment protocol

 

  • Rasmussen Encephalitis is a poorly understood disease which causes progressive severe epilepsy. It is associated with T-cell infiltration on brain biopsy, and curative treatment is hemispherectomy. Medical treatment is usually reserved for older children and individuals with bilateral disease. Rasmussen encephalitis suggested treatment protocol

 

  • Transverse Myelitis is a demyelinating disease of the spinal cord, causing paralysis and other neurological dysfunctions, including bowel and bladder dysfunction. Onset occurs over days to weeks, and recovery usually begins by 3 months after starting treatment. Transverse Myelitis suggested treatment protocol

 

  • Autoimmune Channelopathies are a diverse set of diseases arising from the development auto-antibodies against ion channels in the brain. Disease presentation is dependent on the offending antibodies, but symptoms can include cerebellar ataxia and encephalitis. Autoimmune Channelopathies suggested treatment protocol

 

  • Granulomatous Inflammatory Brain Diseases include ANCA-vasculitides and neurosarcoidosis. They cause a wide variety of symptoms depending on the localization of the lesions, and can be associated with systemic disease as well. Granulomatous IBrainD suggested treatment protocol

 

  • Suspected Inflammatory Brain Diseases represent a group of diseases in which previously healthy children present with inflammatory brain diseases symptoms but no brain biopsy has been performed to confirm the diagnosis. Affected children can have low risk, intermediate risk, or high risk disease level. 
    • At low risk, children respond to steroids treatment immediately and improve without in need of a second line agent. 
    • At intermediate risk, children respond well to steroids treatment along with second line agent, and would not experience life-threatening situations.
    • At high risk, children experience symptoms similar to angiography-negative CNS vasculitis (SV-cPACNS), but they do not have brain biopsy performed for a confirmatory diagnosis. They may experience life-threatening situations and require immediate treatment if possible. 
    • Suspected Inflammatory Brain Diseases suggested treatment protocol