Meta Description: Childhood primary angiitis of central nervous system is a form of vasculitis of unknown etiology primarily affecting small and medium-large sized vessels supplying the brain parenchyma, spinal cord and leptomeninges with an often slowly progressive course. Identification and appropriate diagnosis of children with the disorder is crucial because with standardized treatment good neurological outcome is a realistic goal.
Childhood primary angiitis of central nervous system is a form of vasculitis of unknown etiology primarily affecting small and medium-large sized vessels supplying the brain parenchyma, spinal cord and leptomeninges with an often slowly progressive course. Children with this disorder present with a range of neurological symptoms including intractable seizures, hemiparesis, cranial nerve deficits, severe cognitive deficits, and decreased consciousness.
Identification and appropriate diagnosis of children with the disorder is crucial because with standardized treatment good neurological outcome is a realistic goal. Therapeutic modalities including anti platelet agents, corticosteroids, Azathioprine, Cyclophosphamide and other immunomodulatory agents have been used with variable success remains a rare entity, the poor specificity of the available diagnostic tests and its multiple mimics create a major diagnostic challenge.
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