[Neurological involvement in rheumatic disorders and vasculitis in childhood]

Abstract

Introduction: The rheumatic disorders and vasculites are inflammatory processes affecting connective tissue in different organs. The inflamed cells liberate destructive enzymes which harm tissues, producing IgE, IgM, IgG antibodies and autoimmune complexes.

Objective: To review the current classification of these conditions and describe the commonest neurological complications in children. Infantile systemic lupus erythematosus is the commonest; it affects the dermis, joints, blood vessels, heart, kidney and brain. Neurological features: vertigo, ataxia, convulsions, chorea and headache are seen in 25% of the patients. Juvenile idiopathic arthritis is another inflammatory disorder of unknown origin which affects joints, connective tissue and visceras. Neurological involvement is seen in 5%, in the form of cerebral vasculitis, cervical myelopathy, trapping and compression of peripheral nerves and neuropathy due to arteritis. Sydenham s rheumatic chorea, caused by b haemolytic streptococcal A infection, causing a pathological auto immune response is the best known and has increased in frequency again in recent years. Vasculitis is caused by inflammatory changes in the walls of large, medium and small blood vessels. They lead to deposits of auto immune antigen antibody complexes in the blood vessel walls. In the commonest infantile vasculites neurological complications are uncommon, but not rare, as occurs with Kawasaki disease, infantile Wegener s granulomatosis, Schönlein-Henoch purpura and Behçet's disease. The usual treatment for this group of disorders is corticosteroids and immunosuppressive drugs.