ZNS-Vaskulitiden und entzündliche Hirnerkrankungen bei Kindern und Jugendlichen

Zusammenfassung

Schlaganfälle, Halluzinationen, Bewegungsstörungen und Krampfanfälle gehörten in der Vergangenheit nicht typischerweise zum Alltag der Kinderrheumatologen. Dies ändert sich gerade vielerorts. Unser Wissen um spezifische Entzündungsmechanismen und deren gezielte medikamentöse Behandlung macht uns zu wichtigen Partnern der Kinderneurologen, wenn es darum geht, Kinder und Jugendliche mit entzündlichen Hirnerkrankungen zu diagnostizieren und zu behandeln. Eine Entzündung des Gehirns kann im Rahmen einer Infektion, postinfektiös oder bei einer im Kindesalter eher selteneren systemischen Autoimmunerkrankung, wie beispielsweise einem systemischen Lupus erythematodes (SLE) oder einer Sarkoidose, auftreten. Ungleich häufiger jedoch erkranken vormals komplett gesunde Kinder und Jugendliche an einer isolierten oder primären Entzündung des Gehirns. Zielstrukturen können zerebrale Gefäße, Neurone oder spezifische Oberflächenrezeptoren des Gehirns sein. Die Entzündung hat schwere neurologische oder psychiatrische Symptome zur Folge, die im Entzündungsstadium selbst reversibel sind. Nur die frühzeitige Diagnose und gezielte Behandlung im interdisziplinären Team kann eine irreversible Hirnschädigung verhindern.

Summary

Strokes, hallucinations, movement disorders or seizures have not typically been the bread-and-butter, everyday work of pediatric rheumatologists. However, this is about to change in many centers. Our knowledge and understanding of specific mechanisms of inflammation and their tailored therapies has made us to important partners of pediatric neurologists, when diagnosing and managing children and adolescents with inflammatory brain diseases. Inflammation of the brain can occur with infections, post-infectious or – less commonly in the pediatric age group – in the context of systemic rheumatic diseases such as systematic lupus erythematosus (SLE) or sarcoidosis. By far more frequently, previously perfectly healthy children develop an isolated or primary inflammation of the brain. Target structure can be the cerebral blood vessels, neurons or specific cell surface receptors within the central nervous system (CNS). CNS inflammation can cause severe neurological deficits and psychiatric symptoms. Inflammation is reversible. Early diagnosis and rapid treatment start in an interdisciplinary care team can prevent irreversible brain damage secondary to inflammatory brain injury.

Schlüsselwörter

Entzündung - Vaskulitis - ZNS - Immunsuppression - Antikörper

Keywords

Inflammation - vasculitis - CNS - immunosuppression - antibodies

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