Akute Polyneuritiden und Polyradikuloneuritiden: Ungewöhnliche Ursachen und deren Pathomechanismen

Zusammenfassung

Akute Polyneuritiden sind insgesamt zwar eine seltene, in entwickelten Ländern jedoch die häufigste Ursache für akut auftretende, generalisierte Lähmungen (> 50 %). Das Guillain-Barré-Syndrom (GBS) ist die häufigste Form dieser Erkrankung. Obwohl sich das klinische und pathologische Spektrum des GBS in den letzten Jahren erheblich erweitert hat, lassen sich etwa 15 % der akuten Polyradikuloneuritiden oder Polyneuritiden trotz erweiterter und revidierter Klassifikationen und Diagnosekriterien keiner der eingeführten GBS-Varianten zuordnen. Die auslösenden Pathomechanismen sind bis heute nicht vollständig geklärt, man vermutet aber, dass sowohl das GBS als auch die „untypischen“ akuten Polyneuritiden Varianten der gleichen, zugrunde liegenden immunologischen Dysregulation sind, jedoch durch pathogenetische Mechanismen unterschiedliche akut-neurologische Bilder hervorrufen. Deshalb sprechen auch akute Polyneuritiden, die sich keiner der eingeführten GBS-Varianten zuordnen lassen, ebenso auf eine immunmodulatorische Therapie (die nicht GBS-spezifisch ist) an, was ihr Erkennen und frühzeitiges Behandeln essenziell macht. Da bei den Immunneuropathien kein valider diagnostischer Marker existiert und das Krankheitsbild sehr variabel bleibt, wird die Diagnose klinisch gestellt und ist nicht selten eine Herausforderung. Den akuten Polyneuritiden, die sich keiner der eingeführten GBS-Varianten zuordnen lassen, scheint gemein, dass sie seltener infektassoziiert auftreten und häufiger mit ungewöhnlichen Ursachen und Auslösern assoziiert sind. In diesem Beitrag schildern wir unsere Erfahrungen mit ungewöhnlichen Varianten und Auslösern akuter Polyneuritiden und Polyradikuloneuritiden, die nicht den internationalen Diagnosekriterien des GBS entsprechen. Wir liefern eine ausführliche Literaturübersicht über die möglichen Pathomechanismen und diskutieren mögliche Dispositionen zur Entwicklung der Erkrankung.

Abstract

Although the total incidence rate of acute inflammatory polyneuropathies is low, it is the most frequent cause of acute progressive, generalized paresis in developed countries (> 50 %). The most common form of the disease is the Guillain-Barré syndrome (GBS). Even though the clinical and pathologic spectrum of GBS has substantially grown over the last decade, about 15 % of cases of acute polyneuritis or polyradiculoneuritis do not fulfil the revised and extended diagnostic criteria and classification for GBS and its variants. The underlying pathogenesis still remains unclear. It is assumed that these “untypical” acute inflammatory polyneuropathies and cases fulfilling the GBS criteria are variants of the same underlying immune disorder, but that pathogenetic mechanisms produce different acute neurological syndromes. Thus, immunotherapy (which is not GBS-specific) is also effective for treating acute inflammatory polyneuropathies that do not fulfil the diagnostic criteria for GBS, and early diagnosis and treatment of these cases is essential. Since no reliable serological and electrodiagnostic markers of autoimmune neuropathies are currently available, the diagnosis is based on its clinical presentation. However, clinical symptoms are variable, and a rational diagnostic decision can be challenging. Thus, it is important to know that acute inflammatory polyneuropathies not fulfilling the diagnostic criteria of GBS are less often preceded by an infective condition but frequently associated with uncommon causes and triggers. This report presents our experiences with uncommon variants of inflammatory polyneuropathies and polyradiculoneuritides that do not fulfil the international diagnostic criteria for GBS. We provide a detailed review of the pertinent literature discussing possible pathomechanisms, its clinical associations and possible dispositions.

Schlüsselwörter

akute Polyneuritiden - Immunneuropathien - Guillan-Barré-Syndrom - klinische Varianten - Autoimmunerkrankungen

Key words

acute polyneuritis - immunomediated neuropathies - Guillain-Barré-Syndrome - clinical variants - autoimmune disease

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