Von Neuropathie bis ZNS: Systemische Amyloidose früh erkennen und behandeln

Der Fokus dieses Übersichtsartikels (CME) liegt auf der Erkennung typischer Symptome einschließlich neuromuskulärer, systemischer und seltener ZNS-Manifestationen, den Erfordernissen und Fallstricken hinsichtlich der diagnostischen Verfahren, den typischen Befunden in der Elektromyografie (EMG), Elektroneurografie (NLG) und weiteren elektrophysiologischen Funktionstests sowie den aktuellen Therapieoptionen.

Abstract

Systemic amyloidosis is an important differential diagnosis in progressive polyneuropathies. ATTRv-amyloidosis (hereditary) typically affects peripheral nerves and/or the heart, while ATTRwt-amyloidosis predominantly presents as cardiomyopathy but can also cause neurological symptoms. AL amyloidosis often progresses rapidly and is painful. Early diagnosis and a holistic, interdisciplinary approach to patients is crucial given the therapeutic options now available.

Schlüsselwörter

systemische Amyloidose - neuromuskuläre Manifestation - ATTR-Amyloidose - AL-Amyloidose - Therapie

Key words

systemic amyloidosis - neuromuscular manifestation - therapie

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