Differenzialdiagnose der Amyotrophen Lateralsklerose in der klinischen Praxis

Zusammenfassung

Die Diagnose der Amyotrophen Lateralsklerose (ALS) beruht vorwiegend auf klinischen Kriterien und kann aufgrund des heterogenen Erscheinungsbildes gerade in frühen Krankheitsstadien mitunter schwierig sein. Wegweisend sind der Nachweis einer kombinierten Pathologie des oberen und unteren Motoneurons, der fokale Onset, das Ausbreitungsmuster (Spreading), das typische Verteilungsmuster der Paresen, das Vorhandensein oder Fehlen nicht-motorischer Zusatzsymptome sowie Art und Geschwindigkeit der Krankheitsprogredienz. Dieser Review stellt Charakteristika der ALS sowohl im Hinblick auf die klinische Untersuchung als auch apparativer Zusatzdiagnostik in Abgrenzung zu den wichtigsten Differenzialdiagnosen in Form von Einschlusskörperchenmyositis, Chronisch Inflammatorischer Demyelinisierender Polyneuropathie (CIDP), Mulifokaler Motorischer Neuropathie (MMN), adulter Form der Spinalen Muskelatrophie (SMA), Kennedy-Syndrom und Hereditärer Spastischer Paralyse (HSP) dar.

Abstract

Diagnosis of Amyotrophic Lateral Sclerosis (ALS) relies mainly on clinical criteria and may be difficult in early disease stages due to the heterogenous presentation of the disease. Important features are the combined pathology of upper and lower motor neuron, focal onset, spreading, typical patterns of pareses, the lack or presence of additional non-motoric symptoms, as well as the temporal development of symptoms and disease progression. This review presents the characteristics of ALS related to clinical findings as well as instrumental diagnostics in contrast to its most important differential diagnoses including Inclusion Body Myositis (IBM), Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), Multifocal Motor Neuropathy (MMN), adult forms of Spinal Muscular Atrophy (SMA), Kennedy Syndrome, and Hereditary Spastic Paraplegia (HSP).

Schlüsselwörter

Amyotrophe Lateralsklerose - Differenzialdiagnose - Klinische Zeichen

Key words

Amyotrophic Lateral Sclerosis - differential diagnosis - clinical features

Publication History

Article published online:
14 March 2023

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