Neurophysiologische Befunde bei den Stiff Man-Syndromen

Zusammenfassung

In einem sich klinisch und immunologisch zunehmend ausdifferenzierenden Manifestationsspektrum chronischer Autoimmun- Enzephalomyelitiden sind fluktuierende Rigidität und Spasmen das ungewöhnliche gemeinsame klinische Merkmal der Stiff Man-Syndrome (SMS). Die Autoimmunität richtet sich beim SMS vor allem gegen Proteine inhibitorischer ZNS-Synapsen. Korrespondierend lassen sich neurophysiologische Zeichen einer defekten Inhibition auf verschiedenen motorischen Regulationsebenen nachweisen, besonders im Rückenmark und Hirnstamm, aber auch im motorischen Kortex. Diagnostisch wertvoll ist vor allem die EMG-Polygrafie spontaner und reizinduzierter Spasmen und Myoklonien, weil sie reproduzierbare abnorme Innervationsmuster mit kurzer Latenz identifiziert.

Abstract

In a broadening clinical and immunological spectrum of chronic autoimmune encephalomyelitis, fluctuating rigidity and spasms, an unusual combination, are the common characteristics of stiff man syndromes. Autoimmunity is targeted mainly against proteins of inhibitory CNS synapses. Correspondingly, neurophysiological techniques demonstrate a lack of inhibition at various levels of motor regulation, mainly in the spinal cord and brainstem, but also in the motor cortex. EMG polymyography, particularly of spontaneous and stimulus-induced spasms and jerks, helps one to make the diagnosis by identifying reproducible abnormal innervation patterns with a short latency.

Schlüsselwörter

Stiff Man-Syndrome - Stiff Person-Syndrome - PERM - Myoklonus - antineuronale Autoimmunität

Key words

stiff man syndromes - stiff person syndromes - PERM - myoclonus - antineuronal autoimmunity

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