Zusammenfassung
Anhand von vier Fallberichten wird eine Übersicht über die diagnostischen Aspekte des Botulismus gegeben. In der Akutphase der Erkrankung spielen die sorgfältige Anamnese und der klinische Befund eine wichtige Rolle, doch erst die elektrophysiologische Untersuchung kann einen entscheidenden Beitrag zur Sicherung der Diagnose liefern. Die konventionelle neurophysiologische Diagnostik mit Neurographie und Myographie ist wenig sensitiv, während bei der Untersuchung der neuromuskulären Übertragung in vielen Fällen pathologische Befunde erhoben werden können. Eine Zusammenstellung der elektrophysiologischen Resultate in 4 eigenen und 15 weiteren publizierten Fällen unterscheidet sich von dem, gemäß gewissen Lehrbüchern, typischen elektrophysiologischen Befundmuster: die „klassische” Konstellation mit einem Dekrement in der niedrigfrequenten repetitiven Stimulation (3 - 10 Hz) und mit einem Inkrement in der hochfrequenten Stimulation (20 - 50 Hz) wurde nur in 10,5 % aller Fälle beobachtet, während über die Hälfte der Patienten mit klinisch gesichertem Botulismus einen Normalbefund in der niedrigfrequenten - und ein Inkrement in der hochfrequenten repetitiven Stimulation zeigten (53 %). Insgesamt zeigten somit über 63 % der Patienten eine inkrementale Antwort in der hochfrequenten repetitiven Stimulation. Es wird daher eine höhere Sensitivität der hochfrequenten repetitiven Stimulation im Vergleich mit der niederfrequenten Stimulation postuliert. Die höchste diagnostische Sensitivität erzielt die Einzelfasermyographie, welche jedoch in der Akutphase der Erkrankung nur bedingt durchführbar ist.
Abstract
Based on four cases, diagnostic aspects of botulism are presented. Botulism must be diagnosed initially on the basis of the history and physical findings but electrophysiologic studies are often helpful for the establishment of the diagnosis. Conventional neurophysiologic testing with neuro- and myography has low sensitivity. However, the neurophysiological studies on neuromuscular transmission showed abnormalities in most of the cases. A review of 4 own and 15 previously published cases showed an inconsistency with the typical electrophysiological pattern of botulism, described in some text-books of neurophysiology: The „classical” finding of a decrement in low-rate repetitive stimulation (3 - 10 Hz) accompanied by an increment in high-rate repetitive stimulation (20 - 50 Hz) occurred in only 10,5 % of the cases; whereas, normal results for the low-rate repetitive stimulation and an increment for the high-rate stimulation were found in 53 % of the cases with documented botulism. In summary more than 63 % of patients showed an incremental response in high frequently repetitive stimulation. We postulate a higher sensitivity of the high-rate stimulation in opposite to the low-rate stimulation. However, single-fiber electromyography has the highest diagnostic sensitivity, but the feasability of this method is limited in the acute phase of the disease.
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Dr. med. Adam Czaplinski
Neurologische Universitätsklinik · Kantonsspital Basel
Petersgraben 4
4031 Basel · Schweiz
eMail: aczaplinski@uhbs.ch
