A case of recurrent Miller Fisher syndrome mimicking botulism

Abstract Miller Fisher syndrome (MFS) is a rare and usually monophasic polyradiculoneuropathy characterised by ophthalmoplegia, decreased or absent tendon reflexes, and ataxia. The objective of this study was to report a case of recurrent MFS with a clinical presentation virtually indistinguishable from botulism. The patient was a young man with two episodes of increasing external ophthalmoplegia, ptosis, and ataxia with a long asymptomatic interval in between. The second episode occurred after consumption of rotten fish and was accompanied by gastrointestinal symptoms and an anticholinergic syndrome. Very rarely, MFS can present with a recurrent course. The importance of this case of recurrent MFS lies not only in its long asymptomatic period and identical clinical presentation, but also in its instructiveness regarding the differential diagnosis of MFS, particularly life-threatening botulism.     

Complete bilateral internal ophthalmoplegia as sole clinical sign of botulism: confirmation of diagnosis by single fibre electromyography

Summary A case of complete bilateral internal ophthalmoplegia as the sole clinical sign of botulism is reported. Diagnosis was immediately confirmed by single-fibre electromyography (SFEMG), which revealed abnormally high blocking (14.3%), contrasting with moderately increased jitter (mean consecutive difference in the extensor digitorum communis muscle, 43.9 s). After giving equine botulinum antitoxin and simultaneous forced emptying of the bowels, ocular symptoms completely disappeared within 2 days. Six days, 5 weeks and 6 months after the first SFEMG study, the jitter was still abnormal, even becoming more so with time. Blocking, however, was only rarely observed in the follow-up studies. It is concluded that SFEMG may serve as a useful and sensitive method for the rapid diagnosis of botulinum intoxication, even in cases where no clinical signs of general muscular weakness are apparent.This paper was presented in part at the Congress of the International Medical Society of Motor Disturbances (ISMD), Rome, Italy, 2–4 June 1988     

Infant botulism: Two case reports and electroneuromyogram findings

Botulism is an uncommon severe neuromuscular disorder. We report two recent cases of confirmed infant botulism diagnosed in an 11-week and a 5-month-old infant along with electroneuromyogram (ENMG) findings. Then, we discuss the EMG features of infant botulism. In severe forms of infant botulism, presence of these features might help decide to use botulinum immune globulin. To our knowledge, case 1 is the first case reported in France based on confirmed dust contamination.     

Botulismo en la UCI: proceso de cuidados

Introduction and case evaluation

Botulism is a rare disease in Europe, caused by the bacterium Clostridium botulinum, notifiable, non-transmissible person-to-person and potentially fatal (between 5 and 10%) if not treated quickly. The favourable opinion of the Clinical Research Ethics Committee was obtained. We present the nursing care plan of a 49-year-old man with a diagnosis of bacterial intoxication caused by Clostridium botulinum, secondary to ingestion of beans in poor condition, who was admitted to the ICU for a total of 35 days.

Botulism: Clinical neurophysiological findings

Summary Four cases of mild botulinus type B intoxication are reported with the clinical neurophysiological studies.Electromyographically tetanic stimulation of the N. ulnaris initially led to tetanic potentiation of the muscle action potential. After an interval without any symptoms tetanic reduction became apparent between the fourth and seventh week of the illness.The electroneurographic finding was normal during the observation period of three months.Infrared reflex pupillographic studies revealed that the cholinergic autonomous synapses were disturbed for a period much longer than the symptoms clinically established. The speed at which the pupillographic findings returned to normal varied considerably according to the patient.Electroencephalographic results indicated generalized groups of slow high amplitude waves in three of the four cases during the period of observation.The pathophysiology of botulinus intoxication is discussed with a consideration of the literature and the results of this study.

---

Zusammenfassung Bericht über klinisch-neurophysiologische Ergebnisse einer gutartigen Botulinus-Typ B-Intoxikation anhand von 4 Erkrankungsfällen.Elektromyographisch fand sich bei tetanischer Reizung des N. ulnaris anfänglich eine tetanische Potenzierung des Muskelsummenaktionspotentials. Nach einem unauffälligen Intervall zeigte sich zwischen der 4. und 7. Krankheitswoche eine tetanische Reduzierung.Der elektroneurographische Befund war über den Beobachtungszeitraum von 3 Monaten unauffällig.Infrarot-reflex-pupillographisch konnte gezeigt werden, daß die cholinergen autonomen Synapsen weit über die klinisch feststellbaren Symptome hinaus bei unterschiedlichen Verläufen der Rückbildung gestört waren.Elektroencephalographisch fanden sich bei 3 der 4 Patienten generalisierte Steilwellengruppen, welche sich bei 2 der Erkrankten innerhalb des Untersuchungszeitraumes zurückbildeten.Die Pathophysiologie der Botulinusintoxikation wird unter Berücksichtigung der bisherigen Literatur und anhand der eigenen Untersuchungsergebnisse diskutiert.

     

The recombinant Hc subunit of Clostridium botulinum neurotoxin serotype A is an effective botulism vaccine candidate

Vaccination with recombinant His-tagged isoforms of the Clostridium botulinum Hc domain of neurotoxin serotype A (rAHc) have effectively protected against challenge with active botulinum neurotoxin serotype A. To establish a formulation suitable for human use, rAHc was expressed in Escherichia coli without a His-tag and purified by sequential chromatography on ion-exchange and hydrophobic-interaction resins. Purified rAHc was used to vaccinate mice and survival was evaluated following challenge with active toxin. rAHc-vaccinated mice were protected against an active toxin challenge in mouse models of disease and a dose–response relationship was observed between the dose of rAHc administered and protection. Vaccination with rAHc in the presence or absence of adjuvants was also tested following intramuscular or subcutaneous vaccination to determine the optimal route of vaccination in the context of active toxin challenge. The data presented in the report suggested that rAHc administered with or without adjuvants functioned effectively over time in protecting mice against challenge with neurotoxin suggesting that this form of rAHc may be developed into a human vaccine candidate designed for the prevention of botulism.     

Vascular thrombi and neuronal alterations in human botulism

Summary Pathological study in a case of human botulism showed the presence of minor nerve cell changes and extensive vascular thrombi. The latter occurred chiefly in the central nervous system but occurred in other organs as well. An attempt to put these findings into proper perspective and offer a rational explanation for their frequent occurrence in this syndrome is undertaken. It is suggested that the hemagglutination factor present in botulin may cause changes which play a definite role in the clinical symptomatology. It may account for some observations not explainable by present theories which have limited the action of botulin to the neuromuscular junction.

---

Zusammenfassung Die histopathologische Untersuchung eines Falles von Botulismus beim Menschen ergab geringfügige uncharakteristische Nervenzellveränderungen und ausgedehnte rezente Gefäßthromben, die vorzugsweise im ZNS auftraten, aber auch in anderen Parenchymorganen nachweisbar waren. Die Bedeutung dieser Befunde wird erörtert und ihr häufiges Auftreten beim Botulismus zu erklären versucht. Es wird vermutet, daß der im Botulin enthaltene Hämagglutinationsfaktor eine grundlegende Rolle für die klinische Symptomatik und für einige durch die bisher vorliegenden Theorien nicht erklärbare Beobachtungen spielen dürfte.

     

肌电图在诊断肉毒中毒时的应用价值(附9例临床分析)

报告9例肉毒中毒的肌电图的临床分析。主要特点是用每秒5～50次连续电刺激神经,诱发肌肉动作电位波幅渐增,用此可协助确定肉毒中毒的诊断,但需和Lambert-Eaton综合征鉴别。