Botulism and Eaton-Lambert Syndrome: Novel Concepts in the Classic Presynaptic Neuromuscular Transmission Disorders

The first cases of botulism were recognized by southern German physicians in the late 1700's who adopted the name "botulism" for the often fatal syndrome that occasionally occurred after consumption of blood sausage. Botulus is Latin for "sausage". It was not until 1895 that the Belgian microbiologist, Emile van Ermengen isolated the anaerobic gram positive bacterium from uncooked salted ham which had infected 23 musicians playing at a wake in the Belgian village of Ellezelles. A recurrent theme in case reports, even recent ones, has always been that additional persons have died tragically after the initial diagnosis has been missed. A 20 year old Kenyan nomad prepares sour milk in a gourd for a ceremony and samples the contents through a straw. She becomes ill and dies within 12 hours. After the funeral, the attendants are given refreshments including the sour milk preparation. Between one and four days later, ten fall sick and five die. Clostridium botulinum type A was isolated from the dry contents of the ourd. Similarly, there is a famous report of a woman who died of clostridium botulinum type B intoxication after eating home canned bottle mushrooms. Subsequently two other members of her family became ill and died after "just tasting" the suspicious mushrooms following her funeral. It is, therefore, in the interest of preventing this potent toxin, believed to be the most potent known neural toxic substance on a per milligram basis, that the study of botulism is prudent for all physicians. Indeed, it may be even more relevant to neuro-ophthalmologists as some reports suggest that up to 85% of patients present with double vision. Statistics also suggest that "blurred vision" may affect as many as 77% of cases and eyelid weakness approximately 46% in some series. There is no question that the rapid recognition of this disorder is related to survival.