Bickerstaff’s brainstem encephalitis related to Campylobacter jejuni gastroenteritis

Campylobacter species are microaerophilic spiral-shaped Gram-negative bacilli which cause diarrhoeal and systemic illness in humans and animals. They are the most commonly identified cause of bacterial intestinal disease in the UK and elsewhere in the developed world. The bacterial cell wall of the organism contains an endotoxin which, when released, can result in acute enteritis and complications such as reactive arthritis, erythema nodosum, peripheral neuropathy, haemolytic uraemic syndrome and Bickerstaff’s brain stem encephalitis (BBE). Here we report a case of BBE following a gastrointestinal infection with Campylobacter jejuni. The patient presented with acute onset of confusion and ophthalmoplegia. The cerebrospinal fluid (CSF) showed lymphocytic pleocytosis and raised protein. This acute presentation was preceded by an episode of Campylobacter-related diarrhoea as confirmed by high titres of Campylobacter-specific IgM antibodies.

Over 50 000 laboratory confirmed cases of Campylobacter intestinal disease are reported annually in England and Wales.1 Campylobacter jejuni is the most common species of the genus Campylobacter identified in clinical samples.1 Transmission occurs throughout the year but rises in late spring and early summer, and requires only small inoculums.2 Clinical features of C jejuni infection range from an absence of symptoms to fulminant sepsis and death. In rare cases a potentially life threatening form of post-infectious encephalitis affecting the brainstem can arise.3 Initial colonisation occurs in the jejenum and ileum, which spreads distally to involve the colon and rectum. After a prodromal illness, the patient may develop abdominal pain and bloodstained diarrhoea. Other common complications include septic polyarthritis and peripheral polyneuropathy.

In 1957 Bickerstaff described eight patients with brainstem encephalitis …