Bordetella pertussis (BP), the agent of whooping cough,has not been recogni
zed so far as a cause of permanent cerebellar ataxia in human. We describe
three patients who developed a disabling and permanent cerebellar syndrome
soon after whooping cough. In two patients, diagnosis of BP infection was c
onfirmed by culture of nasopharyngeal secretions. The infection occurred be
tween the age of 13 and 15 years, with neurological symptoms beginning afte
r a delay varying from 3 weeks to 3 months. In our three patients, the cere
bellar syndrome was characterized by dysmetria of ocular saccades, scanning
speech and ataxic gait. Brain MRI demonstrated a pancerebellar atrophy. Th
e pathogenesis of this cerebellar degeneration is not established. Experime
ntal studies have demonstrated that the cerebellum is particularly vulnerab
le to lymphocytosis-promoting fatter (LPF), one of the exotoxins from BP. T
he mechanism of this toxicity might be a marked increase in the cellular le
vels of 3',5'cyclic guanosine monophosphate (cGMP). Since whooping cough is
a bacterial exotoxin-mediated disease, this is the first report of a cereb
ellar syndrome triggered by a bacterial exotoxin. (C) 1999 Elsevier Science
B.V. All rights reserved.