OBJECTIVES: To estimate the incidence rate of Guillain-Barré syndrome variants 
in an unselected population and to describe their clinical features and 
prognosis.
METHODS: A two year prospective multicentre study on the incidence and prognosis 
of Guillain-Barré syndrome was performed in Emilia-Romagna, northern Italy 
(3,909,512 inhabitants). A surveillance system was instituted within the study 
area, which comprised all the neurological departments, private and public 
general hospitals, and practising neurologists. The international classification 
of diseases (ICD) codes 357.XX (any peripheral neuropathy) of hospital 
discharges were also reviewed.
RESULTS: Data were separately analysed for Miller Fisher syndrome and other 
Guillain-Barré syndrome variants. During the study period 18 patients with 
Guillain-Barré syndrome variants including seven with Miller Fisher syndrome 
were recruited; the incidence rates were 0.14/100000/year (95% confidence 
interval (95% CI) 0.07-0.25) for Guillain-Barré syndrome variants (excluding 
Miller Fisher syndrome) and 0.09/100000/year (95% CI 0.04-0.18) for Miller 
Fisher syndrome. Guillain-Barré syndrome variants alone (excluding Miller Fisher 
syndrome) accounted for 10.5% of total cases. Death and relapses were not found. 
Details of clinical, electrophysiological, and CSF findings of Guillain-Barré 
syndrome variants are provided.
CONCLUSIONS: Guillain-Barré syndrome variants other than Miller Fisher syndrome, 
as obtained through a population based study, account for about 10% of total 
cases of Guillain-Barré syndrome and, as a whole, have a good prognosis. Their 
clinical features are heterogeneous; bifacial weakness (associated with other 
signs, mainly sensory disturbances) represents the most frequent finding.