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Neuroborreliosis has its highest incidence in children and the older people. Signs and symptoms are different between the different age groups. The aim of this study was to describe the clinical spectrum of neuroborreliosis in children.The Dutch Paediatric Surveillance system registered cases of childhood neuroborreliosis during 2 years. All Dutch paediatric hospitals took part in this surveillance. Criteria for reporting cases were strictly defined.89 cases of neuroborreliosis were reported; in 66 cases diagnosis was confirmed. Facial weakness was one of the presenting symptoms in 47 cases (71%) and the only symptom in nine children (14%). The five complaints most frequently reported were: malaise, headache, fatigue, fever and neck pain. 52 children (79%) had one or more objective neurological signs at presentation, of which facial nerve palsy, other cranial nerve abnormalities and meningeal signs were most frequent. 14 patients (21%), however, had no neurological signs at physical examination. In these patients, the number of subjective complaints was higher, and the time interval to diagnosis was longer compared with those with objective neurological abnormalities.In this study, 79% of paediatric neuroborreliosis patients presented with neurological signs, most often facial nerve palsy. 21% presented in an atypical way without neurological signs. A thorough neurological examination is essential once neuroborreliosis is considered in children. Even in the absence of neurological signs, neuroborreliosis may be suspected in children with typical antecedents and multiple symptoms. Cerebrospinal fluid investigations are then required to confirm the diagnosis.