Acute cerebellar ataxia in the Netherlands: A study on the association with vaccinations and varicella zoster infection
Introduction
Acute ataxia in childhood has a wide range of causes. The differential diagnosis for acute ataxia in children is broad and includes toxicological, infectious, structural and metabolic causes [1], [2], [3].
The most common cause (40%) of childhood ataxia is acute cerebellar ataxia (ACA) with an estimated incidence of 1:100,000–500,000 children per year [4]. ACA is characterized by the acute onset of truncal ataxia and gait disturbances, sometimes combined with nystagmus or other (involuntary) eye movements [1], [3], [4], [5]. Usually ACA follows a benign (viral) illness. Varicella is preceding ataxia in about one-quarter of the cases. Other possibly related infections are Epstein Barr virus, Enterovirus, mumps, hepatitis A, and Influenza A and B [1], [6], [7], [8], [9], [10], [11], [12]. ACA sometimes follows vaccines, like measles, hepatitis B and Influenza, but a causal relationship has not been established and attributable risks are not known [1], [13], [14], [15], [16].
For varicella, ACA is the most common neurological complication, occurring in 1:4000 children under 15 years of age in the USA [3], [17], [18]. Passive postmarketing surveillance (PMS) data show a reporting rate of ACA following varicella vaccination of 1.5:1,000,000 doses [19]. The enhanced passive safety surveillance system for the National Immunization Programme (NIP) of the Netherlands occasionally receives reports of ataxia, mainly following MMR (measles, mumps, rubella) vaccination [20]. However, in most cases other possible causes of ataxia were not excluded or other viral infections coincided with the vaccinations.
Currently the uptake of varicella zoster vaccination in the NIP is under consideration in the Netherlands [21], [22], [23], [24]. Until now no information is available on incidence rates of total ACA, ACA following varicella infection and the attributable risks of MMR to ACA in the Netherlands. Therefore, this paper presents the results of a survey, conducted through active, prospective pediatric surveillance. We describe the occurrence and clinical aspects of ACA in the Netherlands in 2002 and 2003. Preceding infections were established with special focus on varicella. To assess underreporting and estimate the total burden of ACA in the Netherlands, we compared hospitalized cases of the pediatric surveillance with national hospitalization data using capture–recapture method for two sources. Furthermore a possible association with vaccinations was investigated.
Section snippets
Identification of patients with ACA
Patients with ACA in the study period 2002–2003 (24 months) were identified from three sources:
- 1.
The Netherlands Pediatric Surveillance Unit (NSCK):
This system is an active, prospective surveillance among pediatricians, reporting monthly whether they have seen a patient in hospital with a disease included in the system for that period. Purpose is a better understanding of rare and/or new disorders in childhood. There is national coverage and the response varies between 83% and 92%. For the
Patient characteristics
In 2002 and 2003, NSCK reported 55 children. Four reports were duplicates and seven children did not meet the inclusion criteria for ACA. In the same period, RIVM received eight reports of possible ACA through the safety surveillance system. Only two of these met the inclusion criteria for ACA and one of them was identical to a NSCK-report. Consequently, 45 children with ACA were included. Additional information was obtained for 43 cases through the questionnaire, discharge letter and
Discussion
This study gives an overview of the incidence rate of acute cerebellar ataxia in the Netherlands. The syndrome has a peak incidence between 2 and 4 years and boys were more often affected than girls. The clinical picture is usually mild with sometimes (8%) mild sequelae. We found no association with vaccination.
According to the CRC-estimation the incidence rate of hospitalized ACA for the Netherlands was 0.7:100,000 per year for children under 15 years of age. This is in line with other
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