Abstract

BARRAGAN-PEREZ, Eduardo; VELARDE-ESPINOSA, Silvia; GARZA-MORALES, Saúl  and  HERNANDEZ-AGUILAR, Juan. Non oncologic causes of Kisbourne syndrome. Bol. Med. Hosp. Infant. Mex. [online]. 2005, vol.62, n.3, pp.189-194. ISSN 1665-1146.

Introduction. In 1962 Kinsbourne describes a triad characterized by opsoclonus, myoclonus and ataxia.The incidence is rare; it occurs predominantly in pediatric population. It may express different etiologies; the most frequently cause associated is with paraneoplasia (50% with neuroblastoma).The great variety of diagnosis proposed for the Kinsbourne syndrome (KS) implies a great number of treatments too. Besides KS is rarely seen, the dramatic presentation, sudden appear, characterized triad and torpid evolution that can be in relationship with a paraneoplasia makes the syndrome a entity of great interest for the medical doctors, making of great importance known the different etiologies of each community for establish standardized protocols for the clinical study and follow adequate for these patients. With this revision, we suspect to identify the different etiologies in the KS in the population attended in the Hospital Infantil de Mexico Federico Gomez (HIMFG). Objective: describe and analyze the different causes of KS attended in the HIMFG between 1990-2004. Material and methods. With a retrospective study, we include patients of both sexes, <16 years, seen between 1990 and 2004. We study the frequency of patients seen in that period, the most frequent etiologies and the evolution of these patients. Results. Of 26 patients, the most frequent age of presentation was 21 months of age. The time between the first symptoms and the diagnosis was approx. 3.8 months.The initial symptom in 88.5% was ataxia.The most frequent etiologies related were infections, craneoencephalic trauma and inflammatory process. In 27% of the patients there were no antecedents. Only one of the patients was associated with neuroblastoma. Conclusion. The infection was the most frequent cause in our series that results implies the necessity to make strategies in the search of these patients.

Keywords : Kinsbourne syndrome; myoclonic jerks; opsoclonus; ataxia.

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