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Cau C. 1, Martinoli C. 2, Scotti E. 1, Rosati E. 1, Coriddi F. 1, Bottone A. 1
1 IV Medicina, Azienda Ospedaliera S. Giovanni Addolorata - Roma;
2 DEA, Azienda Ospedaliera S. Giovanni Addolorata - Roma
The present paper synthetically reviews the historical, clinical, etiopathogenetic, anatomic and therapeutic data of a pathogenetically rather complex condition, Kinsbourne’s syndrome. This syndrome was codified for the first time by Kinsbourne in 1962 when he called it myoclonic encephalopathy of infancy. Etiology is unknown but a number of hypotheses have been formulated as regards both idiopathic and secondary forms: the most suggestive is currently the immunological-infectious. Kinsbourne’s syndrome, or infant opsoclonus-myoclonus is an uncommon pathology with acute or subacute onset characterised by opsoclonus, polymyoclonus of the striate muscles, cerebellar ataxia and may be accompanied by infections of the upper respiratory ways and of the gastroenteric apparatus. This disease is most frequently encountered in children (generally aged less than 3) and is often associated with neuroblastoma. Differential diagnosis must always be carried out with other conditions presenting myoclonic-ataxic forms that may mimic Kinsbournes syndrome. The therapeutic results with immunoglobulin and corticotherapy (ACTH or corticoids) have shown benefits only in some patients. Furthermore, recurrences and cerebral complications (dysarthria and mental changes) are frequent. It is important to recognise this syndrome in good time to prevent the implications of the underlying disease and in the interest of prognosis.