Kinsbourne syndrome occurs either spontaneously, in
association with a neuroblastoma, or following an infectious process.
Approximately 50% of cases are linked to an occult
neuroblastoma, but the clinical features and the response to therapy are
comparable with or without neuroblastoma. However, neuroblastoma associated
with Kinsbourne syndrome has a better prognosis than without it. Several
investigations point towards an immunologic process. Children usually present
with an encephalopathy with progressive ataxia, uncontrolled movements
of the head, myoclonic jerks, and chaotic jerking movements of the eyes.
Sometimes it is associated with mental retardation. In 60% of cases,
Kinsbourne syndrome improves with steroid or adrenocorticotropic hormone
(ACTH) administration. Recurrences and sequelae such as speech problems and
mental deficiency are found in approximately 90% of the cases and are
severe in 60%. Usually, Kinsbourne syndrome features improve with treatment of
the underlying neuroblastoma.