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Rare neurologic disorder usually affecting infants and young children. Characterized by sudden onset of brief, repeated, shock-like spasms of several muscles within the arms, legs, or the entire body. Impaired ability to control voluntary movements. Jerking movements of the eyes are most often present. In 50% of patients, a malignant tumor (neuroblastoma) is responsible for the symptoms associated with this syndrome. A viral infection may also be responsible.

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Ataxia-Opsoclonus-Myoclonus Syndrome; Dancing Eyes Syndrome; Dancing Eyes-Dancing Feet Syndrome; MEI Syndrome (Myoclonic Encephalopathy of Infants or Infancy Syndrome); Neuroblastoma Paraneoplastic Syndrome; Opsoclonic Encephalopathy; Opsoclonus-Myoclonus Syndrome.

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None. Sometimes chromosome 1 deletions are found in the associated neuroblastoma tissue.

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Autoimmune disease initiated by a viral infection or a neuroblastoma and attacking the cerebellum (similarity of antigens).

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Mainly clinical. Brain histology reveals widely distributed perivascular lymphocyte infiltration in the brain.

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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.

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Usually these children present for surgery of neuroblastoma or for radiotherapy. Hyperfractionated radiotherapy (twice daily) is used in some of these patients. Check for neuroblastoma location and catecholamine levels. Check for deviation or compression of the airways and great vessels, pneumonia, or pleural effusion with mediastinal or cervical neuroblastomas.

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No optimal anesthetic regimen can be recommended. Even if no catecholamine secretion is detected, hypotension may immediately follow tumor excision. Central neuraxial anesthesia should be avoided in case of paravertebral tumor invading the intravertebral space.

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No known specific pharmacological implications. Often individuals affected with this condition receive chronic corticosteroid therapy and may require perioperative supplements.

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Sydenham Chorea: Emotional instability, purposeless movements, muscular weakness; autoimmune response to acute rheumatic fever.

Kain ZN, Shamberger RS, Holzman RS: Anesthetic management of children with neuroblastoma. J Clin Anesth 5:486, 1993.  [PubMed: 8123275]
Plantaz D, Michon J, Volteau-Couanet D, et al: [Opsoclonus-myoclonus syndrome associated with non-metastatic neuroblastoma. Long-term survival. Study of the French Society of Pediatric Oncologists]. Arch Pediatr 7:621, 2000.  [PubMed: 10911528]
Pranzatelli MR, Tate ED, Kinsbourne M, et al: Forty-one year follow-up of childhood-onset opsoclonus-myoclonus-ataxia: Cerebellar atrophy, multiphasic relapses, ...

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