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.
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
None. Sometimes chromosome 1 deletions are
found in the associated neuroblastoma tissue.
Autoimmune disease initiated by a viral infection
or a neuroblastoma and attacking the cerebellum (similarity of antigens).
Mainly clinical. Brain histology reveals widely
distributed perivascular lymphocyte infiltration in the brain.
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.
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.
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.
No known specific pharmacological
individuals affected with this condition receive chronic corticosteroid
therapy and may require perioperative supplements.
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
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
Pranzatelli MR, Tate ED, Kinsbourne M, et al: Forty-one year follow-up of
childhood-onset opsoclonus-myoclonus-ataxia: Cerebellar atrophy, multiphasic