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Kinsbourne Syndrome. Should we modify our therapeutic behavior?
Last modified: 2014-04-03
Abstract
Introduction: Opsoclonus-myoclonus syndrome or Kinsbourne syndrome is a rare immunomediated disorder characterized by opsolconus, myoclonus, ataxia, irritability and sleep disorders. It is associated with neuroblastoma in up to 45% of cases. Neurological sequelae occur in more than 80% of cases. Treatment is based on immunosuppressive and immunomodulatory therapies, and surgery resection in neuroblastoma cases. For many years corticosteroids have been the gold standard. Afterwards, intravenous immunoglobulin was associated. Due to high prevalence of corticosteroid dependency, adverse events and poor outcome, multiple drug protocols have been proposed. Method: We reviewed clinical records of patients admitted with Kinsbourne syndrome between 2001 and 2013 at Pereira Rossell Children`s Hospital, Montevideo, Uruguay. We analyze clinical features and treatments. Neurological outcome was evaluated in the five children whose follow-up was greater than 2 years. Results: 8 patients were diagnosed in this period, with a mean age of 21 months. Table shows data obtained. All patients were treated with corticosteroids with initial improvement. Six children had cortical dependency, the other two had less than one year of follow-up. In three children with partial response to intravenous immunoglobulin and corticosteroids, or with cortical dependency, Rituximab was performed with clinical improvement. The five children with more than 2 years of follow-up had neurological sequelae including speech delay, cognitive deficits, motor delay, persistent ataxia and behavioral disturbance. Discussion: In our experience Kinsbourne syndrome has a poor clinical outcome so we support multiple drugs treatment, including corticosteroids, intravenous immunoglobulin and rituximab.
References
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Pract Neurol 2011; 11(3):160-6.
2. Brunklaus A, Pohl K, Zuberi SM, de Sousa C.Outcome
and prognostic features in opsoclonus-myoclonus syndrome
from infancy to adult life. Pediatrics 2011; 128(2):e388-94.
3. Gorman MP.Update on diagnosis, treatment, and prognosis
in opsoclonus-myoclonus-ataxia syndrome. Curr Opin Pe-diatr 2010; 22(6):745-50.
4. Pranzatelli M, Tate E. Opsoclonus-myoclonus syndrome.
En: Russell D, Vincent A.Inflammatory and autoimmune
disorders of the nervous system in children. Londres: Mac
Keith Press, 2010:152-73.
5. Matthay KK, Blaes F, Hero B, Plantaz D, De Alarcon P,
Mitchell WG, et al.Opsoclonus myoclonus syndrome in
neuroblastoma a report from a workshop on the dancing eyes
syndrome at the advances in neuroblastoma meeting in Ge-noa, Italy, 2004. Cancer Lett 2005; 228(1-2):275-82.
6. Krug P, Schleiermacher G, Michon J, Valteau-Couanet
D, Brisse H, Peuchmaur M, et al.Opsoclonus-myoclonus
in children associated or not with neuroblastoma. Eur J Pae-diatr Neurol 2010; 14(5):400-9.
7. Arroyo HA, Tringler N, De los Santos C.Síndrome de op-soclonus-mioclonus. Medicina (B Aires) 2009; 69(1 supl
1):64-70.
8. Tate ED, Pranzatelli MR, Verhulst SJ, Markwell SJ,
Franz DN, Graf WD, et al.Active comparator-controlled,
rater-blinded study of corticotropin-based immunotherapies
for opsoclonus-myoclonus syndrome. J Child Neurol 2012;
27(7):875-84.
9. Dalmau J, Rosenfenld M.Opsoclonus myoclonus ataxia.
UpToDate 2012. Disponible en: htpp://www.uptodate.com/
contents/opsolconus-myoclonus-ataxia. [Consulta: 18 mar
2012].
10.Alavi S, Kord Valeshabad A, Moradveisi B, Aminasnafi
A, Arzanian MT.Clinical responses to rituximab in a case of
neuroblastoma with refractory opsoclonus myoclonus ataxia
syndrome. Case Rep Oncol Med 2012; 2012:164082.
11.Pranzatelli MR, Travelstead AL, Tate ED, Allison TJ,
Verhulst SJ.CSF B-cell expansion in opsoclonus-myoclo-nus syndrome: a biomarker of disease activity. Mov Disord
2004; 19(7):770-7.
12.Pranzatelli MR, Tate ED, Swan JA, Travelstead AL, Co-lliver JA, Verhulst SJ, et al. B cell depletion therapy for
new-onset opsoclonus-myoclonus. Mov Disord 2010;
25(2):238-42
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