Dados do Trabalho

Título

ATYPICAL PRESENTATION OF OPSOCLONUS-MYOCLONUS-ATAXIA SYNDROME IN A NEWBORN: A CASE REPORT

Apresentação do caso

This is a newborn patient, male. Vaginal delivery with no complications, preterm birth. The initial physical examination of the newborn (NB) identified a hard and painful mass in the left flank.

The patient was transferred to Neonatal Intensive Care Unit (NICU) for extended workup and monitoring. In the first neurological examination, opsoclonus, myoclonus and ataxia of limbs and trunk were identified. During hospitalization, the NB developed systemic arterial hypertension. In Magnetic Resonance (MRI) an expansive formation was identified in upper and middle thirds of the left kidney. The newborn underwent total left nephrectomy and is being followed up by pediatric neonatology, neurology and oncology outpatients clinics.

Discussão

Opsoclonus-Myoclonus-Ataxia Syndrome, or Kinsbourne Syndrome, is a rare neurological pathology, prevalent in children, caused by autoimmune reactions and/or inflammation in the cerebellum or brain. Clinically, there is muscle incoordination of the trunk (ataxia), rapid eye movements (opsoclonus) and irregular spasms (myoclonus).

Kinsbourne Syndrome (KS) is a neuroimmune pathology frequently associated with post-infectious or paraneoplastic conditions. Post-infectious KS is associated with infections by Enterovirus, Epstein-Barr, Chikungunya, Flavivirus, among others. Neoplastic KS requires screening for primary tumors, especially neuroblastomas.

Often noticed before cancer suspicion, the case described is an early and atypical presentation of KS. After excluding infectious causes, patients with KS should be evaluated with radiologic screening of thorax, abdomen and pelvis.

The treatment of neurological symptoms of KS includes immunoglobulin and/or corticosteroids. In paraneoplastics cases, the immunomodulators are complemented with resection of primary tumor.

Comentários finais

In children with ataxia, opsoclonus and myoclonus symptoms it is mandatory to investigate possible causes for Kinsbourne Syndrome, such as infectious or neoplastic origin. The neurological and oncologic prognosis of patients is affected by time of diagnosis and treatment of primary cause.

Referências (se houver)

Pang KK, et al. A prospective study of the presentation and management of dancing eye syndrome/opsoclonus–myoclonus syndrome in the United Kingdom. European Journal of Paediatric Neurology. 2010; 14(2): 156-161.
Hero B, Schleiermacher G. Update on pediatric opsoclonus myoclonus syndrome. Neuropediatrics. 2013; 44(6): 324-329.
Do Rosário MS, et al. Opsoclonus-myoclonus-ataxia syndrome associated with chikungunya and dengue virus co-infection. International Journal of Infectious Diseases. 2018; 75: 11-14.
Maranhão MVM, Holanda ACF, Tavares FL. Síndrome de Kinsbourne: relato de caso. Revista Brasileira de Anestesiologia. 2013; 63(3): 287-289.
Titulaer MJ, et al. Screening for tumours in paraneoplastic syndromes: report of an EFNS task force. European Journal of Neurology. 2011; 18(1): 19-e3.
Blaes F, Dharmalingam B. Childhood opsoclonus-myoclonus syndrome: diagnosis and treatment. Expert review of neurotherapeutics. 2016; 16(6): 641-648.

Fonte de Fomento (se houver)

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