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KINSBOURNE SYNDROME: A CASE REPORT

Resumo

CASE PRESENTATION: Male infant, 1 year old and 10 months, third child of non-consanguineous parents, 2 healthy older sisters and mother with 2 abortions, presenting with aggressive behavior 3 days after immunization with inactived polio vaccine and Hepatitis B vaccine in December 2021. Progression to ataxic gait and dysphagia after 15 days, with myoclonic movements of the upper limbs and opsoclonus after 30 days. Admitted to the emergency department in January 2022, submitted pelvis, abdomen and chest CT, Cerebrospinal fluid and brain MRI without alterations. Underwent 5 days of pulse therapy with methylprednisolone at 30mg/kg/day, with no change. Then performed IVIG at 2g/kg in 5 days, ceasing opsoclonus and improving gait. Myoclonus was controlled by Clonazepam. Discharged after 14 days but did not maintain the corticosteroid therapy at home. May 2022, he presented recurrence of opsoclonus and worsening of ataxic gait. Admission exam: Regular general condition; irritated; unintelligible speech; trophism, tone and strength preserved. Exacerbated deep reflexes (grade 3), globally. Exalted axial face reflexes. Did not load weight on the lower limbs, making gait assessment difficult. Coordination with dysmetria and global decomposition. A new course of IVIG was performed, with no change to the clinical presentation. Then, a new course of pulse therapy with methylprednisolone was realized with global improvement, except for gait ataxia, in a lesser degree. A new screening for occult neuroblastoma was performed, with normal CT of the pelvis, chest and abdomen. DISCUSSION: Kinsbourne syndrome is a rare inflammatory pathology, with no clear etiology. Up to 50% of cases are paraneoplastic, with neuroblastomas being identified in the majority of them. Despite the extensive search for neuroblastoma at this patient, no tumor processes were identified. He is still awaiting to perform full-body scintigraphy, in order to complete this search. FINAL COMMENTS: Early recognition of Kinsbourne syndrome enables early treatment, leading to faster clinical improvement, with less cognitive sequelae. There is no consensus as to which immunomodulatory treatment to use, which varies according to the response of each patient. The absence of neuroblastoma at the time of the clinical occurrence of the syndrome does not definitively rule out its presence, requiring a new periodic evaluation.

Palavras Chave

Opsoclonus-Myoclonus Syndrome; Pediatrics