ICNC Abstracts, ICNC 2018

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The case of Kinsburn`s Encephalopathy in a child with neuroblastoma of posterior mediastinum
Tetiana Stetsenko

Last modified: 2018-09-09

Abstract


The aim of the article is to present a case of the rare Kinsburn`s Encephalopathy - opsoclonus-myoclonus-ataxia syndrome (OMAS) and to attract the attention to its timely diagnosis.

A clinical case of Kinsburn`s Encephalopathy in a child at the age of 1 year and 9 months is presented in this article.

Neurological status: eyes opsoclonus, truncal tremor, ataxia of the cerebellum. There were also changes in behavior (mood, anxiety, aggression during every day). She became sick in 1 month after acute respiratory infection. There were periods of exacerbation and improvement. MRI of brain was normal. The immunocytological examination of peripheral blood lymphocytes subpopulation (cellular immunity) and determination of the immunoglobulins level (humoral arm). CT of the chest and abdomen was done. An irregular tumor mass was detected on series of chest CT scans. So the common cause of the disease has been determined. It was neuroblastoma.

Помимо диагноза, ребенок сразу же начал получать адекватную терапию - внутривенный иммуноглобулин в дозе 2 г / кг для 5-дневного курса. На фоне полученной терапии у ребенка отмечалась положительная динамика, она начала ходить сама по себе, атактический синдром уменьшился, opsoclonus остался неизменным.

Радикальное удаление легочной нейробластомы было выполнено с последующим руководством к Институту рака.

Согласно результатам анализа морфологии и иммуногистохимии, фенотип, полученный в материале операции, характерен для нейробластомы (код ICD-O 9500/3), pT1bpN0M0, отрицательная форма.


Keywords


neuroblastoma; paraneoplastic syndrome; opsoclonus-myoclonus; Kinsburn`s encephalopathy

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