The case of Kinsburn’s Encephalopathy in a child with neuroblastoma of posterior mediastinum
DOI:
https://doi.org/10.15574/SP.2024.137.138Keywords:
neuroblastoma, paraneoplastic syndrome, opsoclonus-myoclonus, Kinsburn’s encephalopathyAbstract
Opsoclonus-myoclonus-ataxia syndrome (OMAS) is an autoimmune rare disease of the central nervous system with lesion of the cerebellum and its ligaments.
Purpose - to present a case of the rare Kinsburn’s Encephalopathy - OMAS and to attract the attention to its timely diagnosis.
Clinical case. A child at the age of 1 year and 9 months was observed in the neurological department of the National Children’s Specialized Hospital “OKHMATDYT”. Neurological status: eyes opsoclonus, truncal tremor, ataxia of the cerebellum. There were also changes in behavior and a sleep disorder. She became sick in 1 month after acute respiratory infection. True diagnosis was made: Kinsburn’s Encephalopathy - opsoclonus-myoclonus syndrome. A study of the child’s immune status was made and, most importantly, computerized tomography (CT) of the whole body. Mediastinal neuroblastoma was diagnosed.
In addition to the diagnosis, the child receive adequate therapy - intravenous immunoglobulin at a dose of 2 g/kg for a 5-day course. On the background of the received therapy, the child was marked with positive dynamics, she began to walk by herself, the atactic syndrome diminished, opsoclonus remained unchanged.
Consequently, the main cause of autoimmune defeat of the nervous system was detected and the child was directed to the further examination and treatment to the Cancer Institute of the National Academy of Medical Science of Ukraine.
Radical removal of the left hemopleura neuroblastoma was made. According to the results of morphological and immunohistochemistry tests, the phenotype obtained in the material of the operation is characteristic of the neuroblastoma (ICD-O code 9500/3), pT1bpN0M0, negative form. In the future, the child continued to receive courses of immunosuppressive therapy.
The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies.
No conflict of interests was declared by the authors.
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