Rare case: doctors of Pediatric University told about the «dancing eyes» syndrome
Doctors of the Pediatric University diagnosed «Kinsbourne encephalopathy» to a child from Veliky Novgorod. This syndrome is extremely rare: the chance of getting it is 1 in 10 million.
A 1,5 year old boy from Veliky Novgorod went to a nursery for the first time, but soon fell ill with acute respiratory disease. The infection was severe: temperature rose up to 40 degrees. On the second day of disease, the child had tremors of the hands and head, he could not stand and sit. The boy spent 9 days in the regional hospital, and after visible improvement, he was discharged home. After a month and a half a second hospitalization was required: the child's temperature increased, and neurological symptoms intensified. The patient was sent for examination to the psychoneurological Department of SPbSPMU.
Specialists of the Pediatric University immediately suspected a rare disease – Kinsbourne encephalopathy. But to make a correct diagnosis, it was necessary to exclude neuroinfections. For this doctors performed extensive laboratory tests and a lumbar puncture. The presence of acute infection was not confirmed. This meant that the baby really had a rare disease.
Kinsbourne encephalopathy or, as it is also called, opsoclonus-myoclonus syndrome – is an autoimmune disease of the сentral nervous system. This disease is diagnosed in only 1 in 10 million people a year. The first symptoms appear in the return from 4 months to 6 years, often after an infection. A characteristic feature is chaotic movements of the eyeballs, hence another name for the disease – the «dancing eyes» syndrome. Patients with Kinsbourne encephalopathy have a high risk of tumor formation, so it is very important to detect the syndrome in time and start treatment.
The baby from Veliky Novgorod was thoroughly examined. Doctors performed CT scans of the chest, mediastinum and «multi-slice» computed tomography of the abdominal cavity and retroperitoneal space. In the chest of the child had revealed a space-occupying lesion. It became clear that the operation was necessary. After removal of the tumor and histological examination, it was found that the formation was benign.
The postoperative period went well and on the background of drug therapy the child's condition has improved. Independent walking resumed, the eyeballs stopped moving erratically, and salivation stopped. The boy was left under the supervision of neurologists and oncologists.
- Now the boy is at home and continues hormone therapy, – said the head of the psychoneurological Department of the Pediatric University Elena Efet and added: «The disease requires long-term monitoring, relapses are possible, it is necessary to monitor whether there is no growth of the tumor, be sure to adjust the dosage of hormonal drugs, so we are constantly in touch with doctors and parents of the baby».
At the moment there is no single approach to the treatment of Kinsbourne encephalopathy, but considering the immune nature of the development of this disease, doctors use glucocorticosteroids, adrenocorticotropic hormone and genetically engineered antitumor agents. The most important thing is to suspect the syndrome in time and start its treatment.
Date of publication: 11.08.2020