Modern principles of diagnosis and treatment of Kinsbourne encephalopathy (a clinical case)
DOI:
https://doi.org/10.14739/2310-1210.2020.4.208413Keywords:
child, opsoclonus-myoclonus syndrome, diagnosis, treatment, prognosisAbstract
Opsoclonus-myoclonus syndrome (OMS) or Kinsbourne encephalopathy is a unique, rare neurological disease that causes difficulties in diagnosis, examination and treatment tactics. This article presents some organizational clinical, psychological and rehabilitation issues.
The aim of the study was to develop treatment tactics and means of preventing the recurrent development of neurological, behavioral and cognitive impairment in this pathology.
Results. Diagnosis of the disease was carried out based on a detailed study of anamnestic, molecular genetic, scintigraphic, neuroimaging, ultrasound, lymphocytic and neurological (opsoclonus, myoclonus, ataxia, behavioral and sleep disorders) signs. The severity of a child condition was determined using the Pranzatelli M. and Matthay K. scales. The clinical picture was represented by specific involuntary, arrhythmic, chaotic, multidirectional saccadic eye movements with horizontal, vertical, torsional components, behavioral changes, sleep disturbances, motor coordination dysfunction, the presence of myoclonic seizures, myoclonic jerks in the limbs and trunk, cerebellar ataxia and tremor. Slight cerebrospinal fluid lymphocytic pleocytosis was noted in the child, as it was described in the literature. Due to the fact that Kinsbourne encephalopathy has a paraneoplastic etiology in most cases, the child underwent brain MRI and CT as well as laboratory and instrumental examinations of the internal organs. The neuroimaging examination of the child’s brain indicated no focal density changes.
To rule out neuroblastoma, the child underwent ultrasound examination of the abdominal cavity organs and retroperitoneal space, a blood test for tumor markers, and measurements of catecholamine levels. To rule out parainfection and autoimmune processes, serum antibodies to various viruses were studied. Diagnosis of OMS was based on the analysis of the clinical picture, medical history and screening tests for oncopathology which was ruled out after a cytological study.
The prolonged multiple use of a combination of glucocorticoid, immunosuppressive and immunoglobulin therapy has been the essence of successful treatment. Currently, relative improvements in the emotional-volitional sphere can be reported - age-appropriate intelligence and vocabulary skills, pulls himself up to stand and walks without support. Ocular opsoclonus rarely occurs – more often in jitteriness.
Conclusions. The rare occurrence and anatomical and biological characteristics of this disease emphasize the importance of search for new specific methods of diagnosis and treatment to implement timely measures aimed at slowing the disease progression.
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