Paraneoplastic acute ataxia (opsoclonus-myoclonus syndrome) in children, description of two clinical cases
CLINICAL OBSERVATION
Abstract
The relevance of studying opsoclonus-myoclonus syndrome in children is due to the complexity of diagnosing this rare pathology, the frequent association of this condition with the neoplastic process, the peculiarities of therapy and the possibility of a recurrent course. This is one of the few paraneoplastic syndromes that occurs in both children and adults, although the mechanisms of immune dysfunction vary. Neurological dysfunction is caused by an autoimmune process and is observed in 3% of children with neuroblastoma. There is evidence of the development of the syndrome in celiac disease. In other cases, the disease may be associated with a viral infection (caused by Epstein–Barr viruses, Coxsackie B, influenza enteroviruses). There is evidence of rare cases of opsoclonus myoclonus syndrome associated with Lyme disease. The clinical picture is represented by ataxia, myoclonic paroxysms and saccading movements of the eyeballs. The article provides brief literature data on the etiology and clinical criteria of o syndrome opsoclonus-myoclonus in children, the importance of oncological search for the primary developing symptoms of ataxia, myoclonus and opsoclonus. The paper describes the authors’ own observations of two young patients who were treated at the St. Petersburg State Pediatric Medical University and the Children’s City Hospital No. 22 with the diagnosis of opsoclonus-myoclonus syndrome caused by poorly differentiated neuroblastoma of the paravertebral region and the retroperitoneal space. The article presents a description of the etiology (paraneoplastic process in the background of neuroblastoma), clinical picture, features of the course of the disease and therapy, data of neuroimaging, electroencephalographic studies and laboratory tests. Features of the first case are due to the rapid development of neurological symptoms, in the second observation, the syndrome of opsoclonus-myoclonus debuted in the early postoperative period. The study of opsoclonus-myoclonus syndrome presents significant difficulties, due to both the rarity of this pathology and the difficulties of diagnosis. Patients are more likely to seek medical care precisely because of the development of neurological symptoms, rather than because of the clinical manifestations of the tumor. To standardize the diagnosis and treatment, it is necessary to combine data on all diagnosed cases, which was the basis for writing this work.
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