neurosurgeryНейрохирургияRussian journal of neurosurgery1683-32952587-7569Издательский дом "МедИНК"10.17650/1683-3295-2020-22-1-77-82neurosurgery-838Research ArticleНАБЛЮДЕНИЕ ИЗ ПРАКТИКИFROM PRACTICEХирургическое лечение болезни Лермитта–Дюкло (диспластической ганглиоцитомы мозжечка)Surgical treatment of Lhermitte–Duclos disease (dysplastic gangliocytoma of the cerebellum)БимурзинА. А.BimurzinA. A.

630087 Новосибирск, ул. Немировича-Данченко, 132 / 1

132 / 1 Nemirovicha-Danchenko St., Novosibirsk 630087

https://orcid.org/0000-0001-7003-5549КалиновскийА. В.KalinovskyA. V.

630087 Новосибирск, ул. Немировича-Данченко, 132 / 1

132 / 1 Nemirovicha-Danchenko St., Novosibirsk 630087

a_kalinovsky@neuronsk.ruФГБУ «Федеральный центр нейрохирургии» Минздрава России (Новосибирск)РоссияFederal Neurosurgical Center (Novosibirsk), Ministry of Health of RussiaRussian Federation2020110420202217782Copyright © Бимурзин А.А., Калиновский А.В., 20202020Бимурзин А.А., Калиновский А.В.Bimurzin A.A., Kalinovsky A.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.therjn.com/jour/article/view/838

Цель работы – описать клинический случай редкого опухолевидного поражения мозжечка – болезни Лермитта–Дюкло.

Клиническое наблюдение

Клиническое наблюдение. В Федеральный центр нейрохирургии (г. Новосибирск) поступил больной 40 лет. Из анамнеза известно, что в 2017 г. у него выполнено вентрикулоперитонеальное шунтирование справа по поводу гипертензионно-гидроцефального синдрома, вызванного объемным образованием правой гемисферы мозжечка. В июне 2017 г. пациент был консультирован нейрохирургом. Позже выявлены увеличение размеров образования, частичная атрофия дисков зрительных нервов. Ввиду наличия жалоб пациента на головную боль, атаксию, головокружение, а также недостаточного эффекта от шунтирования, проведено микрохирургическое удаление объемного образования мозжечка путем парамедианной субокципитальной правосторонней краниотомии. Операция выполнена под постоянным нейрофизиологическим контролем, благодаря чему стала возможна тотальная резекция образования в пределах интактной мозговой ткани. По результатам гистологического и иммуногистохимического исследований диагностирована диспластическая ганглиоцитома мозжечка (болезнь Лермитта–Дюкло) I степени злокачественности. В послеоперационном периоде общемозговая симптоматика полностью регрессировала, сохранился вестибулоатаксический синдром. Пациент выписан в удовлетворительном состоянии. За 7 мес наблюдения отрицательной динамики не наблюдалось.

Заключение

Заключение. Из-за редкости болезни Лермитта–Дюкло единые подходы к ее лечению на данный момент не разработаны, однако при симптомном течении имеются показания к хирургическому удалению ганглиоцитомы. При этом радикальность резекции должна быть соотнесена с риском появления неврологических нарушений. В нашем случае хирургическая тактика лечения позволила улучшить состояние больного, предотвратить дальнейшее прогрессирование болезни и поставить точный гистологический диагноз.

The study objective is to describe a clinical case of a rare disorder of the cerebellum, Lhermitte–Duclos disease.

Clinical case

Clinical case. The Federal Neurosurgical Center (Novosibirsk) admitted a male patient, 40 years old. Medical history showed that in 2017 he underwent ventriculoperitoneal shunt due to hypertensive hydrocephalus syndrome caused by space-occupying mass of the right hemisphere of the cerebellum. In June of 2017, the patient was consulted by a neurosurgeon. Later, an increase in the mass, partial atrophy of the optic disks were observed. Due to the patient’s complaints of headache, ataxia, vertigo, as well as insufficient effect of the shunt, microsurgical removal of the space-occupying mass in the cerebellum by right paramedian suboccipital craniotomy was performed. Gangliocytoma removal was performed under constant neurophysiological control which allowed to fully resect the gangliocytoma inside intact brain tissue. Histological and immunohistological examinations allowed to diagnose grade I dysplastic gangliocytoma of the cerebellum (Lhermitte–Duclos disease). In the postoperative period, brain symptoms regressed fully, vestibulocerebellar syndrome persisted. The patient was discharged in satisfactory condition. No negative dynamics were observed in 7 months of follow-up.

Conclusion

Conclusion. Due to the rareness of Lhermitte–Duclos disease, currently there are no established approaches to treatment, however, in symptomatic course there are indications for surgical removal of the gangliocytoma. Radicality of resection should be correlated with the risk of neurological defects. In our case, surgical treatment allowed to improve the patient’s condition, prevent further progression of the gangliocytoma and obtain an accurate histological diagnosis.

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The authors declare that there are no conflicts of interest present.