European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society · 2014
An 8-year old boy with continuous spikes and waves during slow sleep presenting with positive onconeuronal antibodies.
Клинический случайСтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Hu Lin-Yan, Shi Xiu-Yu, Feng Chen, Wang Jian-Wen, Yang Guan, Lammers Stephen H T, Yang Xiao Fan, Ebrahimi-Fakhari Darius, Zou Li-Ping
Аннотация
OBJECTIVE: To determine the etiology of epilepsy with continuous spikes and waves during slow sleep (CSWS)/electrical status epilepticus during sleep (ESES) in an 8-year old boy with a history of neuroblastoma and opsoclonus-myoclonus.
MATERIAL & METHODS: A combination of clinical characterization and follow-up, video EEG and laboratory investigations.
RESULTS: We report the case of an 8-year old boy with a history of neuroblastoma and opsoclonus-myoclonus, who presented with intellectual disability, pharmacotherapy-resistant epilepsy and CSWS/ESES. Although the patient's neuroblastoma had been successfully treated 8 years prior to presentation and an extensive workup did not show a tumor reoccurrence, testing for onconeuronal antibodies was positive for anti-Ma2 and anti-CV2/CRMP5 antibodies. High-dose intravenous methylprednisolone and a taper of oral methylprednisolone were given, leading to a significant clinical improvement. During the taper the patient's condition and EEG manifestations deteriorated again necessitating another cycle of steroid therapy, which lead to a stable improvement. During a 6-month follow-up no CSWS/ESES was seen on EEG and anti-Ma2 and anti-CV2/CRMP5 antibodies remained undetectable.
CONCLUSION: This case suggests that onconeuronal antibodies may be involved in the pathogenesis of CSWS/ESES.
MATERIAL & METHODS: A combination of clinical characterization and follow-up, video EEG and laboratory investigations.
RESULTS: We report the case of an 8-year old boy with a history of neuroblastoma and opsoclonus-myoclonus, who presented with intellectual disability, pharmacotherapy-resistant epilepsy and CSWS/ESES. Although the patient's neuroblastoma had been successfully treated 8 years prior to presentation and an extensive workup did not show a tumor reoccurrence, testing for onconeuronal antibodies was positive for anti-Ma2 and anti-CV2/CRMP5 antibodies. High-dose intravenous methylprednisolone and a taper of oral methylprednisolone were given, leading to a significant clinical improvement. During the taper the patient's condition and EEG manifestations deteriorated again necessitating another cycle of steroid therapy, which lead to a stable improvement. During a 6-month follow-up no CSWS/ESES was seen on EEG and anti-Ma2 and anti-CV2/CRMP5 antibodies remained undetectable.
CONCLUSION: This case suggests that onconeuronal antibodies may be involved in the pathogenesis of CSWS/ESES.
Предметные рубрики MeSH
Anti-Inflammatory AgentsAntibodies, NeoplasmAntigens, NeoplasmBrain NeoplasmsChildDrug Resistant EpilepsyElectroencephalographyEpilepsyHumansHydrolasesIntellectual DisabilityMaleMethylprednisoloneMicrotubule-Associated ProteinsNerve Tissue ProteinsNeuroblastomaOpsoclonus-Myoclonus SyndromeSleepStatus EpilepticusStrabismus