Long-term neurological outcomes of children with neuroblastoma with opsoclonus-myoclonus syndrome

Abstract

Background: Neuroblastoma with opsoclonus-myoclonus syndrome (OMS-NB) is a rare disease in children. Few studies of long-term outcome of children with OMS-NB were conducted. This study aimed to review the rate of recovery of neurological symptoms and the long-term neurological outcomes of children with OMS-NB. Methods: This study retrospectively assessed 14 children with OMS-NB diagnosed at Peking University First Hospital from May 2011 to November 2019. Demographic data, neurological symptoms, oncological status and treatments were retrospectively reviewed from the records. Neurological sequelae were recorded by clinical and remote follow-up. Results: During the acute stage, myoclonus and ataxia were observed in all children while opsoclonus was observed in 10/14 children. The median durations of neurological symptoms were 15 months (range, 5-48 months). Approximately 93% (13/14) children revealed neurological sequelae. Significant correlations were as follows: motor retardation with female gender (P<0.001) and residual tumors (P<0.05); language impairment with non-adrenal-gland-located tumors (P<0.05). There were no obvious factors that had a statistical relationship with cognitive disorder or behavioral changes. Conclusions: Children with OMS-NB have favorable outcomes in terms of neurological symptoms. Neurological sequelae occurred in almost all children. Children with different features tend to reveal different sequalae. Features of female gender and residual tumors tend to reveal motor retardation while that of non-adrenal-gland-located tumors tend to reveal language impairment.

Five Years Follow-up of Opsoclonus–Myoclonus–Ataxia Syndrome-Associated Neurogenic Tumors in Children

“…Speech and behavioral problems are especially detected. [19][20][21][22] The rate of neurological sequelae, including the persistence of OMAS symptoms, was reported as 56.5% in one study, while in another, it was reported between 20 and 60%. 13,22 In our study, the rate of sequelae is 33%.…”

Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients

“…Sun et al, in a retrospective review of 14 children with OMAS and neuroblastoma, reported that approximately 93% children had some neurological sequelae, with behavioral changes being the most common, followed by language impairments, motor retardation, and cognitive disorders. They also reported that the risk factors for neurological sequelae were the female sex, a residual tumor, and a non-adrenal-gland-located tumor [ 90 ]. Goh et al followed three children with OMAS from 4 to 10 years of age and reported that significant deficits in attention, processing speed, visuospatial skills, and language were observed in the long term [ 91 ].…”

Late cognitive and adaptive outcomes of patients with neuroblastoma‐associated opsoclonus‐myoclonus‐ataxia‐syndrome: A report from the Children's Oncology Group

“…10 Given the rarity of this disease, however, the limited research on the neurocognitive functioning of patients with NA-OMAS has been largely based on retrospective studies involving small samples and case reports. [11][12][13][14][15][16][17][18] There have been no prospective trials that included serial assessment of cognitive or psychological outcomes.…”