Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome
Abstract
:1. Introduction
2. Materials and Methods
3. Results
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
References
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Case | Gender | Age at Onset | Age at Diagnosis | Clinical Features at Presentation | OMS Score at Diagnosis | Presence of Neuroblastoma | Treatment | Response | Age at Last Follow-Up |
---|---|---|---|---|---|---|---|---|---|
1 | Male | 23 months | 25 months | Unsteady gait, abnormal eye movements, opsoclonus, titubation, intention tremor and ataxia | 8/15 | No | Prednisolone IVIG | Good | 13 years |
2 | Female | 30 months | 34 months | Unsteady gait, vomiting, opsoclonus and regression in language | 12/15 | No | Dexamethasone | Good | 7 years 6 months |
3 | Female | 17 months | 17 months | Limb tremors, ataxia, loss of lower limb and truncal control and loss of speech | 11/15 | Yes | Surgical resection of neuroblastoma Dexamethasone Cyclophosphamide | Good | 7 years 8 months |
Case | Age | Intellectual Functioning | Other Domains Tested |
---|---|---|---|
1 | 4 years 11 months | FSIQ: 99 VIQ: 98 PIQ: 101 | Attention (selective): – Visuospatial skills: ++ Language (receptive): – Language (expressive): – |
6 years 7 months | FSIQ: 66 VIQ: 78 PIQ: 67 | Processing speed: ++ Language (receptive): – Language (expressive): – | |
8 years 4 months | FSIQ: 5 VIQ: 53 PIQ: 71 | Attention (working memory): ++ Processing speed: ++ Visuospatial skills: ++ Language (receptive): ++ Language (expressive): ++ Academic (reading): ++ Academic (spelling): ++ | |
2 | 5 years 6 months | FSIQ: 100 VIQ: 108 PIQ: 104 | Attention (sustained): – Attention (working memory): – Processing speed: – Visuospatial skills: + Memory (new learning): – Memory (delayed recall): – |
7 years 6 months | FSIQ: 81 VIQ: 81 PIQ: 82 | Attention (sustained): ++ Attention (working memory): – Processing speed: – Visuospatial skills: + Language (receptive): – Memory (new learning): – Memory (delayed recall): – Academic (reading): – Academic (spelling): – | |
3 | 5 years 3 months | FSIQ: 81 VIQ: 95 | Not tested |
7 years 8 months | FSIQ: 68 VIQ: 70 PIQ: 69 | Attention (sustained): ++ Processing speed: ++ Language (receptive): ++ Language (expressive): – Memory (new learning): – Memory (delayed recall): – Academic (reading): – Academic (spelling): – |
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Goh, E.L.; Scarff, K.; Satariano, S.; Lim, M.; Anand, G. Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome. Children 2020, 7, 103. https://doi.org/10.3390/children7090103
Goh EL, Scarff K, Satariano S, Lim M, Anand G. Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome. Children. 2020; 7(9):103. https://doi.org/10.3390/children7090103
Chicago/Turabian StyleGoh, En Lin, Kate Scarff, Stephanie Satariano, Ming Lim, and Geetha Anand. 2020. "Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome" Children 7, no. 9: 103. https://doi.org/10.3390/children7090103