ABSTRACT
Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder with unknown etiology and pathogenesis. There is not enough knowledge about psychological development of children with OMS. AIM: To study an influence of the time of disease onset and children's age and type of disease course on psychological development of children with OMS. MATERIAL AND METHODS: The study included 26 children with OMS of age between 1 year 7 months and 13 years, 10 boys and 16 girls. The following methods were used: analysis of patient's development records, psychological interview with parents, assessment of emotional, behavioral, motion state and interpersonal relations. RESULTS AND CONCLUSION: The disease onset at the early stage of ontogenesis (before 3 years) results into severe psychological disorders. Patients at the age between 3 and 5 years demonstrate the maximal number of developmental delays with possibilities of compensation. The article discusses a possible role of psychotraumatic and psychosocial factors in psychological disorders in children with OMS. The influence of age should be taken into account in planning of treatment and rehabilitation and evaluation of disease prognosis.
Subject(s)
Child Development , Mental Disorders , Opsoclonus-Myoclonus Syndrome , Adolescent , Child , Child, Preschool , Cognition , Female , Humans , Infant , Male , Mental Disorders/etiology , Opsoclonus-Myoclonus Syndrome/complications , Opsoclonus-Myoclonus Syndrome/psychology , Parents , PrognosisABSTRACT
INTRODUCTION: Sociosanitary improvements experienced in western society have significantly increased the survival of patients with opsoclonus-myoclonus-ataxia syndrome (OMAS). However, several studies have reported neurological, cognitive-behavioral and development persistent deficits in 70-80% of these patients. PATIENTS AND METHODS: We reviewed cases of neuroblastic tumors diagnosed in a total period of 13 years and six months (from January 2000 to May 2013) and its association with OMAS in the pediatric service of a general hospital of a third level. Furthermore, it conducts a full neuropsychological study in three children diagnosed with OMAS. RESULTS: We objectified deficits in intelligence, attention, processing speed, memory, language, visuospatial and visuoconstructive skills, fine motor skills and executive functions. In addition, we found alterations in the psychological profile. CONCLUSIONS: Data emphasize the role of the cerebellum in complex cognitive processing in child population probably linked to neurodevelopmental deficits in this structure caused by deficiencies of the immune system. The results are interpreted in the framework of child neuropsychology and their interest in studying the brain-behavior relationships in the dynamic context of brain development.
TITLE: Perfil neuropsicologico en el sindrome opsoclono-mioclono-ataxia como forma de presentacion de tumores neuroblasticos.Introduccion. Las mejoras sociosanitarias experimentadas en la sociedad occidental han incrementado de forma significativa la supervivencia de los pacientes con el sindrome opsoclono-mioclono-ataxia (SOMA). Sin embargo, diversos estudios han informado de deficits neurologicos, cognitivo-conductuales y de desarrollo persistentes en el 70-80% de estos pacientes. Pacientes y metodos. Se revisan los casos de tumores neuroblasticos diagnosticados en un periodo total de 13 años y seis meses (desde enero de 2000 a mayo de 2013) y su asociacion a SOMA en el servicio de pediatria de un hospital general de tercer nivel. Ademas, se lleva a cabo la evaluacion neuropsicologica exhaustiva de tres niños diagnosticados de SOMA. Resultados. Hemos objetivado deficits en inteligencia, atencion, velocidad de procesamiento, memoria, lenguaje, habilidades visuoespaciales y visuoconstructivas, motricidad fina y funciones ejecutivas. Ademas, hemos comprobado alteraciones en el perfil psicologico. Conclusiones. Se aportan datos que enfatizan el papel del cerebelo en el procesamiento cognitivo complejo en poblacion infantil, probablemente vinculado a alteraciones neuromadurativas de esta estructura motivadas por deficiencias del sistema inmunologico. Los resultados encontrados son interpretados en el marco conceptual de la neuropsicologia Infantil y su interes por estudiar las relaciones cerebro-conducta en el contexto dinamico del desarrollo cerebral.
Subject(s)
Brain Neoplasms/physiopathology , Brain Neoplasms/psychology , Neuroblastoma/physiopathology , Neuroblastoma/psychology , Opsoclonus-Myoclonus Syndrome/physiopathology , Opsoclonus-Myoclonus Syndrome/psychology , Brain Neoplasms/etiology , Child , Female , Humans , Male , Neuroblastoma/etiology , Neuropsychological Tests , Opsoclonus-Myoclonus Syndrome/complicationsABSTRACT
Opsoclonus-myoclonus syndrome is a rare but serious neurological condition resulting in loss of control of eye movements, often accompanied by difficulties in posture and movement control with reports of sensory sensitivities potentially impacting on behavior. This pilot study characterizes the presence of atypical sensory behaviors in opsoclonus-myoclonus syndrome through questionnaire survey of a cohort of families. The Short Sensory Profile, Vineland Adaptive Behavior Scale, and Developmental Behaviour Checklist were distributed to 30 families; 16 were returned anonymously. Atypical sensory behaviors were identified in a large proportion (62.5%). Children reported as being more anxious showed greater sensitivity to auditory stimuli, U(14) 11, P = .026. This is consistent with recent recognition of more extensive disease neurocognitive effects in Opsoclonus-myoclonus syndrome. Further research is needed to increase understanding of the complex pathology of this disease and to provide indicators for sensory and behavioral as well as pharmacological interventions.
Subject(s)
Opsoclonus-Myoclonus Syndrome/physiopathology , Sensation Disorders/physiopathology , Adolescent , Anxiety/epidemiology , Child , Child, Preschool , Cohort Studies , Family , Female , Humans , Infant , Male , Opsoclonus-Myoclonus Syndrome/epidemiology , Opsoclonus-Myoclonus Syndrome/psychology , Pilot Projects , Prevalence , Sensation Disorders/epidemiology , Surveys and QuestionnairesABSTRACT
BACKGROUND: Kinsbourne syndrome or opsoclonus myoclonus syndrome (OMS) is characterized by rapid, involuntary, irregular conjugate eye movements (opsoclonus), myoclonic jerking of the limbs and trunk, ataxia, and behavioral disturbances. In general, the outcome of neurologic and behavioral symptoms is poor. Studies on quality of life (Qol) and recovery after very long-term follow-up of children with OMS are lacking. METHODS: We studied long-term cancer survival, neurologic recovery, and Qol of a consecutive series of eight patients with OMS that were treated in our center. Two cross-sectional follow-up evaluations were conducted at a 3-year interval (T1 and T2). RESULTS: In four out of eight children with OMS a neuroblastoma (NBL) was diagnosed and surgically removed completely. All children received immuno-modulatory therapy, consisting of prednisone (three children), prednisone and ACTH (four children), or prednisone, ACTH, and gammaglobulin (one child). At T1, median follow-up time was 9.5 years (range 3-14 years) after OMS onset and at T2, 11.6 years (range 6-17 years). Neurologic functioning improved in all children, reflected by a significant improvement of the mean Z-score in the motor domain of the Qol questionnaire at T2 as compared to T1. In contrast, seven children continued to have a severe developmental delay at T2. No significant difference in any of the variables was found between NBL survivors and OMS patients without NBL. CONCLUSION: Cognitive and behavioral impairments and not a motor impairment, such as ataxia, appear to be predominant at long-term in children with OMS.
