Neurobehavioral and Psychosocial Functioning of Children With Opsoclonus‐myoclonus Syndrome

Abstract

SUMMARY Childhood opsoclonus‐myoclonus syndrome (OMS) is a movement disorder which typically strikes children in the early preschool years, seriously affecting intellectual, social‐emotional and general adaptive development. This series of 13 cases with well‐documented neurological histories, aged 1.7 to 16.3 years, provides an initial systematic evaluation of these children's neuropsychological, psychosocial and adaptive status. As expected, children with OMS had significantly reduced intelligence and severe speech and motor output problems; however, most of them also demonstrated a range of preserved neurocognitive abilities and impressive goal‐directedness and communicative effort. Psychosocial problems included mild behavioral impairment on the Achenbach Child Behavior Checklist, and severe adaptive limitations on the Vineland Adaptive Behavior Scales despite relatively strong social skills. Developmental factors and likelihood of subcortical localization are discussed, and practical guidelines are provided for behavioral and educational management of these children. RÉSUMÉ Caractéristiques neurocomporiementales el psychosocials du syndrome opsoclonies‐myoclonies Le syndrome infantile opsoclonies‐myoclonies (OMS) est un trouble moteur qui frappe les enfants dans les premières années préscolaires. affectant gravement le développement intellectuel, socio‐affectif et d'adaptation générate. Cette serie de 13 cas âgés de 1,7 à 16,3 ans, avec une histoire neurologique bien documentée fournit un début d'ébut d'évaluation systématique de l'état neuropsychologique, psychosocial et adaptatif. Comme on pouvait s'y attendre les enfants avec OMS ont une intelligence significativement réduite et des problèmes graves de langagc et de motricité; néanmoins, la plupart d'entre eux conservaient des aptitudes neurocognitives, la capacité d'agir avec un but et de communiquer. Les problèmes psychosociaux incluaient un trouble comportemental modéréà la Achenbach Child Behavior Checklist et de graves limitations aux Vineland Adaptive Behavior Scales en dépit d'activités sociales relativement évoluées. Les facteurs de développement et la localisation sous corticale vraisemblable sont discutés et des conseils sont donnés pour la prise en charge comportementale et pédagogique de ces enfants. ZUSAMMENFASSUNG Verhaltensneurologischer mid psychosozialer Status bei Kindren mit Opsoklonus‐Myoklonus Syndrom Das Opsoklonus‐Myuoklonus Syndrom (OMS) ist eine Bewegungsstörung, die typischerweise bei Kindern im frühen Schulalter auftritt und die intellektuelle, sozio‐emotionale und allgemeine adaptive Entwicklung schwer beeinträchtigt. Diese Studie beinhaltet 13 Fälle im Alter von 1,7–16,3 Jahren mit gut dokumentierten neurologischen Anamnesen und liefert eine erste systematische Beurteilung des neuropsychologischen, psychosozialen und adaptiven Status dieser Kinder. Wie erwartet hatten OMS Kinder eine signifikant verminderte Intelligenz und schwere sprachliche und molorische Problems, jedoch zeigten die meisten von ihnen auch eine Reihe erhalten...

Long-term outcome of ten children with opsoclonus-myoclonus syndrome

“…In terms of ataxia, our patients demonstrated a better outcome as compared with the cases in the literature [6,8,[12][13][14][15][16]. As shown in Table 1, only one of our patients had mild ataxia at follow-up; four were graded as minimal with normal gait and no motor handicap in everyday life, but they were not age appropriate in standing on one leg.…”

“…Papero et al [14] reported T scores of more than 60 in 7/8 of the children in the CBCL; IQ ranged from 50-87 and verbal fluency was disrupted in 12/13. In the study by Pohl et al [16], 32/54 patients had a learning disability and 33/54 a speech abnormality.…”

“…The long-term neurological outcome of patients with OMS has been studied in patients with associated neuroblastoma [7,8,13,15] and in mixed populations of patients with OMS of paraneoplastic, "idiopathic" or parainfectious origin [6,14,16,24]. In these reports, paraneoplastic OMS was seen in 7-90% of the patients.…”

“…There are also studies of mixed populations, including patients with OMS of paraneoplastic or parainfectious origin [6,12,14,16,24]. The reports of long-term outcome include descriptions of persistent and disabling ataxia, speech abnormalities, behavioural problems, and learning disabilities of differing severity and frequency.…”

Autopsy case of opsoclonus–myoclonus–ataxia and cerebellar cognitive affective syndrome associated with small cell carcinoma of the lung

“…The prognosis of OMA associated with neoplasm has generally been poor. In children, the development of opsoclonus–myoclonus symptoms is often associated with permanent behavioral and cognitive deficits 13, 14…”