Cognitive Functioning in Patients with Pediatric-Onset Multiple Sclerosis, an Updated Review and Future Focus.

Pediatric-onset multiple sclerosis (POMS) is relatively rare, but as technology and neuroimaging advance, an increasing number of cases are identified, and our understanding of how multiple sclerosis (MS) impacts the developing brain improves. There are consistent findings in the literature highlighting the impact of MS and other demyelinating diseases on cognitive functioning and cognitive development. We also have a better understanding of how POMS impacts psychosocial functioning and functional outcomes in daily living. This paper hopes to review findings associated with cognitive and psychosocial functioning in patients with POMS, as well as explore more recent advances in the field and how they relate to cognitive and psychosocial outcomes. We also discuss the ongoing need for future studies with a focus on better understanding deficits and disease correlates, but also preventative measures and potential rehabilitation.

Long-Term Neurocognitive, Psychosocial, and Magnetic Resonance Imaging Outcomes in Pediatric-Onset Acute Disseminated Encephalomyelitis.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder that is usually self-limited. Recent studies have suggested ongoing neurological deficits and neurocognitive impairment in these patients. Little information on the correlation of clinical and neuroimaging markers in ADEM is available. We examined potential clinical factors (e.g., age of onset, acute symptom duration, magnetic resonance imaging [MRI] lesions) and their relation to neurocognitive and psychosocial outcomes. METHODS: This is a retrospective chart review of consecutive pediatric patients diagnosed with ADEM between 2006 and 2012. Patients were evaluated with standard neurological assessment, MRI of the brain, and neuropsychological evaluation. RESULTS: Twenty-three patients with ADEM with average age at neuropsychological assessment of 10.1 years (±3.50) were included. Five (22.7%) patients were impaired on three or more neurocognitive measures. Psychosocial problems were reported in 20%-40% of patients. Earlier age of onset was correlated with poorer sustained attention and psychosocial problems, whereas acute symptom duration and Expanded Disability Status Scale were not. MRI outcomes were correlated with psychosocial outcomes but not neuropsychological findings. CONCLUSIONS: Our findings suggest lingering cognitive and psychosocial deficits in children with a history of ADEM. Clinical features and MRI findings correlated more strongly with psychosocial outcomes than cognitive functioning. Further studies are needed to confirm relationships and other possible contributing factors to lingering deficits.

Cognitive deficits in pediatric-onset multiple sclerosis: what does the future hold?

Pediatric-onset multiple sclerosis is relatively rare and research regarding disease characteristics is in its infancy. Regardless, there are a growing number of studies finding early cognitive deficits in this population. There are some similarities in outcomes to those seen in the adult-onset multiple sclerosis population, but also several important differences. With specific regard to cognitive functioning in pediatric-onset multiple sclerosis, there is evidence of deficit in nearly a third of patients, with impairment primarily in areas of processing speed, visual-spatial processing and language. There are additional findings of fatigue and depression that impact functional outcomes requiring further attention in assessment and treatment considerations. This paper also explores other areas requiring increased focus, including treatment and outcomes, neuroimaging and additional disease-modifying factors (comorbidities, socioeconomic status, race and so on).

Fatigue and depression in children with demyelinating disorders.

Fatigue and depression have been shown to be significant problems in children with multiple sclerosis. The rate at which these conditions occur in children with other acquired demyelinating syndromes is unknown. In this cross-sectional study, the authors evaluated 49 children with demyelinating disorders (multiple sclerosis and acute disseminated encephalomyelitis) and 92 healthy controls for depression and/or fatigue using the Behavior Assessment System for Children, Second Edition behavior and mood rating scale and Varni PedsQL Multidimensional Fatigue Scale. The parents of acquired demyelinating syndrome patients were more likely to report elevated depressive symptoms (30.8% vs 10.8%, P = .008). Elevated parent and self-reported total fatigue (25% vs 0%, P < .001, 26.7% vs 8.6%, P = .024) was seen in the patient cohort. The authors conclude that fatigue and depression are far more common in children with acquired demyelinating syndromes than in controls. Clinical attention to and implementation of effective therapies oriented toward these conditions in children with acquired demyelinating syndromes is needed.

Acuted disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is a disorder of the central nervous system (CNS) characterized by an acute event, typically with encephalopathy, in which diffuse CNS involvement occurs. It may follow an infectious event and occurs more commonly in young children. Pulse steroid treatment is frequently used to treat ADEM. Although ADEM is typically described as a benign condition, with children generally recovering motor function and resolution of lesions on magnetic resonance imaging (MRI), residual cognitive deficits may occur. This chapter aims to review the clinical features, typical presentation, differential diagnosis, treatment and prognosis of ADEM.

Disease progression in pediatric multiple sclerosis: disparities between physical and neurocognitive outcomes.

Growing information regarding the neurocognitive burden of pediatric multiple sclerosis (MS) has become available in recent years. Whereas natural history studies suggest a longer time to irreversible disability in the pediatric population with MS than in the adult MS population, increasing knowledge suggests a relatively rapid accrual of cognitive deficits in this young population. This article will explore this paradox, taking into account studies that have focused on neuroimaging, cognition and outcomes in pediatric MS.

Cerebellar mutism in pediatric acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis is a demyelinating process affecting multiple areas of the central nervous system, frequently including the cerebellum. Cerebellar insult may lead to absence of speech or cerebellar mutism. Cerebellar mutism often occurs in young children after posterior fossa tumor resection, and generally appears as part of a larger subset of neurobehavioral signs and personality changes known as posterior fossa syndrome. Information on the impact of widespread cerebellar involvement on speech production, behavior, and long-term outcomes in acute disseminated encephalomyelitis is limited. We describe cases of acute disseminated encephalomyelitis with predominantly cerebellar involvement, with specific attention to cerebellar mutism. We conducted a retrospective chart review of children diagnosed with acute disseminated encephalomyelitis between 2005-2009 at a pediatric multiple sclerosis and demyelinating disorders clinic. Of 19 patients diagnosed with acute disseminated encephalomyelitis, six (32%) manifested primary cerebellar involvement. Of these six, four (67%) exhibited acute language disturbance, with three (50%) exhibiting mutism. The three patients with cerebellar mutism experienced protracted speech and language deficits after follow-ups from 6 months to 4 years. Widespread cerebellar involvement in acute disseminated encephalomyelitis may result in cerebellar mutism, in addition to persistent neurocognitive and behavioral problems.