Clinical Presentation

Initial multiple sclerosis (MS) symptoms significantly vary. A confounding factor is the difficulty associated with detecting subtle findings, such as sensory changes or mild symptoms, in young children who have limited body awareness.

Encephalopathy is a common presenting symptom of ADEM. Several studies have shown that encephalopathy may also occur with a first episode of MS or neuromyelitis optica (NMO) and is usually reported in younger patients.^{[30, 31, 32, 33]}ADEM occurs most often in younger children. Thus, rather than being disease specific (ie, MS vs ADEM), the presence of encephalopathy may be related to immaturity of the brain or the immune system in younger patients.

Seizures as first event (as part of the encephalopathy or isolated) in the pediatric population were also reported (29%), usually in ADEM cases and less frequently in MS.^{[34, 32, 33, 35]}

The initial clinical course in the vast majority of patients with pediatric MS is relapsing-remitting^[21]to the point that a progressive course from onset in a pediatric patient with MS is a red flag. The annualized relapse rate in pediatric-onset MS has been estimated to be between 0.38 and 0.87 for the whole relapsing-remitting period in the few studies with mean disease duration of 10 years or more.^[21]

A prospective study of patients with MS at a large MS center showed that patients with an onset before age 18 years had a higher relapse rate during the first few years of their disease than adults seen at the same institution.^[36]While most relapsing-remitting patients have been reported to recover completely and quickly after relapses, disability can result from an incomplete recovery from relapses. In rare pediatric cases, patients later develop a more insidious progression of disability with or without superimposed exacerbations, called secondary progressive MS.

Differential Diagnoses

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Media Gallery

Periventricular increased T2 signal, as shown in this FLAIR image, can be seen in neuromyelitis optica given the dense concentration of aquaporin 4 water channels in this area.
Increased T2 signal at the cervicomedullary junction, as seen in a pediatric patient with MS.
An enhancing lesion in the right superior frontoparietal region. Image 4 shows the same lesion on FLAIR imaging.
FLAIR image of lesion in the right superior frontoparietal region.
This sagittal T2 image of the cervical spine shows small hyperintense lesions typical of MS at C2 C3.
This axial FLAIR image shows bilateral, left predominant confluent signal change. There is also an area of gray matter involvement in the right frontal region. This image would not be sufficient to distinguish an episode of ADEM from pediatric MS in a pre-pubertal child.

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Author

Alice K Rutatangwa, DO, MSc Fellow in Pediatric Multiple Sclerosis/Neuroimmunology, Department of Neurology, University of California, San Francisco, School of Medicine

Alice K Rutatangwa, DO, MSc is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Pediatric Academic Societies, Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Emmanuelle L Waubant, MD, PhD Professor of Clinical Neurology and Pediatrics, Director, Regional Pediatric MS Clinic at UCSF, Director, MS Fellowship Program at UCSF, University of California, San Francisco, School of Medicine; Medical Director, Nancy Davis Center Without Walls

Emmanuelle L Waubant, MD, PhD is a member of the following medical societies: American Academy of Neurology, American Neurological Association

Disclosure: Received grant/research funds from Roche for research project; Received grant/research funds from Biogen Idec for fellowship grant; Received honoraria from Teva for speaking and teaching; Received consulting fee from Novartis for board membership; Received honoraria from Genzyme for speaking and teaching; Received honoraria from Questcor for speaking and teaching.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA Professor of Pediatrics, Neurology, Neurosurgery, and Psychiatry, Medical Director, Tulane Center for Autism and Related Disorders, Tulane University School of Medicine; Pediatric Neurologist and Epileptologist, Ochsner Hospital for Children; Professor of Neurology, Louisiana State University School of Medicine

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association, American Neurological Association, Association of Military Surgeons of the US, Child Neurology Society, Southern Pediatric Neurology Society

Disclosure: Nothing to disclose.

Additional Contributors

Teri L Schreiner, MD, MPH Instructor, Fellow in Neuroimmunology, Children's Hospital Colorado, University of Colorado School of Medicine

Teri L Schreiner, MD, MPH is a member of the following medical societies: American Academy of Neurology, Child Neurology Society

Disclosure: Received grant/research funds from National MS Society for employment; Received consulting fee from Questcor Pharmaceuticals for consulting; Received grant/research funds from Teva Pharmaceuticals for other.

Pediatric Multiple Sclerosis Clinical Presentation

Clinical Presentation

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