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Boy with slightly impaired coordination

Reviewed by Krupa Pandey, MD

Living Art Enterprises, LLC/Science Source

A 10-year-old boy, typically active, presents with slightly impaired coordination and facial weakness. His parents noticed that his gait in particular seems impaired, though to his knowledge he had not been injured. His mother reports a history of meningoencephalitis. A sagittal T2-weighted MRI sequence shows a portion of the brainstem with a large demyelinating plaque in the dorsal part of the medulla and several other lesions in the periventricular regions of the brain. Spinal fluid is normal.

What's the diagnosis?

Schilder disease

Pediatric multiple sclerosis

Acute disseminated encephalomyelitis

Transverse myelitis

Neuromyelitis optica

This young patient is probably presenting with pediatric multiple sclerosis (MS). It is estimated that MS onset before the age of 18 years accounts for 3%-5% of the general population of patients with this autoimmune disease. The condition represents the most common nontraumatic, disabling neurologic disorder among young adults. Disease prevalence is highest between the ages of 13 and 16. In children older than 10, a female predominance is seen, suggesting a hormonal role in pathogenesis. The vast majority (up to 98%) of children and adolescents with MS have a relapsing-remitting course. Overall, pediatric MS has a milder course than adult MS but can lead to significant disability at an early age. Although pediatric patients may experience more frequent relapses, data also suggest that children seem to recover more quickly from episodes than adults.

In children and adolescents, MS most typically manifests with sensory disturbances and impaired coordination. The most commonly reported symptoms in pediatric MS are sensory, motor, and brainstem dysfunction, though cognitive and emotional disorders can emerge over time.

Younger children will often show multifocal symptoms but with the onset of adolescence may begin to present with only a single focal symptom, as is often the case with adult patients.

Diagnosis of pediatric MS goes hand-in-hand with a diagnosis of clinically isolated syndrome (CIS) or sporadic acute disseminated encephalomyelitis (ADEM). CIS is diagnosed when symptoms last for over 24 hours with possible inflammatory demyelination but without encephalopathy. To confirm an MS diagnosis, two or more clinical episodes must occur at least 30 days apart. MRI can both confirm diagnosis and offer great value in monitoring disease progression in the brain and spinal cord. Of note, differentiating the first episode of juvenile MS from ADEM is a significant clinical challenge.

When it comes to treating relapses, the approach in children is similar to that of adults. Therapy may consist of an intravenous pulse of methylprednisolone (20-30 mg/kg/day for 3-5 days). In 2018, the FDA approved the use of the oral MS therapy Gilenya (fingolimod) for the treatment of patients 10 years of age or older with relapsing forms of MS. Providers can also adapt treatments approved for adults for pediatric patients.

Krupa Pandey, MD, Director, Multiple Sclerosis Center, Department of Neurology & Neuroscience Institute, Hackensack University Medical Center; Neurologist, Department of Neurology, Hackensack Meridian Health, Hackensack, NJ

Krupa Pandey, MD, has serve(d) as a speaker or a member of a speakers bureau for: Bristol-Myers Squibb; Biogen; Alexion; Genentech; Sanofi-Genzyme

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