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Demyelinating disease in children can present as acute disseminated encephalomyelitis (ADEM), transverse myelitis, or optic neuritis. Although these diseases are typically monophasic, recurrences do occur that might suggest multiple sclerosis (MS). Thirty cases of demyelinating disease diagnosed by clinical and MRI findings at Children's Hospital of Birmingham, AL, during a 10-year period were reviewed. Mean patient age was 7.8 ฯฎ 4.7 years (range, 7 months to 17 years). Methylprednisolone (2-10 mg/kg/d for 5 days) was used in 24 patients, with improvement in all but 1. Follow-up data were available for 23 patients (mean followup, 23 ฯฎ 21 months; range, 1 month to 8 years). Sixteen patients (53%) were diagnosed with ADEM; 75% of these patients presented with altered mental status (AMS), and 33% had seizures. Five patients with ADEM (31%) had up to three recurrences. Transverse myelitis occurred in 7 patients (23%), including 1 child with concurrent ADEM; the only recurrence in this group occurred in a patient with Devic's disease. Optic neuritis was the presenting symptom in 4 patients (13%), 2 of whom now have MS. Clinically definite MS was diagnosed in 7 patients (23%; mean age, 12 ฯฎ 3 years). Risk factors for MS included MRI findings of perpendicular periventricular white matter lesions without deep gray matter involvement and CSF with oligoclonal bands or increased IgG synthesis. These data show that 40% of our patients with demyelinating disease experienced recurrence, but MS was not likely to develop in patients who presented with AMS, seizures, or transverse myelitis.
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