Last modified: 2014-04-03
Abstract
Introduction: Inflammatory demyelinating disease spectrum of the central nervous system comprises of Acute Disseminated Encephalomyelitis (ADEM), Multiple Sclerosis (MS), Clinically Isolated Syndrome (CIS) and Neuromyelitis Optica (NMO). Early diagnosis is imperative for optimal functional and neurological outcome.
Methods: Overall 35 cases presented between 2009 and 2013, with 11(31.4%) having recurrent episodes. These were classified according to the International MS study group and revised McDonald criteria (2010).
Results: Amongst the 11 patients with recurrent demyelination, majority were multiple sclerosis (8/11, 72.7%, 3 oligoclonal bands positive), followed by NMO (2/11, 18.2%) and multiphasic ADEM (1/11, 9%). The predominant clinical manifestations in MS were hemispheric syndrome (36.4%) followed by visual symptoms (22.7%) and encephalopathy(18.2%). One patient with NMO presented with recurrent episodes of encephalopathy with subclinical myelopathy without any visual involvement. In multiple sclerosis, lesions were predominantly juxtacortical and periventricular. One patient had diffuse bilateral involvement and another had tumefactive lesions. The acute attacks in MS and NMO were treated with steroids and azathioprine has been continued in all as the only disease modifying agent. Only one episode of relapse in one patient has been noted after starting azathioprine.
Conclusion: Atypical features like encephalopathy, tumefactive and diffuse bilateral lesions on neuroimaging have been infrequently reported in pediatric MS.1 Azathioprine, currently underutilized2, could be a suitable alternative to interferon and glatiramer acetate, particularly in resource limited settings.
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References
- Verhey LH et al. Neuroimag Clin N Am 2013; 23:227–243
- Casetta I et al. CD003982