Acquired demyelinating disorders of central nervous system: A pediatric cohort

Abstract

Objective:This is a retrospective chart review of consecutive children with acquired demyelinating disorders presenting to a north Indian tertiary care hospital over 4 years. The aim of this review is to describe all the patients (with single event as well as those with recurrences) with detailed description of those who recurred.Materials and Methods:Overall 35 cases were reviewed and their clinical presentations, diagnosis, management, and follow-up are being presented.Results:Out of 35 cases, 24 did not show any recurrences (seven acute disseminated encephalomyelitis (ADEM) and 17 clinically isolated syndromes). Amongst the 11 patients with recurrent demyelination, majority were multiple sclerosis (8/11, 72.7%) followed by neuromyelitis optica (NMO; 2/11), and multiphasic ADEM (1/11). The median disease duration and follow-up since onset for those with recurrent episodes is 4 years (2.5-4.5 years). Steroids caused significant improvement in acute episodes of demyelination. However, recurrent demyelinating disorders like multiple sclerosis and NMO required long-term immunomodulation. Azathioprine currently is the most favored long-term immunomodulator used in NMO. Interferon-β and glatiramer acetate are currently recommended for multiple sclerosis. However, azathioprine may be a suitable alternative in a resource-limited setting.Conclusion:The consensus definitions for these groups of disorders need further validation in the pediatric age group. Studies with larger population size are required to characterize features that predict future recurrences.

Magnetic Resonance Imaging in the Acquired Demyelinating Disorders: A Pediatric Cohort Study

“…Curiously, but in accordance to what was observed in an Indian cohort, our younger (under 10 years old) patients had a majority of monofocal presentations [11].…”

Clinical and radiological profiles, treatment, and outcome of pediatric acquired demyelinating disorders of central nervous system

“…The most common presenting features of MS were hemiparesis, quadriparesis, and convulsions followed by sensory disturbances, bowel and bladder disturbances, and visual disturbances as observed in other studies. [2] Preceding febrile illness was observed in all three patients. All three patients with MS presented with encephalopathy in contrast to other studies where encephalopathy was not the presenting feature.…”

“…This group of disorders includes acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), clinically isolated syndrome (CIS), transverse myelitis, optic neuritis, and neuromyelitis optica (NMO). [2] Diagnosis of these disorders mainly depends on clinical findings and neuroimaging features. [3] Early institution of steroid therapy improves the outcome because of the autoimmune nature of this group of disorders.…”

“…[3] Early institution of steroid therapy improves the outcome because of the autoimmune nature of this group of disorders. [2] Exact knowledge about the incidence, clinical features, diagnosis, and treatment outcome is not known in pediatric age group. International Pediatric MS Study Group (IPMSSG) has proposed consensus definitions for this group of disorders for proper diagnosis and management and to facilitate uniformity in research.…”

Neurodevelopmental Disorders: The Journey, the Dreams and their Realization

“…In addition, it has contributed to the development of 2.5:1 ketogenic diet and proved its efficacy in institutional research (3) . The division also, has been the frontrunner in diagnosing, managing and optimizing the treatment for autoimmune encephalitis (8) , leukodystrophies (9) , and acquired demyelinating disorders of CNS (10) . Also, the research focusing on domiciliary m a n a g e m e n t o f i n t r a n a s a l a n d b u c c a l midazolam has helped to standardize the management protocols for seizures at home (11).…”