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Congress: ECR24
Poster Number: C-14864
Type: EPOS Radiologist (scientific)
DOI: 10.26044/ecr2024/C-14864
Authorblock: G. Ilangovan, A. N. Parimalai, S. ZUBAIR AHMED, M. K. Logudoss, M. MANIMARAN, M. Vogu, B. Kaveri, D. Avinash; Chennai/IN
Disclosures:
Gurubharath Ilangovan: Nothing to disclose
Anand Narsingam Parimalai: Nothing to disclose
SYED ZUBAIR AHMED: Nothing to disclose
Murali Krishna Logudoss: Nothing to disclose
MAHENDRAN MANIMARAN: Nothing to disclose
Mounika Vogu: Nothing to disclose
Balasubramaniam Kaveri: Nothing to disclose
Dhas Avinash: Nothing to disclose
Keywords: CNS, Neuroradiology brain, Paediatric, CT, MR, MR-Spectroscopy, History, Imaging sequences, Laboratory tests, Developmental disease, Genetic defects, Metabolic disorders
Purpose  Neurodegenerative disorders of childhood refer to a group of rare conditions that result from specific genetic, or biochemical defects, chronic viral infection, or toxic substances, leading to progressive damage of the nervous system. Degenerative brain diseases are infrequent in the pediatric population and when they manifest in early childhood, can result in severe developmental and physical impairments. Moreover, the acquisition of new developmental milestones does not exclude the existence of a degenerative disorder.Childhood Neuro-degenerative pathologies include a wide exhaustive spectrum...
Read more Methods and materials To review the etiology, pathology, and clinical issues of commonly encountered neurodegenerative diseases of childhood.To discuss the imaging features and distinct patterns of each disease using MRI and CT. 
Read more Results Metachromatic leukodystrophy:It is a lysosomal storage disorder with decreased arylsulfatase A leading to sphingolipid accumulation and progressive demyelination. It is the most common inherited leukodystrophy [10] and has three forms Late infantile (most common) Juvenile Adult Imaging findings:Centrifugal spread of demyelinationStarts in corpus callosum, splenium and deep parietooccipital white matter and frontal, temporal white matter are affected later and spares subcortical U-fibers and cerebellum.Classic butterfly patternSymmetric hyperintensities around frontal horns, atria (Fig 1 A)Tiger patternStripes of perivenular myelin sparing in white matter (Fig 1 A).Adrenoleukodystrophy:It...
Read more Conclusion Imaging evaluation, particularly through MRI, plays a central role in the diagnosis, management, and ongoing monitoring of neurodegenerative disorders in childhood. Advanced imaging modalities offer valuable insights into the structural, functional, and metabolic changes associated with these conditions, aiding in their differentiation, prognostication, and the development of targeted therapies.As our understanding of these disorders continues to evolve, imaging techniques will remain indispensable tools for unravelling their complexities and improving the care of affected children.  The integration of imaging data with...
Read more References Leukodystrophy in Children: A Pictorial Review of MR Imaging FeaturesJung-Eun Cheon, In-One Kim, Yong Seung Hwang, Ki Joong Kim, Kyu-Chang Wang, Byung-Kyu Cho, Je Geun Chi, Chong Jai Kim, Woo Sun Kim, and Kyung Mo YeonRadioGraphics2002 22:3, 461-476   Santosh Rai PV, Suresh BV, Bhat IG, Sekhar M, Chakraborti S. Childhood adrenoleukodystrophy - Classic and variant - Review of clinical manifestations and magnetic resonance imaging. J Pediatr Neurosci. 2013 Sep;8(3):192-7. doi: 10.4103/1817-1745.123661. PMID: 24470810; PMCID: PMC3888033   Subacute Sclerosing Panencephalitis Findings at MR Imaging, Diffusion MR Imaging, and Proton MR Spectroscopy R. Nuri Sener American Journal...
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