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 of entities amongst which the most commonly encountered are - Metabolic diseases, Glycogen storage disorders, Leukodystrophies, SSPE, Wilsons, Frederich ataxia, Lipoidosis, Hellerverden Spatz disease, Ataxia telangiectasia, Multiple sclerosis.

Given the complexity of these conditions, an accurate early diagnosis can help to guide & plan prompt therapy, follow-up protocols to assess the therapeutic response or disease progression, and further genetic counselling and prevention in siblings.

 Dedicated CT and MR evaluation of these cases usually show some typical patterns with specific lobar / sites of parenchymal involvement and are seen as signal intensity changes with/without associated cerebral atrophy.