Neurodegenerative disorders of childhood are complicated diseases with wide range of systematic involvement. These diseases
often pose great challenge to clinicians in terms of diagnosis and management. The purpose of this article is to outline a systematic
approach to a child presenting with suspected neurodevelopmental regression. Many inherited metabolic disorders present with
neural regression. The clinical approach depends upon the age of presentation, site of involvement in brain. Sound clinical knowledge
and better approach leads to early diagnosis, better management and above all genetic counselling. As the medical science is in the
track of rapid progression several treatment modalities are in the pipeline for neurodegenerative syndromes, early diagnosis and
referral to higher centres can bring a better future to the child.
Keywords: Neurodegenerative diseases; Hepatomegaly; White Matter; Grey Matter