Abstract
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.
Highlights
Neurodegenerative disorders of childhood include large, heterogeneous group of diseases that result from specific genetic and biochemical defects, chronic viral infections, and varied unknown causes
The acquisition of new developmental milestones does not exclude the existence of a degenerative disorder
First step would be to ascertain the age of onset of regression and the acquisition of various milestones prior to that
Summary
Neurodegenerative disorders of childhood include large, heterogeneous group of diseases that result from specific genetic and biochemical defects, chronic viral infections, and varied unknown causes. The hallmark of a neurodegenerative disease is regression and progressive deterioration of neurologic function with loss of speech, vision, hearing, or locomotion, often associated with seizures, feeding difficulties, and impairment of intellect [1]. The acquisition of new developmental milestones does not exclude the existence of a degenerative disorder. Most degenerative CNS disorders can be divided clinically into three groups: graymatter diseases, white-matter diseases, and system diseases [1,2]. Classification Approach to child with marked regression:
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