LAUSR

Annals of Indian Academy of Neurology ¦ Volume 24 ¦ Issue 6 ¦ November-December 2021 953 Dear Editor, Batten’s disease is a rare neurodegenerative disorder named after a British pediatrician Frederick Batten, who described the clinical entity in 1903.[1] It is considered to be a juvenile variant of neuronal ceroid lipofuscinoses (JNCL). These are a group of disorders characterized by abnormal deposition of substances like lipofuscin, ceroid, and lipopigments in the neurons of brain and other body tissues.[2] This accumulation leads to progressive atrophy of areas of brain which manifests as various neurological impairments. The early clinical features may include seizures, ataxia, and vision loss beginning between the ages of 5 and 8 years. The disease is progressive and invariably fatal leading to death in early 20s.[3] It is probably the first case report of JNCL with only seizures as presentation.