Developmental regression can be described as the progressive loss of previously acquired skills which is relatively rare. Opsoclonus myoclonus syndrome is an uncommon autoimmune disorder in which a patient’s body attacks its own nervous system. It usually affects young children. Complicated developmental regression may lead to diagnosis of Opsoclonus Myoclonus Syndrome (OMS). A 3 years and 1 month old, the female child presented the history of convulsion, tonic contraction of upper and lower limb, up rolling of eye ball, frothing from mouth and post ictal drowsiness at the age of 1.5 years. On examination her general condition was unstable and systemic examination was not normal (Temp- 102.2 F). Her hematological and hepatic tests were done. Magnetic Resonance Imaging (MRI) report of her brain conclude that few T2 and FLAIR hyper intense foci are noted in bilateral periventricular deep white matter possibility of myelin pallor likely. We show that Opsoclonus myoclonus syndrome can be associated with developmental regression. Neurologist must be aware of such presentation. Our findings highlighted the need for increased recognition of developmental regression leading OMS as an urgent and treatable condition.
Key words: Opsoclonus myoclonus syndrome (OMS), Developmental regression, Paraneoplastic, Myelin pallor, Ataxia, Cerebellar nuclei degeneration.