DR. ASHWINI RAUT

Dr.VIRNA SHAH, Dr.Mansha Daswani

Abstract

A 16 year old male child presented with history of night blindness since childhood.He had been having trouble maintaining balance while walking since 7 years&trouble speaking since 10 months.History of Intramuscular botulinum toxin given to the tongue 2 months ago&T. Trihexphenidyl 2mg BD&T. Pramipexole 0.5mg TDS was tried.On general examination child had dysarthria, dystonia ,ataxia &decreased intellectual ability.Vision in both eyes was 6/6.Ocular movements showed bhlepharospasm, mild restriction in all gazes with nystagmoid movements & ill sustained pupils.Fundus examination of both eyes revealed pale optic disc with bull’s eye maculopathy and flecked retina.MRI brain reports showing symmetrically altered signal intensity in bilateral anteromedial globus pallidus showing T1 concentric hypo and hyperintense signal,T2 central hyper with peripheral hypointense signal,suggestive of Hallervorden Spatz Syndrome.He was started on a trial of T Haloperidol 0.25mg OD as per the neurologist.