HGPS (Hutchinson-Gilford -Progeria syndrome)

Eight year old male child, born of non- consanguinity presented with complaints of irritability and drowsiness with history of left sided weakness. He was stunted and had hypertension at presentation. Also he had dysmorphic features and skin manifestations with skeletal deformities and muscle wasting.  Motor examination revealed muscle wasting with increased tone and decreased power on both sides. In Hutchinson -Gilford -Progeria syndrome, large and small vessel occlusive disease due to atherosclerosis can occur. CVA and Stroke can also result due to cerebral infarction or carotid artery occlusion. In our case, patient had MRI brain suggestive of areas of acute infarct and also evidence of chronic infarct and MR angiography suggestive of vasculopathic changes. Patient was started antihypertensives and tab asprin and gradually his sensorium improved, BP got normalized and planned discharge and advised regular follow up and physiotherapy.

Keywords: Progeria, Stroke, HGPS.