Introduction

• Megaloencephalic leuco-encephalopathy, formally known as Van der Knaap disease, refers to a rare inherited autosomal recessive disease
• Characterized by macrocephaly with diffuse confluent white matter T2/FLAIR hyper-intensity in the sub-cortical white matter.
• Here is a  case    of   megaloencephalic   leucoencephalopathy (van der knaap disease) diagnosed on CT and MRI in FAAMCH IN LOWER ASSAM OF NORTHEAST INDIA.

1. Tu YF, Chen CY, Huang CC, Lee CS. Vacuolating megalencephalic leukoencephalopathy with mild clinical course validated by diffusion tensor imaging and MR spectroscopy. American journal of neuroradiology. 2004 Jun 1;25(6):1041-5.
2. Osborn AG, Salzman KL, Jhaveri MD, Barkovich AJ. Diagnostic imaging: brain E-book. Elsevier Health Sciences; 2015 Dec 24.

Dr Vishal Bahadur Chetry

Post Graduate Trainee, Department of Radiodiagnosis, Fakhruddin Ali Ahmed Medical College and Hospital, Barpeta, Assam