Tigroid white matter on MRI is seen in?: (A) Pantothenate kinase deficiency, (B) Pelizaeus-merzbacher disease, (C) Neuroferritinopathy, (D) Aceruloplasminemia
Answer is B. The tigroid pattern/ leopard skin sign occurs on MRI head is seen in Pelizaeus-merzbacher disease, due to creation of islands of perivascular myelin due patchy myelin deficiency. It is a rare hypomyelination syndrome caused by mutation in proteolipid protein, PLP 1 gene at chromosome Xq22. Child will show slow psychomotor development with nystagmus(pendular eye movements), hypotonia, extrapyramidal symptoms and spasticity. Tigroid pattern is also seen in metachromatic leucodystrophy.