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Radiology Neuroradiology b06d4abd

Tigroid white matter on MRI is seen in?

A
Pantothenate kinase deficiency
B
Pelizaeus-merzbacher disease
C
Neuroferritinopathy
D
Aceruloplasminemia
High-Yield Explanation
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.

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