Presentation
The patient was well till the age of 5 years when he started to develop dysarthria and dystonia.
Patient Data
Symmetric bilateral abnormal low signal on T2-weighted and blooming on GRE WI (due to abnormal accumulation of iron) in the globus pallidus with central high signal (due to gliosis and spongiosis), giving the eye of the tiger sign.
Case Discussion
Pantothenate kinase-associated neurodegeneration (PKAN) is an autosomal recessive disorder causing involuntary spasticity and progressive dementia. It is a subset of neurodegeneration with brain iron accumulation (NBIA).
Classical PKAN tends to have onset before 6 years of age, whereas atypical PKAN manifests at a mean age of 14 years.
The eye of the tiger sign refers to symmetric bilateral abnormal low signal on T2-weighted MRI (due to abnormal accumulation of iron) in the globus pallidus with central high signal (due to gliosis and spongiosis).
The eye of the tiger sign is most classically associated with pantothenate kinase-associated neurodegeneration.
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