Pantothenate kinase-associated neurodegeneration - eye of the tiger sign
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- updated 8 Sep 2022:
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Pantothenate kinase-associated neurodegeneration (PKAN), historically also known as Hallervorden-Spatz syndrome, 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.
-<p><a href="/articles/pantothenate-kinase-associated-neurodegeneration" title="Pantothenate kinase-associated neurodegeneration (PKAN)">Pantothenate kinase-associated neurodegeneration (PKAN)</a>, historically also known as <strong>Hallervorden-Spatz syndrome</strong>, is an autosomal recessive disorder causing involuntary spasticity and progressive dementia. It is a subset of <a href="/articles/neuroferritinopathy">neurodegeneration with brain iron accumulation (NBIA)</a>.</p><p>Classical PKAN tends to have onset before 6 years of age, whereas atypical PKAN manifests at a mean age of 14 years.</p><p>The <strong>eye of the tiger sign</strong> refers to symmetric bilateral abnormal low signal on T2-weighted MRI (due to abnormal accumulation of iron) in the <a href="/articles/globus-pallidus">globus pallidus</a> with central high signal (due to gliosis and spongiosis).</p><p>The eye of the tiger sign is most classically associated with <a href="/articles/pantothenate-kinase-associated-neurodegeneration">pantothenate kinase-associated neurodegeneration</a>.</p>- +<p><a href="/articles/pantothenate-kinase-associated-neurodegeneration" title="Pantothenate kinase-associated neurodegeneration (PKAN)">Pantothenate kinase-associated neurodegeneration (PKAN)</a>, historically also known as <strong>Hallervorden-Spatz syndrome</strong>, is an autosomal recessive disorder causing involuntary spasticity and progressive dementia. It is a subset of <a href="/articles/neuroferritinopathy">neurodegeneration with brain iron accumulation (NBIA)</a>.</p><p>Classical PKAN tends to have onset before 6 years of age, whereas atypical PKAN manifests at a mean age of 14 years.</p><p>The <a href="/articles/eye-of-the-tiger-sign-globus-pallidus" title="Eye of the tiger sign (globus pallidus)"><strong>eye of the tiger sign</strong></a> refers to symmetric bilateral abnormal low signal on T2-weighted MRI (due to abnormal accumulation of iron) in the <a href="/articles/globus-pallidus">globus pallidus</a> with central high signal (due to gliosis and spongiosis).</p><p>The eye of the tiger sign is most classically associated with <a href="/articles/pantothenate-kinase-associated-neurodegeneration">pantothenate kinase-associated neurodegeneration</a>.</p>