Eye Of The Tiger Sign Differential Diagnosis

Neuropathology of the eye of the tiger sign in hallervorden spatz syndrome.

Eye of the tiger sign differential diagnosis. Axonal degeneration with accumulation of spheroidal inclusions can be seen histologically. Crossref medline google scholar. 8 dooling ec schoene w richardson ep. White matter changes are atypical for nbia diseases.

The eye of the tiger sign in cmri is a feature associated with syndromes of neurodegeneration with brain iron accumulation nbia. The eye of the tiger sign refers to abnormal low t2 signal on mri due to abnormal accumulation of iron in the globus pallidus with a longitudinal stripe of high signal due to gliosis and spongiosis. Dev med child neurol 1995. Co poisoning corticobasal ganglionic degeneration.

No prevalence studies have yet been done on this disease or its different forms. 2 the pathognomonic eye of the tiger sign is seen in both forms of the disease. Eye of the tiger sign is seen in hallervorden spatz syndrome also known as pantothenate kinase associated neurodegeneration pkan. Overall nbia is a clinical diagnosis that should be pursued if characteristic abnormalities are seen on mri.

It is low signal intensity rings surrounding the central high signal intensity regions in the medial aspect of bilateral globus pallidus on t2 weighted mri fig. An eye of the tiger sign is a specific magnetic resonance imaging mri pattern a key diagnostic feature of pantothenate kinase associated neurodegeneration pkan. The surrounding hypointensity of the globus pallidus is due to excess iron accumulation. The mri usually shows an area of hyperintensity in the medial globus pallidus that has been called the eye of the tiger sign but this is not pathognomonic.

This is classically described in hallervorden spatz syndrome. The complete differential diagnosis includes. Intellectual impairment rp and optic atrophy are also associated with the disease. 2 late onset pkan presents by 20 years manifesting a progressive picture of dementia parkinsonism dystonia anarthria aphonia and incontinence.

Eye of the tiger sign refers to a low intensity globus pallidus with a central region of high intensity in the anteromedial globus pallidus. The differential diagnosis for progressive dystonia in children includes idiopathic generalized dystonia dopa responsive dystonia myoclonic dystonia metachromatic leukodystrophy niemann pick disease type c and lesch nyhan syndrome 4. This sign describes characteristic low signal surrounding a central region of high signal in the anteromedial globus pallidus on t2. There is no specific treatment.