Hallervorden Spatz syndrome - eye of the tiger sign

Case contributed by Dr Abdallah Al Khateeb

Presentation

Recurrent falls and progressive spasticity. Dysarthria for the last three years. Febrile seizures at age of 10 years. Her brother had similar symptoms and imaging findings.

Patient Data

Age: 20 years
Gender: Female

Symmetrical bilateral low T2 signal intensity surrounding a central high T2 signal intensity region in the medial aspect of the globus pallidus, which is described as eye of the tiger sign.

Case Discussion

The brain MRI findings, the patient's history and physical examination findings, along with the similar family history allowed a suggestion of Hallervorden Spatz syndrome. However, the eye of the tiger sign is suggestive but not pathognomonic of this entity. 

A trial of carbidopa did not show noticeable improvement of her spasticity. 

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Case information

rID: 45520
Published: 6th Jun 2016
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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