Pantothenate kinase-associated neurodegeneration - eye of the tiger sign
Diagnosis almost certain
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
{"current_user":null,"step_through_annotations":true,"access":{"can_edit":false,"can_download":true,"can_toggle_annotations":true,"can_feature":false,"can_examine_pipeline_reports":false,"can_pin":false},"extraPropsURL":"/studies/49610/annotated_viewer_json?lang=us"}
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 pantothenate kinase-associated neurodegeneration. 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.
Unable to process the form. Check for errors and try again.