'Ears of the Lynx' sign

Case contributed by Prof Alan Coulthard

Presentation

Progressive lower limb spasticity and weakness since childhood. Increased tone upper limbs. Mild cognitive impairment.

Patient Data

Age: Young adult
Gender: Male
MRI

"Ears of the Lynx" sign in SPG11

Axial Flair image shows high signal intensity extending a short distance from the anterior horns of the lateral ventricles, with corresponding low signal intensity on the T1 weighted image. This appearance is the 'Ears of the Lynx' sign, named for the tufts of hair characteristically seen on the ears of these animals. Thinning of the corpus callosum is seen on the midline sagittal T2 weighted image.

Case Discussion

The 'Ears of the Lynx' sign has been described in patients with hereditary spastic paraplegia with thin corpus callosum (HSP-TCC) caused by mutations of the spatacsin vesicle trafficking associated (SPG11) gene, coding spatacsin. The mutation was present in this patient. The sign may also be seen in SPG15, which is caused by mutation in a different gene (zinc finger fyve domain-containing protein 26, ZFYVE26),  encoding spastizin.

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Case information

rID: 72453
Published: 26th Nov 2019
Last edited: 27th Nov 2019
Tag: signs, mri
Inclusion in quiz mode: Excluded

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