'Ears of the Lynx' sign
Progressive lower limb spasticity and weakness since childhood. Increased tone upper limbs. Mild cognitive impairment.
"Ears of the Lynx" sign in SPG11
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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.
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.
- 1. B. Pascual, S.T. de Bot, M.R. Daniels, M.C. França, C. Toro, M. Riverol, P. Hedera, M.T. Bassi, N. Bresolin, B.P. van de Warrenburg, B. Kremer, J. Nicolai, P. Charles, J. Xu, S. Singh, N.J. Patronas, S.H. Fung, M.D. Gregory, J.C. Masdeu. “Ears of the Lynx” MRI Sign Is Associated with SPG11 and SPG15 Hereditary Spastic Paraplegia. (2019) American Journal of Neuroradiology. 40 (1): 199. doi:10.3174/ajnr.A5935 - Pubmed