Ears of the lynx sign (brain)
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The ears of the lynx sign refers to abnormal T2/FLAIR cone-shaped hyperintensity at the tip of the frontal horn of the lateral ventricles in the region of forceps minor which resembles the tufts of hair crowning the ears of a lynx.
This sign is seen in hereditary spastic paraplegia with thin corpus callosum (HSP-TCC), the commonest form of which is spastic paraplegia 11 (SPG11), a form of hereditary spastic paraplegia associated with mutations of the identically-named spastic paraparesis gene 11 (SPG11) on chromosome 15 which codes for spatacsin 1,2.
The sign may also be seen in:
- SPG15, another cause of "hereditary spastic paraplegia with thin corpus callosum", which is caused by a mutation in the zinc finger five domain-containing protein 26 (ZFYVE26) gene, encoding spastizin
- Marchiafava-Bignami disease 3
- 1. Riverol M, Samaranch L, Pascual B et al. Forceps Minor Region Signal Abnormality “Ears of the Lynx”: An Early MRI Finding in Spastic Paraparesis with Thin Corpus Callosum and Mutations in the Spatacsin Gene(SPG11)on Chromosome 15. Journal of Neuroimaging. 2009;19(1):52-60. doi:10.1111/j.1552-6569.2008.00327.x
- 2. Pascual B, de Bot S, Daniels M et al. “Ears of the Lynx” MRI Sign Is Associated with SPG11 and SPG15 Hereditary Spastic Paraplegia. AJNR Am J Neuroradiol. 2019;40(1):199-203. doi:10.3174/ajnr.a5935
- 3. Pacheco F, Rego M, do Rego J, da Rocha A. “Ears of the Lynx” Sign in a Marchiafava-Bignami Patient: Structural Basis and Fiber-Tracking DTI Contribution to the Understanding of This Imaging Abnormality. J Neuroimaging. 2012;24(2):205-7. doi:10.1111/j.1552-6569.2012.00714.x