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 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 spatacsin vesicle trafficking associated (SPG11) gene, codes spatacsin.
The sign may also be seen in:
-
SPG15, another cause of
the hereditary"hereditary spasticparaplegiasparaplegia with thin corpus callosum", which is caused by a mutation in the zinc fingerfyvefive domain-containing protein 26 (ZFYVE26) gene, encoding spastizin. This sign has also been described in -
Marchiafava-Bignami disease 3
.
Differential diagnosis
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ependymitis granularis
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a normalan anatomic variant, commonly seen - can appear similar but is usually less pronounced
-
-<p>The <strong>ears of the lynx sign</strong> refers to abnormal T2/FLAIR cone-shaped hyperintensity at the tip of the frontal horn of the lateral ventricles in the region of <a href="/articles/forceps-minor">forceps minor</a> which resembles the tufts of hair crowning the ears of a lynx.</p><p>This sign is seen in <a href="/articles/hereditary-spastic-paraplegia">hereditary spastic paraplegia</a> with thin <a href="/articles/corpus-callosum">corpus callosum</a> (HSP-TCC), a form of hereditary spastic paraplegia associated with mutations of the spastic paraparesis gene 11 (<em>SPG11</em>) on chromosome 15 <sup>1,2</sup>. The spatacsin vesicle trafficking associated (<em>SPG11</em>) gene, codes spatacsin. The sign may also be seen in SPG15, another of the hereditary spastic paraplegias, which is caused by a mutation in the zinc finger fyve domain-containing protein 26 (<em>ZFYVE26</em>) gene, encoding spastizin. This sign has also been described in <a href="/articles/marchiafava-bignami-disease-1">Marchiafava-Bignami disease</a> <sup>3</sup>.</p><h4>Differential diagnosis</h4><ul><li>- +<p>The <strong>ears of the lynx sign</strong> refers to abnormal T2/FLAIR cone-shaped hyperintensity at the tip of the <a href="/articles/lateral-ventricle-1">frontal horn of the lateral ventricles</a> in the region of <a href="/articles/forceps-minor">forceps minor</a> which resembles the tufts of hair crowning the ears of a lynx.</p><p>This sign is seen in <a href="/articles/hereditary-spastic-paraplegia-with-thin-corpus-callosum">hereditary spastic paraplegia with thin corpus callosum (HSP-TCC)</a>, the commonest form of which is spastic paraplegia 11 (SPG11), a form of <a href="/articles/hereditary-spastic-paraplegia">hereditary spastic paraplegia</a> associated with mutations of the identically-named spastic paraparesis gene 11 (<em>SPG11</em>) on chromosome 15 which codes for spatacsin <sup>1,2</sup>.</p><p>The sign may also be seen in:</p><ul>
- +<li>
- +<a href="/articles/spg15">SPG15</a>, 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 (<em>ZFYVE26</em>) gene, encoding spastizin</li>
- +<li>
- +<a href="/articles/marchiafava-bignami-disease-1">Marchiafava-Bignami disease</a> <sup>3</sup>
- +</li>
- +</ul><h4>Differential diagnosis</h4><ul><li>
-<li>a normal variant, commonly seen</li>-<li>can appear similar but usually less pronounced</li>- +<li>an <a href="/articles/anatomical-variants">anatomic variant</a>, commonly seen</li>
- +<li>can appear similar but is usually less pronounced</li>
Images Changes:
Image 4 MRI (FLAIR) ( create )
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