Presentation
Patient from another country, who presented with gradual progressive lower limbs weakness and spasticity. The patient was completely normal before 3 years.
Patient Data
Diffuse thinning of the corpus callosum, more pronounced in the anterior half, without any abnormal signal or focal lesions. There is also abnormal T2/FLAIR cone-shaped hyperintensity at the tip of the frontal horn of the lateral ventricles, resembling Ears of the lynx sign. Mild bilateral frontoparietal atrophy is noted, with mild dilatation of the bilateral lateral ventricles. Relatively mildly decreased size of bilateral thalami.
The remainder of the brain is unremarkable. Incidental finding of bilateral maxillary sinuses retention cysts and small Thornwaldt cyst.
There is 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.
Case Discussion
The ears of the lynx sign on FLAIR MR imaging is highly specific for the most common genetic subtypes of hereditary spastic paraplegia with a thin corpus callosum. When this sign is present, there is a high likelihood of a genetic mutation, particularly associated with SPG11 or SPG15, even in the absence of family history.
Unable to process the form. Check for errors and try again.