25-year-old woman with Turner syndrome and intellectual impairment. Past diagnosis of epilepsy; admitted to hospital for diagnostic video-EEG monitoring for episodes initially thought to be non-epileptic.
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Standard T1 and T2 weighted scans were supplemented by 3-D FLAIR and 3-D T1 weighted acquisitions. Lateral, third and fourth ventricles are mildly enlarged and cerebral cortical sulci are generally wider than normal. The cerebellum also shows evidence of at least mild atrophy. The medial aspects of both temporal lobes are markedly dysplastic. The hippocampal formations posteriorly appear within normal limits, but body and pes are unrecognizable, due to lack of normal folding and lack of normal internal structure. Relative thinness of the corpus callosum reflects generalised tissue loss.
Conclusion: Bilateral dysplastic temporal lobes. Mild to moderate generalised atrophy. Moderate cerebellar atrophy.
The MRI shows a significant degree of volume loss. The hippocampi are quite dysmorphic. There are fetal posterior cerebral arteries. The is quite a bit of cerebellar atrophy.
The patient was found to have multiple episodes of atonic seizures in hospital, and had a variety of electrographic abnormalities, thought represent Lennox-Gastaut syndrome (LGS).