Right perisylvian syndrome

Case contributed by Ali Alsmair
Diagnosis certain

Presentation

Presented with longstanding epilepsy, not diagnosed yet

Patient Data

Age: 10 years
Gender: Female
mri

Polymicrogyria with thickening involving the right perisylvian cortex (of the right frontal and temporal lobes) associated with blurring of the grey-white matter differentiation and widening of the right sylvian fissure.
Multiple non-specific tiny T2/FLAIR hyperintense foci involving bilateral cerebral white matter, without mass effect or diffusion restriction.
Increase of the AP diameter of the skull, with relative narrowing of the transverse diameter, suggesting scaphocephaly.

Normal cerebellar parenchyma.
Normal ventricular system and subarachnoid spaces.
Bilateral cerebral white matters non-specific T2/FLAIR hyperintense foci.
Normal corpus callosum.
No ICH, space-occupying lesion or shift of midline structures.
Normal brainstem.
Normal arterial and venous flow voids.
No restricted diffusion.
Both cerebellopontine angles are clear.
No tonsillar herniation.
Normal sella.
Both mastoids are clear.

Case Discussion

The perisylvian syndrome is a non-curable condition characterized by polymicrogyria in the perisylvian/perirolandic region, it is commonly bilateral rather than unilateral.

Clinical symptoms include: seizures, pseudobulbar palsy, dysarthria, restriction of tongue movements and delayed milestones 1.

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