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Tuberous sclerosis

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Seizures with adenoma sebaceum

Patient Data

Age: 5 years
Gender: Female
mri

Multiple subependymal nodules of various sizes, the largest one (2 x 1.3 cm) located at the left foramen of Monro. They show an iso-to high signal to the grey matter on T1WI, isosignal on T2WI, and high signal FLAIR with enhancement on postcontrast sequences. Asymmetrical dilatation of the lateral ventricles left larger than right.

Multiple and bilateral cortical and subcortical areas of low signal on T1WI and high signal on T2WI/FLAIR with no enhancement on postcontrast sequences in keeping with cortical/subcortical tubers. Few linear bands of high signal T2WI/FLAIR are seen bilaterally in keeping with radial bands sign.

Case Discussion

Case of tuberous sclerosis (or Bourneville disease) showing at least two major features of the disease according to the tuberous sclerosis diagnostic criteria.

The largest subependymal nodule located at the left foramen of Monro may be considered as a subependymal giant cell astrocytoma, considering its size (>1 cm), marked enhancement following IV contrast, and the obstruction of the ipsilateral foramen of Monro.

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