Encephalocele

Case contributed by Tim Luijkx
Diagnosis almost certain

Presentation

Increasing frequency and severity of dyscognitive/absence seizures from once a fortnight to daily. Severity now generalized tonic clonic, dyscognitive with lips smacking and hand wringing. New seizure disorder.

Patient Data

Age: 14 years
Gender: Male
mri

20 x 18 x 16 mm encephalocele seen in the right greater sphenoidal wing, containing a small portion of dysplastic temporal pole, extending to the base of the pterygoid plates. The foramen rotundum is difficult to see but probably lies medial to the cephalocele. The defect measures 11 x 5 mm.

Suggestion of a subtle encephalocele on the contralateral side, measuring 5 mm in diameter, immediately anteromedial to the foramen ovale. 

Minor protrusion of the right cerebellar tonsil below the foramen magnum is consistent with anatomical variation.

Otherwise structurally normal brain with no migrational abnormality. Normal hippocampi. Normal myelination.

Annotated image

Annotated images showing right temporal encephalocele (image 1 and 3) and possible very small left temporal encephalocele (image 2).

Case Discussion

The MRI shows a right anteromedial temporosphenoidal encephalocele and a possible tiny left anteromedial temporosphenoidal cephalocele on the contralateral side.

This is a frequent location for encephaloceles and is a check area, especially in cases of a suspected epileptogenic focus. They can be very small, as possibly is the case here on the left and should not be confused with the anatomical foramina in the skull base (foramen ovale and foramen rotundum).

Depending on the clinical symptoms encephaloceles may be resected. If they are overlooked unncessary damage from repeated epileptic activity may occur to other structures such as the hippocampi.

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