Encephalocoeles (multiple)

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Patient presenting with temporal lobe epilepsy

Patient Data

Age: 45 years
mri

Multiple meningoencephalocoeles are noted:

  • Left temporal lobe into the left sphenoid sinus, containing the uncus and the tip of the left temporal horn. The temporal horn and the entorhinal sulcus are distorted, pulled anteromedially in direction of the herniation, with a small focus of T2 hyperintensity abutting ventricle.
  • Left frontal sinus and anterior enthmoidal air cells (separate bony defects) with the anterior aspect of gyrus rectus within the frontal sinus with distortion and subtle T2 hyperintensity involving the nearby anteromedial frontal cortex.
  • Right frontal sinus and right anterior ethmoid sinus (separate bony defects).

Additionally, there are signs of intracranial hypertension: 

  1. enlarged Meckel's caves bilaterally
  2. partially empty fossa
  3. prominent bilateral optic nerve sheaths 
  4. enlarged CSF space around oculomotor nerves (CNII)

 

ct

Multiple bony defects (left medial sphenoid, bilateral frontal and anterior ethmoid) in the locations of MRI detected meningoencephalocele.

Case Discussion

Idiopathic intracranial hypertension can be clinically occult in the presence of meningocoeles, but become florid when a dural repair is performed. As such a suspicion based on imaging features is crucial. 

In this case this is almost certainly the underlying cause. Not only are there supportive imaging features, but the demographic is also appropriate (overweight middle aged female). 

 

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