Purpose or Case Report: To present a very rare case of a pontine stroke in a 13-year-old boy with NF-2.

Methods & Materials: 13-year-old boy with clinically proven neurofibromatosis type 2 (bilateral acusticus schwannoma, meningeoma of the cavernous sinus, schwannoma of the cauda equina), who developed a left-sided brachium pontis stroke. Clinical, laboratory and imaging findings (MRI, MRA, angiography) are presented and discussed.

Results: The patient was referred for sudden onset of dizziness and vertigo. He presented an ataxic gait pattern and complained in addition about a reduced hearing ability on the left side.
MRI revealed the known bilateral acustic schwannoma and a lesion in the right cavernous sinus presumed to be a meningioma.
In addition a lesion of the left brachium pontis with intense restriction of diffusion in the DWI was seen initially interpreted as a glioma.
Biopsy was performed that showed thickening of the vessel wall of the small vessels indicative of vasculopathy. No tumor cells or signs of inflammation could be observed.
Intense coagulation work-up including protein C, protein S, homocystein, anticardiolipid antibodies were unremarkable. MR-angiography and conventional angiography could not detect any vascular pathology.
Conclusions: Childhood stroke -although rare- is a well known complications of NF-1 but not of NF-2.
Until now there are only 3 case reports about childhood stroke in NF-2. Two of these describe an infratentorial location as in our case.
Our pathologic specimen revealed changes of the vessel wall that could be an expression of a vasculopathy although the imaging findings of the larger vessels do not support this assumption.