Giant intracranial aneurysm in a ten-year-old boy with parry romberg syndrome. A case report and literature review.

SUMMARY: Parry-Romberg syndrome (PRS) is a rare acquired syndrome consisting of progressive hemiatrophy of the face.We present a child with PRS and progressive neurological deficit caused by a giant intracranial aneurysm and reviewed the literature concerning all intracranial abnormalities in patients with PRS.A literature search identified 27 articles reporting on 88 patients ith PRS and intracranial abnormalities. Ipsilateral brain calcification and hemiatrophy are the most prominent features on CT scan and hyperintense white matter lesions are most frequently seen on T2-weighted MRI. Although lacking precise prevalence data, intracranial abnormalities are not uncommon in patients with PRS. We found three other PRS patients with intracranial aneurysms. Our case and literature search suggests a possible association between PRS and intracranial aneurysms. We consider this association important for clinical practice and recommend including intracranial vascular diseases in the differential diagnosis when dealing with a PRS patient with neurological symptoms.