SUMMARY:We report a case of Parry-Romberg syndrome in a 32-year-old woman presenting with intermittent headache and mild sensory disturbance. MR imaging revealed minimal asymmetric atrophy of the right hemisphere with a few nonspecific white matter hyperintensities. Diffusion tensor imaging and fiber tractography, however, demonstrated clear fiber derangement, especially in the sensory tract of the right cerebral white matter. P arry-Romberg syndrome (PRS) is an acquired progressive facial hemiatrophy of the skin and subcutaneous tissue, and sometimes underlying bony structure, with an occasional involvement of the nervous system [1][2][3][4][5] The atrophic process commonly appears during the first and second decades of life and affects mainly the face below the forehead. 6 The most common neurologic manifestation is epilepsy; however, there are many other neurologic features, such as migraine, facial pain, and structural brain lesions.
1-8We report a case of a patient with PRS presenting with sensory disturbance and subtle white matter changes. We confirmed white matter involvement of PRS by the use of diffusion tensor imaging (DTI) and fiber tractography with 3T MR imaging.
Case ReportA 32-year-old woman presented with intermittent headache, progressive left hemihypoesthesia (lower extremity), and right hemifacial paresthesia for 2 years. The patient recalled that the progressive facial asymmetry started 20 years previously. A physical examination revealed skull depression under the thin skin in the left frontal and right parieto-occipital region, bilateral facial hemiatrophy with alopecia, and bilateral enophthalmos, being more prominent on the left side. On a neurologic examination, the patient showed hyperactive deep tendon reflexes on both knee jerks with a left extensor plantar response, along with a peripheral facial palsy on the right side. Laboratory examinations such as blood counts, renal and hepatic function tests, hypercoagulability screening, and autoantibodies revealed normal findings.Conventional MR images as well as DTIs were obtained by using a 3T MR imaging scanner (Signa HDx; GE Healthcare, Milwaukee, Wis). T2-weighted images and fluid-attenuated inversion recovery (FLAIR) images showed a few tiny hyperintensities in the right frontoparietal periventricular white matter, right globus pallidus, left lateral thalamus, and right upper pons (Fig 1A, -B). Mild asymmetric atrophy of the right midbrain was noted. A 3D volume rendering image from the T1-weighted images depicted bilateral asymmetric scalp and calvarial atrophy (Fig 1C).DTI was performed by using a Stejskal-Tanner sequence with single-shot spin-echo-type echo-planar imaging (TR/TE, 10,000/82 ms; matrix size, 128 ϫ 128; FOV, 240 mm; 66 axial sections; 2.4-mm section thickness). We obtained diffusion-weighted images along 25 different directions with a b-value of 1000 s/mm 2 , as well as an image without diffusion weighting (b ϭ 0 s/mm 2 ). Brain fiber tracking was performed by using software on a workstation (FuncTool; GE Healthcare)....