Parry-Romberg syndrome: clinical, electrophysiological and neuroimaging correlations

Abstract

Parry-Romberg syndrome (PRS) is a rare disorder, described in the nineteenth century by Caleb Parry and Moritz Romberg, characterized by acquired and slowly progressive atrophy of one side of the face. The pathogenesis of PRS is still unclear. Immune-mediated processes are thought to be a basic factor in PRS etiology, but autonomic nervous system might also be impaired. A case of PRS in a 26-year-old woman with coexisting disturbances in the lower left limb is presented. The multimodal electrophysiological studies were done, including electroencephalography, visual, brain auditory, somatosensory and trigeminal somatosensory evoked potentials, blink reflex, standard neurographic and electromyographic examinations, quantitative sensory tests and autonomic tests. Neuroimaging studies consisted of brain MR, single voxel proton MR spectroscopy, diffusion tensor imaging with fiber tractography. Based on multimodal electrophysiological and neuroimaging studies, it was concluded that the impairment in PRS is multisystemic, i.e., motor, sensory, and autonomic. A cortical origin of the symptoms is possible.

Fig. 1

M.S., 26 years old. Progressive facial hemiatrophy (PFH) on the right side

Fig. 2

MRI, axial FSE T2-weighted images. a The atrophy of the upper frontal gyrus, paracentral lobule and the medial part of precentral gyrus in the right (R) frontal lobe. b Relative sparing of the lateral part of precentral gyrus and discrete hiperintensity of the white matter in the upper part of the right (R) frontal lobe

Fig. 3

¹H-MR spectroscopy, single voxel method. a Spectrum obtained from a voxel located in the subcortical white matter of involved right frontal lobe revealed decreased level of N-acetylaspartate (NAA) and increased level of choline (Cho) and mioinositol (mI) in comparison to control voxel (intrinsic control) located in the corresponding area in left frontal lobe (b)

Fig. 4

MR Diffusion tensor imaging (DTI). a Fractional anisotropy parametric map shows significantly decreased fractional anisotropy (FA) value (0.476) of the white matter in the right (R) upper frontal gyrus in comparison to corresponding area in the left upper frontal gyrus (0.641). b Fiber tractography shows involvement of the right (R) pyramidal tract (reduction the amount of fibers)