title: 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.
authors: Department of Neurology, Wrocław Medical University, Borowska 213, 50-556 Wrocław, Poland. s.budrewicz@wp.pl
authors: Sławomir
authors: S
authors: Budrewicz
authors: Magdalena
authors: M
authors: Koszewicz
authors: Ewa
authors: E
authors: Koziorowska-Gawron
authors: Paweł
authors: P
authors: Szewczyk
authors: Ryszard
authors: R
authors: Podemski
authors: Krzysztof
authors: K
authors: Słotwiński
mesh_terms: D000328:Adult; D001224:Aspartic Acid / Q000031:analogs & derivatives / Q000378:metabolism; D001921:Brain / Q000378:metabolism / Q000473:pathology; D002794:Choline / Q000378:metabolism; D004569:Electroencephalography; D004576:Electromyography; D005150:Facial Hemiatrophy / Q000473:pathology* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007294:Inositol / Q000378:metabolism; D059906:Neuroimaging; D012684:Sensory Thresholds / Q000502:physiology; D013223:Statistics as Topic; D014316:Tritium
chemical_list: D014316:Tritium; D001224:Aspartic Acid; D007294:Inositol; C000179:N-acetylaspartate; D002794:Choline