Abstract
A case of hemifacial atrophy (Parry—Romberg disease) is discussed. Electrophysiological and immunological studies were performed. Electromyography, blink reflex and trigeminal evoked potential abnormalities indicate that the brain stem may be implicated in the aetiology of the disease. Immunological evidence favoured this possibility and demonstrated possible involvement of the noradrenergic system. Hyperactivity of the brain stem sympathetic centres, possibly caused by an autoimmune process, may be the primary cause of the cutaneous and subcutaneous atrophy in Parry—Romberg disease.
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Archambault L, Fromm NK. Progressive facial hemiatrophy.Arch Neurol Psychiatr 1932;27: 529–584.
Wartenberg. Progressive facial hemiatrophy.Arch Neurol Psychiat 1945;54: 75–96.
Johnson RV, Kennedy WR. Progressive facial hemiatrophy (Parry-Romberg Syndrome).Am J Ophtalmol 1969;67: 561–564.
Lakhani PK, David TJ. Progressive hemifacial atrophy with scleroderma and ipsilateral limb wasting (Parry—Romberg Syndrome).J R Soc Med 1984;77: 138–139.
Stohr M, Petruch F, Scheglmann K. Somatosensory evoked potentials following trigeminal nerve stimulation in trigeminal neuralgia.Ann Neurol 1981;9: 63–66.
Garrel S, Barret L, Maynard R. Potentiels évoqués somesthésiques du trijumeau chez I'homme — Premiers résultats.Rev EEG Neurophysiol 1982;12: 129–134.
Ongerboer de Visser BW. Comparative study of corneal and blink reflex latencies in patients with segmental or with cerebral lesions. In: Desmet JE, ed.Advances in Neurology, Vol. 39,Motor Control Mechanisms in Health an Disease. New York: Raven Press, 1983; 757–772.
Kimura J. Effect of hemispheral lesions on the contralateral blink reflex.Neurology 1974;24: 168.
Shichida K, Tomita Y, Takashima S, Takeshita K. Blink reflex in children with neurological disorders: analysis of ipsilateral early component R1 and late component R2.Brain Dev 1988;10: 289–294.
Leandri M, Parodi CI, Favale E. Normative data on scalp responses evoked by infra-orbital nerve stimulation.Electroencephalogr Clin Neurophysiol 1988;71: 415–421.
Lindvall O, Bjorklund A. Organization of catecho-lamine neurons in the rat central nervous system. In: Iversen SD, Iversen SH, Nyper S, eds.Handbook of Psychopharmacology. New York: Plenum Press, 1978;9: 139–231.
Loewy AD, Mackeller S, Saper CB. Direct projection from the A5 catecholamine cell group to the intermedio-lateral cell column.Brain Res 1979;174: 309–314.
Westlund KN, Coulter JD. Descending projections of the locus coeruleus and sub coeruleus-medial parabrachial nuclei in monkey: axonal transport studies and dopamine beta hydhoxylase immunocytochemistry.Brain Res Rev 1980;2: 235–264.
Jones BE, Moore RY. Ascending projections of the locus coeruleus in the rat. II. Autoradiographic study.Brain Res 1977;127: 23–53.
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Lonchampt, P., Emile, J., Pélier-Cady, M.C. et al. Central sympathetic dysregulation and immunological abnormalities in a case of progressive facial hemiatrophy (Parry-Romberg disease). Clinical Autonomic Research 5, 199–204 (1995). https://doi.org/10.1007/BF01824007
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DOI: https://doi.org/10.1007/BF01824007