Central sympathetic dysregulation and immunological abnormalities in a case of progressive facial hemiatrophy (Parry-Romberg disease).
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.
Laboratory of Clinical Electrophysiology, Regional Hospital, Angers, France.
P
P
Lonchampt
J
J
Emile
M C
MC
Pélier-Cady
B
B
Cadou
A
A
Barthelaix
D000293:Adolescent; D001767:Blinking / Q000502:physiology; D001933:Brain Stem / Q000503:physiopathology; D004576:Electromyography; D005071:Evoked Potentials / Q000502:physiology; D005150:Facial Hemiatrophy / Q000276:immunology* / Q000503:physiopathology*; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008297:Male; D009434:Neural Pathways / Q000503:physiopathology; D013564:Sympathetic Nervous System / Q000503:physiopathology*; D014276:Trigeminal Nerve / Q000502:physiology
D007074:Immunoglobulin G