Parry-Romberg syndrome with dysphonia - 30/05/08
MA Rafai,
FZ Boulaajal,
B El Moutawakil,
M Bourezgui,
M Sibai,
M Mahtar,
M Chekkouri,
I Slassi
Voir les affiliationsParry-Romberg syndrome (PRS) is a rare clinical entity consisting of progressive hemifacial atrophy that resembles a sabre cut. It is more frequent in women and classically begins at a young age. The aetiology is poorly understood. A number of associations have been reported, particularly seizures. An associated dysphonia has not previously been reported in the literature.
Case report: A 29 year-old man, with an history of familial conflict, progressively developed a left sided PRS with dysphonia over 4 years. There was atrophy of the ipsilateral vocal cord; otherwise neurological examination was normal, as was the brain CT scan.
Different mechanisms have been proposed: an immunological disorder, a consequence of sympathetic dysfunction or of infection. Management relies upon reconstructive surgery and the symptomatic treatment of possible psychological or behavioural disorders.
13 references.
Plan
© 2007 Elsevier Masson SAS. Tous droits réservés.
Vol 163 - N° 12
P. 1246-1248 - décembre 2007 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.