Parry-Romberg syndrome with dysphonia

Parry-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.