Parry-Romberg progressive facial hemiatrophy: pathogenic and evolutionary considerations with regard to a case followed up for a long period
Introduction. The Parry-Romberg syndrome is a clinical condition in which there is progressive facial hemiatrophy which may be associated with neurological, ocular, cutaneous and auto-immune disorders. It is of unknown aetiology, although many theories have been put forward. Clinical case. We present a case of left facial hemiatrophy associated with ipsilateral lingual hemiatrophy and signs of cortico-cerebellar dysfunction, the neuroimaging findings (NMR) and clinico-radiological follow-up covered a period of over 10 years. Conclusion. The data on the clinical course and neuroimaging, together with our review of the literature, suggest that the Parry-Romberg syndrome may be considered to be a dysgenetic process which may originate during the first stages of CNS embryogenesis.
Caso clínico Presentamos un caso en que se asocia hemiatrofia facial izquierda, hemiatrofia lingual ipsilateral y síntomas de disfunción córticocerebelosa, sus hallazgos de neuroimagen (RMN) y su seguimiento clinicorradiológico durante más de 10 años.
Conclusión Los hallazgos clínicos evolutivos y en neuroimagen, así como la revisión bibliográfica sugieren que el síndrome de ParryRomberg podría ser considerado como un proceso disgenético cuyo origen se podría situar en las primeras etapas de la embriogénesis del SNC
