Dados do Trabalho

Título

CASE REPORT: PARRY-ROMBERG SYNDROME

Apresentação do caso

A 6-year-old male patient with a history of neurodevelopmental delay, autism spectrum disorder and epilepsy. He had facial asymmetry with reduced subcutaneous tissue with erythematous macules on the left hemiface, without involvement of the cranial nerves. Brain MRI showed a global increase in CSF spaces due to a moderate reduction in brain volume, more evident on the left, with a slight diffuse reduction in the thickness of the white matter, associated with a reduction in hippocampal volume. The patient's mother had a similar dermatological alteration, with progressive atrophy in the left hemiface, without etiological definition and had no associated neurologic symptoms.

Discussão

Parry-Romberg Syndrome is a rare disease characterized by progressive facial hemiatrophy, which may be associated with other systemic alterations, mainly neurological. Neurologic symptoms include seizures, cerebral hemiatrophy, trigeminal neuralgia, hemiparesis ipsilateral to the facial lesion. There is still no etiology for this syndrome, but it is believed that it may be related to trauma, immunity and autoimmune disorders. It usually occurs in the first decade of life, with slow progression for 2 to 20 years, and after this period, a period of stabilization is reached. Diagnosis is based on clinical history and physical examination, as well as exclusion of other conditions.

Comentários finais

Early identification is important for intervention and proper management of affected patients in order to reduce the comorbidities that accompany these individuals. Immunosuppressive drugs may be considered in some patients, especially in cases of brain involvement.

Palavras Chave

PARRY-ROMBERG SYNDROME; PROGRESSIVE HEMIFACIAL ATROPHY;

Declaração de conflito de interesses de TODOS os autores

There were no conflicts of interest.