J Korean Child Neurol Soc.  2003 May;11(1):144-149.

A Case of Parry-Romberg Syndrome with Intractable Seizure

• Heo YJ
• Kim SW
• Song DW
• Chung HJ

Abstract

Parry-Romberg syndrome, first described in 1825 by Parry and in 1846 by Romberg, is a rare disorder characterized by a progressive hemifacial atrophy of the skin and adipose tissue and atrophy of muscle, cartilage, and underlying bony structures. It is sometimes accompanied with such complications as ophthalmologic abnormality, localized alopecia and neurologic symptoms, for example, contralateral Jacksonian epilepsy, trigeminal neuralgia, migrane and hemiplegia. The onset is slow and progressive, starting at the first or second decade of life and lasting for 2-10 years, ending with a face being "burned out". It is often associated with epilepsy but the link between these two conditions is poorly understood. In patients with progressive hemifacial atrophy, a high incidence of abnormal neuroradiologic findings in the brain has been reported. Brain MRI findings include cerebral hemiatrophy, cortical calcification, unilateral focal infarction in the corpus callosum, diffuse deep and subcortical white matter signal changes and mild cortical thickening. We report a case of Parry-Romberg syndrome in a 5-year-old boy who had a progressive Rt. facial hemiatrophy with intractable epilepsy and basal ganglia calcification from brain MRI.

Keyword

Progressive hemifacial atrophy; Parry-Romberg syndrome; Intractable seizure

MeSH Terms

Adipose Tissue
Alopecia
Atrophy
Basal Ganglia
Brain
Cartilage
Child, Preschool
Corpus Callosum
Epilepsy
Facial Hemiatrophy*
Hemiplegia
Humans
Incidence
Infarction
Magnetic Resonance Imaging
Male
Neurologic Manifestations
Seizures*
Skin
Trigeminal Neuralgia