Progressive facial hemiatrophy (Parry Romberg syndrome) presenting with partial seizures and migraine headache a rare case report

Abstract Progressive facial hemiatrophy is also known as Parry-Romberg syndrome. It is a rare, slowly progressive, neurocutaneous syndrome of unknown aetiology. It is characterised by unilateral facial atrophy of subcutaneous tissue, skin, muscles and bone. The common neurological complications associated with this disease are migraine and epilepsy. Method Case report A 45 years old female with history of thinning of right side of her face for the past 16 years, migraine type of head ache for the past 8 years and left focal seizure for the past 6 years duration. On examination she had atrophy of right side of her face, involving cheek, buccal and zygomatic areas with facial asymmetry. The skin over this area was not indurated and not adherent to underlying tissues. Her neurological, ophthalmological and dental evaluations were normal. Biochemical parameters were within normal limits. Anti nuclear antibody, Rheumatoid factor and Antidouble stranded DNA were negative. Imaging showed atrophy of soft tissues on right side of her face and bilateral globus pallidus calcification.Discussion and conclusion In our patient the facial hemiatrophy started at about 29 years of age, on the right side of the face, which progressed slowly over a period of 10 years and became static for the past 5-6 years. Most common type of seizure associated with Parry-Romberg syndrome is partial seizure (11percent) as seen in our patient. Migraine may occur in up to 45 percent of these patients. Reported imaging findings were meningeal enhancement, intra cranial calcification and cerebral atrophy. There is no definitive treatment. Symptomatic treatment for symptoms like seizures and reconstructive surgery for facial disfigurement can be considered. While dealing with common neurological symptoms like head ache and seizures, we have to consider such uncommon disorder in appropriate settings.