Facial Myokymias In Parry-Romberg Syndrome

Abstract

A 65-year-old man came to our hospital for an outpatient neurological assessment. He had a 20-year history of hemifacial atrophy associated with hemitongue atrophy ( Figure 1A), intermittent unilateral jaw spasms leading to frequent tongue bites, unilateral alopecia ( Figure 1B), parotid and submandibular gland atrophy, and tooth loss. Despite the extreme discomfort experienced by the patient, he had never received a full evaluation and diagnosis. On neurological assessment, myokymias of the jaw-closing muscles (massetez and temporalis) were detected (video) and confirmed by the presence of myokymic discharges during an electromyographic study. No other neurological signs were recognized. Laboratory and neuroimaging investigations were normal. A diagnosis of Parry-Romberg syndrome was made. This is a rare neurological disorder, mostly sporadic, and characterized by hemifacial atrophy involving the skin, the fat, the connective tissue, and sometimes the bone.

Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

“…Asai and colleagues ( 74 ) presented a case of PHA syndrome associated with contralateral cerebral atrophy. The facial myokymias were also noted in PHA ( 75 ). Seifert and colleagues reported two cases PHA with chronic focal encephalitis ( 76 ).…”