Parry-Romberg syndrome (PRS), also known as idiopathic progressive hemifacial atrophy, is a rare neurocutaneous disease characterized by the loss of skin and subcutaneous fat from the face, muscles, and bones causing unilateral atrophy. Most patients only require soft tissue augmentation, although the syndrome has varying degrees of severity [1]. Facial hemitrophy progresses slowly and often affects one side of the face. Its pathophysiology, according to some authors, may be related to an inflammatory meningoencephalic with vasculitis, or a chronic vasomotor disorder. Ophthalmic involvement is common, with progressive enophthalmia being a frequent finding [2].

Treatment is usually esthetic using different procedures for correcting facial atrophy of cosmetic dermatological surgery [3].

A 49-year-old woman sought neurological care due to epileptic seizures that started 21 years ago. The crises described were mostly focal, with compromised consciousness, symptomatology related to the temporal lobe with automatisms and sensory hallucinations. In addition, she also had occasional episodes of generalized seizures that occurred during sleep. Physical examination: right hemifacial hypotrophy with hemilingual atrophy and right enophthalmos were observed (Figure 1). No other significant neurological deficits were found. She reported a family history of epilepsy. On examinations: polysomnography showed increased airway resistance. Magnetic resonance imaging of the brain showed: volume reduction in the right cerebral hemisphere (Figure 2). Electroencephalogram showed marked signs of right temporal cortico-subcortical dysfunction with 2–4 Hz waves, slow wave bursts (Figure 3). Polysomnography was requested due to complaints of sleep problems, and showed mild sleep apnea-hypopnea syndrome, with an apnea-hypopnea index of 10.9/h and 124 micro-arousals and good response to continuous positive airway pressure (CPAP) with a pressure of 7 cm H₂O. She was referred for otorhinolaryngological evaluation. Performed computerized tomography (CT) of the paranasal sinuses that showed mucous thickening in the left maxillary sinus and nasal septum deviation. Despite the treatment with divalproate sodium 500 mg 12/12 h, and phenobarbital 100 mg at night, as prescribed by the neurologist who was monitoring her, lacosamide 100 mg 12/12 h was added to the therapeutic regimen with an important reduction in epileptic events that started to occur sporadically, especially when there was therapeutic failure.

Parry-Romberg syndrome is described as an uncommon disorder, whose neurological symptoms include headache, trigeminal neuralgia, and focal epilepsy, more common in females, usually manifested in the first decade of life [4],[5]. Computed tomography and MRI are excellent methods for diagnosing CNS alterations, and the characteristic findings are: white matter hypersignal on T2 and FLAIR, leptomeningeal enhancement, intracranial calcifications, and cerebral atrophy [6],[7],[8].

Differential diagnosis includes diseases that present with cerebral hemiatrophy, such as Rasmussen encephalitis and Sturge-Weber syndrome, but in these we do not observe the typical hemifacial alterations like in Parry-Romberg syndrome [5]. Advanced MRI sequences are currently little explored in Parry-Romberg syndrome and may help explain its pathophysiology.

The cause of hemilateral cutaneous atrophy in the face of a patient with Parry-Romberg syndrome does not yet have a known etiopathogenesis [9],[10],[11]. It is known that immunological disorders are involved in the pathophysiology of the disease [12]. During the active phase of progressive atrophy, there are some medications that help to control and stabilize other symptoms such as headache, trigeminal neuralgia, and focal epilepsy. In the case reported here, the patient had altered brain activity in the right temporal cortico-subcortical region with 2–4 Hz waves, slow wave bursts, after performing an electroencephalogram [13],[14].

The presented case corroborates the syndromic diagnosis of Parry-Romberg syndrome. Complementary exams such as MRI and cranial CT ruled out other neurological conditions that could justify the progressive clinical presentation. The description and scientific documentation of case reports that explain how the diagnosis is made, as well as the clinical management of the disease, is relevant.