Parry Romberg's disease with intractable partial epilepsy.

Area of Science:

• Neurology
• Dermatology
• Autoimmunology

Background:

• Parry Romberg's syndrome is a rare condition characterized by progressive hemifacial atrophy.
• Neurological complications, particularly epilepsy, are significant sequelae of this syndrome.
• The underlying etiology of Parry Romberg's syndrome remains largely unknown, with limited understanding of its pathogenesis.

Observation:

• A 17-year-old female presented with clinical features consistent with Parry Romberg's syndrome.
• The patient exhibited intractable epilepsy, a common neurological complication associated with the syndrome.
• Seizure activity in this patient was refractory to standard antiepileptic treatments.

Findings:

• Administration of systemic corticosteroids resulted in complete cessation of epileptic seizures.
• This positive therapeutic response suggests a potential autoimmune mechanism underlying the epilepsy in this case.
• The findings align with previous reports indicating a possible autoimmune basis for neurological manifestations in Parry Romberg's syndrome.

Implications:

• Epilepsy associated with Parry Romberg's syndrome may be steroid-responsive in select cases.
• This highlights the importance of considering an autoimmune etiology in patients with Parry Romberg's syndrome and intractable epilepsy.
• Systemic corticosteroids represent a potential therapeutic option for managing epilepsy in this patient cohort, warranting further investigation.