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Parry Romberg's disease with intractable partial epilepsy.

Amit Haldar1, Arabinda Mukherjee

  • 1Department of Neurology, Vivekananda Institute of Medical Sciences and Ramkrishna Mission Seva Pratisthan, Sarat Bose Road, Kolkata, India. docamithaldar@rediffmail.com

Neurology India
|June 15, 2007
PubMed
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Parry Romberg's syndrome, a rare condition causing facial atrophy, can lead to difficult epilepsy. Systemic corticosteroids effectively stopped seizures in a teen, suggesting an autoimmune link and potential steroid responsiveness.

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.

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  • 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.