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[Neuroretinitis, Parry-Romberg syndrome, and scleroderma]
A Karim1, M Laghmari, W Ibrahimy
1Service d'Ophtalmologie A, Hôpital des Spécialités, Rabat, Maroc. abdelouahedkarim@hotmail.com
Journal Francais D'Ophtalmologie
|October 27, 2005
Summary
Progressive hemifacial atrophy, also known as Parry-Romberg syndrome, is a rare condition. This case highlights neuroretinitis as an unusual ocular manifestation of this syndrome.
Area of Science:
- Ophthalmology
- Neurology
- Genetics
Background:
- Progressive hemifacial atrophy (Parry-Romberg syndrome) is a rare disorder characterized by progressive atrophy of facial tissues.
- Ocular and neurological symptoms are common, with enophthalmos and eyelid changes being most frequent.
- Neuroretinitis is a rare manifestation of Parry-Romberg syndrome.
Observation:
- A 22-year-old woman presented with a 10-year history of progressive hemifacial atrophy.
- She experienced blurred vision, mild enophthalmos, eyelid atrophy, and loss of cilia.
- Ocular examination revealed bilateral vitreitis and neuroretinitis in the left eye.
Findings:
- Etiological investigations detected antinuclear antibodies and a positive Rose-Waaler latex test.
- The patient exhibited atrophy on the right side of her body.
- Corticosteroid therapy (1 mg/kg/day) was initiated with positive outcomes.
Implications:
- This case underscores the diverse ophthalmologic manifestations of Parry-Romberg syndrome.
- The etiology of Parry-Romberg syndrome remains controversial, with potential links to scleroderma and autonomic nervous system disorders.
- The pathogenesis of acute neuroretinitis in this context requires further investigation.