Eye movement involvement in Parry-Romberg Syndrome: a clinicopathologic case report
A A Zubcov-Iwantscheff1, F Thomke, H H Goebel
1University Eye Hospital, Johann-Wolfgang-Goethe-Universitat Frankfurt/Main, Frankfurt, Germany. zubcov@argus-augen-op.de
Strabismus
|September 13, 2008
Summary
This study details a rare case of Parry-Romberg syndrome (PRS) presenting with a 12-year progressive eye motility disorder. Muscle biopsy revealed an absence of striated muscle in the medial rectus, offering new pathological insights into PRS-related diplopia.
Area of Science:
- Ophthalmology
- Neurology
- Pathology
Background:
- Parry-Romberg syndrome (PRS) is a rare disorder characterized by progressive hemifacial atrophy.
- Diplopia, or double vision, is an uncommon but reported symptom in PRS.
- Previous explanations for diplopia in PRS include enophthalmos, orbital atrophy, nerve dysfunction, and mechanical restrictions.
Observation:
- A 38-year-old woman with PRS experienced a 12-year history of progressive bilateral diplopia.
- Muscle biopsy of the medial rectus muscle was performed.
- The biopsy showed a complete absence of striated muscle tissue.
Findings:
- This is the first documented pathological finding of striated muscle loss in an extraocular muscle in a patient with Parry-Romberg syndrome.
- The absence of muscle tissue provides a direct pathological explanation for the observed eye motility disorder.
Implications:
- This finding may necessitate a re-evaluation of the pathophysiology of ocular motility disorders in PRS.
- Understanding the direct muscle pathology could lead to improved diagnostic approaches and potential therapeutic strategies for PRS patients with diplopia.
- Highlights the importance of detailed pathological examination in rare systemic diseases affecting ocular structures.
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