Ophthalmoparesis secondary to stiff-person syndrome - a case report

ABSTRACT Stiff-Person syndrome is a rare neuro-immunological condition predominantly affecting proximal and axial limb muscles, with a higher incidence in females aged between the third and seventh decades of life. Ophthalmological involvement is a significant aspect, often correlated with cerebella...

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Main Authors: Henrique Rabelo de Azeredo, Bruno Silveira Santana, Ricardo Cardoso de Matos, Eric Pinheiro de Andrade
Format: Article
Language:English
Published: Sociedade Brasileira de Oftalmologia 2025-07-01
Series:Revista Brasileira de Oftalmologia
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Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0034-72802025000100511&lng=en&tlng=en
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Summary:ABSTRACT Stiff-Person syndrome is a rare neuro-immunological condition predominantly affecting proximal and axial limb muscles, with a higher incidence in females aged between the third and seventh decades of life. Ophthalmological involvement is a significant aspect, often correlated with cerebellar dysfunction and various nystagmus forms. A 53 year-old female with a history of hypothyroidism and rectal polypectomy, presenting with diplopia, dizziness, and postural instability, eventually diagnosed with Stiff-Person syndrome. Treatment involves medications like diazepam and gabapentin, besides intravenous immunoglobulin therapy and pulse therapy. The patient's ocular manifestations included diplopia, nystagmus, and visual acuity decline. Despite treatment yielding limited ocular improvements, pain reduction and tremor relief were notable. Differential diagnosis excluded other neuromuscular disorders, emphasizing the guarded prognosis of Stiff-Person syndrome, with a negative impact on patients’ quality of life. Recognizing the significance of Stiff-Person syndrome lies not only in its association with common autoimmune diseases but also in its profound impact on ocular health. While early diagnosis and management are crucial to improve the quality of life and allow the patients more independence in their activities, a deeper understanding of its ophthalmological implications is essential for optimizing patient care and outcomes. Further research into the ocular aspects of Stiff-Person syndrome is warranted to enhance our comprehension and therapeutic approaches in this complex condition.
ISSN:1982-8551