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Published on: September 20, 2018
Blepharospasm associated with multiple system atrophy: a case report and review of the literature
Maki Kagohashi1, Yasuyuki Okuma, Kenji Fujishima
1Department of Neurology, Juntendo University Izu-Nagaoka Hospital, 1129 Nagaoka, Izu-Nagaoka, Tagata, Shizuoka 410-2295, Japan.
Abstract:
Although blepharospasm has been occasionally associated with parkinsonism, it has rarely been reported in patients with multiple system atrophy (MSA). We report a 65-year-old woman with MSA who developed blepharospasm seven years after onset, rendering her functionally blind. Clinical course and the findings of magnetic resonance imaging indicated cerebellar type MSA. The blink reflex studies showed prolonged R2 response and enhanced recovery cycle, indicating an increased excitability of the brainstem interneurons. These results suggest that pathophysiology of blepharospasm in MSA is similar to that of essential blepharospasm. Recognition of blepharospasm in MSA patients is important, as blepharospasm is a treatable feature in this otherwise intractable disorder.
Insights
Blepharospasm, a rare symptom in multiple system atrophy (MSA), can cause functional blindness. This study suggests its pathophysiology in MSA resembles essential blepharospasm and is treatable.
Area of Science:
- Neurology
- Neuroscience
- Ophthalmology
Background:
- Blepharospasm is infrequently associated with parkinsonism and rarely reported in multiple system atrophy (MSA).
- Multiple system atrophy (MSA) is a progressive neurodegenerative disorder.
- Blepharospasm can significantly impair quality of life.
Purpose of the Study:
- To report a case of blepharospasm in a patient with cerebellar type MSA.
- To investigate the pathophysiology of blepharospasm in MSA.
- To highlight the importance of recognizing blepharospasm as a treatable feature in MSA.
Main Methods:
- Case report of a 65-year-old woman with MSA.
- Clinical assessment and magnetic resonance imaging (MRI) for MSA typing.
- Blink reflex studies to assess brainstem interneuron excitability.
Main Results:
- The patient developed blepharospasm seven years after MSA onset, leading to functional blindness.
- MRI confirmed cerebellar type MSA.
- Blink reflex studies revealed prolonged R2 response and enhanced recovery cycle, suggesting increased brainstem interneuron excitability.
Conclusions:
- The pathophysiology of blepharospasm in MSA may be similar to that of essential blepharospasm.
- Blepharospasm is a potentially treatable symptom in patients with MSA.
- Early recognition and management of blepharospasm can improve outcomes for MSA patients.
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