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.

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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