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Blepharospasm 40 years later.

Giovanni Defazio1, Mark Hallett2, Hyder A Jinnah3

  • 1Department of Basic Medical Sciences, Neurosciences and Sensory Organs, "Aldo Moro", University of Bari, Bari, Italy.

Movement Disorders : Official Journal of the Movement Disorder Society
|February 11, 2017
PubMed
Summary
This summary is machine-generated.

Blepharospasm, a form of adult-onset focal dystonia, presents with diverse motor and nonmotor symptoms, indicating a heterogeneous condition. Research over 40 years suggests a multifactorial cause and a network disorder involving multiple brain regions.

Keywords:
blepharospasmclinical featuresdystoniapathophysiology

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Area of Science:

  • Neurology
  • Movement Disorders

Background:

  • Blepharospasm (BSP) was proposed as adult-onset focal dystonia 40 years ago.
  • Clinical hallmark: prolonged orbicularis oculi muscle spasms.
  • Phenomenologically heterogeneous, with diverse motor and nonmotor symptoms.

Purpose of the Study:

  • Provide a comprehensive overview of blepharospasm findings over the past 40 years.
  • Highlight the heterogeneity of blepharospasm.
  • Discuss current understanding of its etiology, pathophysiology, and treatment.

Main Methods:

  • Comprehensive literature review of studies on blepharospasm over 40 years.
  • Analysis of clinical, neurophysiological, and neuroimaging findings.
  • Synthesis of evidence regarding genetic, epigenetic, and environmental factors.

Main Results:

  • Blepharospasm involves varied periocular muscle activations beyond spasms.
  • Nonmotor manifestations include psychiatric, cognitive, and sensory disturbances.
  • Evidence points to multifactorial etiology and a network disorder involving multiple brain regions.

Conclusions:

  • Blepharospasm is a heterogeneous condition with both motor and nonmotor symptoms.
  • Requires assessment tools that capture both symptom types.
  • Etiology is multifactorial, involving genetic and environmental factors.
  • Considered a network disorder, not solely a basal ganglia disorder.
  • Treatment remains symptomatic.