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Author Notes:

Corresponding author: Prof. Alfredo Berardelli, Department of Neurology and Psychiatry, “Sapienza” University of Rome, Viale dell’Università 30, 00185 Rome, Italy; alfredo.berardelli@uniroma1.it

Relevant conflicts of interests/financial disclosures: Nothing to report.

Subject:

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Clinical Neurology
  • Neurosciences & Neurology
  • dystonia
  • blepharospasm
  • pathophysiology
  • clinical features
  • DEEP BRAIN-STIMULATION
  • BENIGN ESSENTIAL BLEPHAROSPASM
  • OBSESSIVE-COMPULSIVE SYMPTOMS
  • QUALITY-OF-LIFE
  • BILATERAL PALLIDAL STIMULATION
  • INVOLUNTARY EYELID CLOSURE
  • CRANIAL-CERVICAL DYSTONIA
  • IDIOPATHIC FOCAL DYSTONIA
  • ADULT-ONSET BLEPHAROSPASM
  • BOTULINUM TOXIN TREATMENT

Blepharospasm 40 Years Later

Tools:

Journal Title:

Movement Disorders

Volume:

Volume 32, Number 4

Publisher:

, Pages 498-509

Type of Work:

Article | Post-print: After Peer Review

Abstract:

Forty years ago, C.D. Marsden proposed that blepharospasm should be considered a form of adult-onset focal dystonia. In the present paper, we provide a comprehensive overview of the findings regarding blepharospasm reported in the past 40 years. Although prolonged spasms of the orbicularis oculi muscles remain the clinical hallmark of blepharospasm, patients with blepharospasm may be characterized by various types of involuntary activation of periocular muscles. In addition to motor features, blepharospasm patients may also have nonmotor manifestations, including psychiatric, mild cognitive, and sensory disturbances. The various motor and nonmotor symptoms are not present in all patients, suggesting that blepharospasm is phenomenologically a heterogeneous condition. This emphasizes the need for tools for severity assessment that take into account both motor and nonmotor manifestations. The cause of blepharospasm remains elusive, but several lines of evidence indicate that blepharospasm is a multifactorial condition in which one, or several, as yet unknown genes together with epigenetic and environmental factors combine to reach the threshold of the disease. Although blepharospasm was originally believed to be solely a basal ganglia disorder, neurophysiological and neuroimaging evidence point to anatomical and functional involvement of several brain regions. The contribution of multiple areas has led to the hypothesis that blepharospasm should be considered as a network disorder, and this might reflect the varying occurrence of motor and nonmotor manifestations in blepharospasm patients. Despite advances in the aetiology and pathophysiology, treatment remains symptomatic.

Copyright information:

© 2017 International Parkinson and Movement Disorder Society.

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