Brueghel’s syndrome

  Brueghel’s syndrome is also known as: (1) bilateral blepharospasm syndrome; (2) blepharospasm-mouth and jaw dystonia syndrome; (3) bean dystonia syndrome.  (2) Outline: Brueghel’s syndrome is a rare idiopathic spasticity of the eyelids and mouth and jaw in adults. It is characterized by progressively stronger contractions of all facial, lingual and cervical muscles in intermittent episodes, which can be enhanced by agitation and finally become continuous. Adult onset, often around 60 years of age, is more common in women.  Etiology: The cause is unknown and there is no genetic factor. It may be a manifestation of extrapyramidal abnormalities or dystonia of the nucleus pulposus, and may occasionally be caused by psychiatric factors.  Ocular features: idiopathic blepharospasm. Long-term episodes may result in mild ptosis.  V. Systemic features: 1. Oral and mandibular dystonia. At the onset, all facial muscles, tongue muscles and neck muscles of the patient gradually strengthen contraction, initially intermittent episodes, may increase due to emotional excitement, and finally a continuous state.  2. Slanting neck, arm movement disorder.  VI. Treatment: Avoid mental stimulation and emotional excitement, and give sedatives during seizures. Partial excision of the upper eyelid skin is feasible in cases of laxity.