Blepharospasm-oromandibular dystonia syndrome (Brueghel's syndrome). A variant of adult-onset torsion dystonia?

AUTOR(ES)

Marsden, C D

RESUMO

Thirty-nine patients with the idiopathic blepharospasm-oromandibular dystonia syndrome are described. All presented in adult life, usually in the sixth decade; women were more commonly affected than men. Thirteen had blepharospasm alone, nine had oromandibular dystonia alone, and 17 had both. Torticollis or dystonic writer's camp preceded the syndrome in two patients. Eight other patients developed toritocollis, dystonic posturing of the arms, or involvement of respiratory muscles. No cause or hereditary basis for the illness were discovered. The evidence to indicate that this syndrome is due to an abnormality of extrapyramidal function, and that it is another example of adult-onset focal dystonia akin to spasmodic torticollis and dystonic writer's cramp, is discussed.

Documentos Relacionados

• Sandhoff disease mimicking adult-onset bulbospinal neuronopathy.
• Multiple adult-onset xanthogranuloma, an uncommon diagnosis
• A longitudinal study of adult-onset asthma incidence among HMO members
• Serum vitamin E concentrations in adult-onset spinocerebellar degeneration.
• Clinical manifestations and treatment of adult-onset asthma and periocular xanthogranuloma