Pathophysiology of blepharospasm and oromandibular dystonia. 1985

A Berardelli, and J C Rothwell, and B L Day, and C D Marsden

The pathophysiology of reflexes mediated by the fifth and seventh cranial nerves has been studied in 16 patients with blepharospasm and oromandibular dystonia compared with normal age-matched subjects. The EMG activity of the dystonic spasms in the periocular and jaw muscles was similar to that described in other muscles in patients with generalized torsion dystonia. The latency of the R1 and R2 components of the blink reflex and of the corneal reflex was normal. However, the amplitude and the duration of the R1 and R2 and the duration of the corneal reflex were increased. In some patients the R1 component was also present on the side contralateral to the stimulus, while in normal subjects it was present only on the ipsilateral side. The excitability cycle of recovery of the R2 component of the blink reflex after a prior conditioning shock was enhanced in the patients. There were no EEG potentials preceding blepharospasms in the patients, although a Bereitschaftspotential was seen beginning some 500 ms prior to voluntary blinks in the same individuals. Exteroceptive suppression in the contracting masseter and orbicularis oculi muscles was absent in 40 to 50 per cent of the patients. The jaw jerk was present in all the patients with normal latency. These results indicate that the neuronal arcs of the facial reflexes in blepharospasm and oromandibular dystonia are normal. However, there is an abnormal excitatory drive, perhaps from the basal ganglia, to the facial motoneurons and the interneurons which mediate the facial reflexes in the brainstem.

UI MeSH Term Description Entries
D008297 Male Males
D008538 Meige Syndrome A syndrome characterized by orofacial DYSTONIA; including BLEPHAROSPASM; forceful jaw opening; lip retraction; platysma muscle spasm; and tongue protrusion. It primarily affects older adults, with an incidence peak in the seventh decade of life. (From Adams et al., Principles of Neurology, 6th ed, p108) Blepharospasm-Oromandibular Dystonia,Brueghel Syndrome,Idiopathic Orofacial Dyskinesia,Blepharospasm-Oromandibular Dyskinesia,Blepharospasm-Oromandibular Dystonia Syndrome,Blepharospasm-Oromandibular Dystonia Syndrome, Idiopathic,Idiopathic Blepharospasm-Oromandibular Dystonia Syndrome,Syndrome, Blepharospasm-Oromandibular Dystonia,Blepharospasm Oromandibular Dyskinesia,Blepharospasm Oromandibular Dystonia,Blepharospasm Oromandibular Dystonia Syndrome,Blepharospasm Oromandibular Dystonia Syndrome, Idiopathic,Blepharospasm-Oromandibular Dyskinesias,Blepharospasm-Oromandibular Dystonia Syndromes,Blepharospasm-Oromandibular Dystonias,Dyskinesia, Blepharospasm-Oromandibular,Dyskinesia, Idiopathic Orofacial,Dyskinesias, Blepharospasm-Oromandibular,Dyskinesias, Idiopathic Orofacial,Dystonia Syndrome, Blepharospasm-Oromandibular,Dystonia Syndromes, Blepharospasm-Oromandibular,Dystonia, Blepharospasm-Oromandibular,Dystonias, Blepharospasm-Oromandibular,Idiopathic Blepharospasm Oromandibular Dystonia Syndrome,Idiopathic Orofacial Dyskinesias,Orofacial Dyskinesia, Idiopathic,Orofacial Dyskinesias, Idiopathic
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009119 Muscle Contraction A process leading to shortening and/or development of tension in muscle tissue. Muscle contraction occurs by a sliding filament mechanism whereby actin filaments slide inward among the myosin filaments. Inotropism,Muscular Contraction,Contraction, Muscle,Contraction, Muscular,Contractions, Muscle,Contractions, Muscular,Inotropisms,Muscle Contractions,Muscular Contractions
D012018 Reflex An involuntary movement or exercise of function in a part, excited in response to a stimulus applied to the periphery and transmitted to the brain or spinal cord.
D001767 Blinking Brief closing of the eyelids by involuntary normal periodic closing, as a protective measure, or by voluntary action. Orbicularis Oculi Reflex,Reflex, Blink,Reflex, Corneal,Reflex, Orbicularis Oculi,Winking,Blink Reflexes,Corneal Reflexes,Orbicularis Oculi Reflexes,Blink Reflex,Reflexes, Blink,Reflexes, Orbicularis Oculi
D003315 Cornea The transparent anterior portion of the fibrous coat of the eye consisting of five layers: stratified squamous CORNEAL EPITHELIUM; BOWMAN MEMBRANE; CORNEAL STROMA; DESCEMET MEMBRANE; and mesenchymal CORNEAL ENDOTHELIUM. It serves as the first refracting medium of the eye. It is structurally continuous with the SCLERA, avascular, receiving its nourishment by permeation through spaces between the lamellae, and is innervated by the ophthalmic division of the TRIGEMINAL NERVE via the ciliary nerves and those of the surrounding conjunctiva which together form plexuses. (Cline et al., Dictionary of Visual Science, 4th ed) Corneas
D004576 Electromyography Recording of the changes in electric potential of muscle by means of surface or needle electrodes. Electromyogram,Surface Electromyography,Electromyograms,Electromyographies,Electromyographies, Surface,Electromyography, Surface,Surface Electromyographies
D005123 Eye The organ of sight constituting a pair of globular organs made up of a three-layered roughly spherical structure specialized for receiving and responding to light. Eyes
D005260 Female Females

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