These special varieties of involuntary movements appear in later adult life, with a peak age of onset in the sixth decade. Women are affected more frequently than men. The most common type is characterized by forceful opening of the jaw, retraction of the lips, spasm of the platysma, and protrusion of the tongue; or the jaw may be clamped shut and the lips may purse (Fig. 4-8B). Other patterns include lateral jaw deviation and bruxism. Common terms for this condition are Meige syndrome, after the French neurologist who gave an early description of it, and Brueghel syndrome, because of the similarity of the grotesque grimace to that of a subject in a Brueghel painting called De Gaper. Difficulty in speaking and swallowing (due in part to spasmodic dysphonia) and blepharospasm are also frequently conjoined, and occasionally patients with these disorders develop torticollis or dystonia of the trunk and limbs. A number have tremor of affected muscles or of the hands as well. All these prolonged, forceful spasms of facial, tongue, and neck muscles had in the past followed the administration of phenothiazine and butyrophenone drugs (tardive dyskinesia). More often, however, the dyskinetic disorder induced by neuroleptics is somewhat different, consisting of choreoathetotic chewing, lip smacking, and licking movements (tardive orofacial dyskinesia, rabbit-mouth syndrome; see later).
Very few cases of the Meige syndrome have been studied neuropathologically. In most of them no lesions were found. In one patient there were foci of neuronal loss in the striatum (Altrocchi and Forno); another patient showed a loss of nerve cells and the presence of Lewy bodies in the substantia nigra and related nuclei (Kulisevsky et al); both are of uncertain significance.
A form of focal dystonia that affects only the jaw muscles has been described (masticatory spasm of Romberg); a similar dystonia may be a component of orofacial and generalized dystonias. In the cases described by Thompson and colleagues, the problem began with brief periods of spasm of the pterygoid or masseter muscle on one side. Early on, the differential diagnosis includes bruxism, hemifacial spasm, the odd rhythmic jaw movements associated with Whipple disease, and tetanus. As the illness progresses, forced opening of the mouth and lateral deviation of the jaw may last for days and adventitious lingual movements may be added. A form that occurs with hemifacial atrophy has been described by Kaufman. An intermittent spasm that is confined to one side of the face (hemifacial spasm) is not, strictly speaking, a dystonia and is considered with disorders of the facial nerve in Chap. 44.
As with the other focal and regional dystonias, substantial success has been obtained with injections of botulinum toxin into the masseter, temporal, and medial pterygoid muscles. High doses of benztropine and related anticholinergic drugs may be helpful, but are not as effective as botulinum toxin treatment. Many other drugs have been used in the treatment of these craniocervical spasms, but none has effected persistent benefit.