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Lingual, Facial, and Oromandibular Spasms
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These special varieties of involuntary movements also 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 6-2B) Other patterns include lateral jaw deviation and bruxism Common terms for this condition are the Meige syndrome, after the French neurologist who gave an early description of it, and the Brueghel syndrome, because of the similarity of the grotesque grimace to that of a subject in a Brueghel painting called De Gaper Dif culty in speaking and swallowing (spastic or 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 have followed the administration of phenothiazine and butyrophenone drugs More often, however, the disorder induced by neuroleptics is somewhat different, consisting of choreoathetotic chewing, lip smacking, and licking movements (tardive orofacial dyskinesia, rabbit-mouth syndrome; see later on and pages 1025 and 1327) 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) Treatment Many drugs have been used in the treatment of these craniocervical spasms, but none has effected a cure Much greater success has been obtained with injections of botulinum toxin into the masseter, temporal, and internal pterygoid muscles This is true also for spasmodic dysphonia (discussed more fully on page 428), which responds favorably to injections of the toxin into the thyroaretynoid vocalis muscles 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 High doses of benztropine may be helpful, but local botulinum toxin injection is probably the best alternative An intermittent spasm that is con ned to one side of the face (hemifacial spasm) is not, strictly speaking, a dystonia and is considered with disorders of the facial nerve on page 1184
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task-speci c dystonias Men and women are equally affected, most often between the ages of 20 and 50 years In the commonest form, writer s cramp, the patient observes, upon attempting to write, that all the muscles of the thumb and ngers either go into spasm or are inhibited by a feeling of stiffness and pain or hampered in some other inexplicable way (similar to what is shown in Fig 6-2C) The spasm may be painful and can spread into the forearm or even the upper arm and shoulder Sometimes the spasm fragments into a tremor that interferes with the execution of uid, cursive movements Immediately upon cessation of writing, the spasm disappears Although the disturbance in writer s cramp is usually limited to the speci c act of writing, it may involve other equally demanding manual tasks At all other times and in the execution of grosser movements, the hand is normal, and there are no other neurologic abnormalities Many patients learn to write in new ways or to use the other hand, though that, too, may become involved A few of our younger patients have developed spasmodic torticollis at a later date The performance of other highly skilled motor acts, such as playing the piano or ngering the violin, may acquire a similar highly task dependent spasm ( musician s cramp ) or in the past, telegrapher s palsy The loss of lip in trombonists and other instrumentalists represents an analogous phenomenon, seen only in experienced musicians In each case a delicate motor skill, perfected by years of practice and performed almost automatically, suddenly comes to require a conscious and labored effort for its execution Discrete movements are impaired by a spreading recruitment of unneeded muscles (intention spasm), a feature common to athetotic states The nature of these disorders is quite obscure They had been classed in the past as occupational neuroses, and a psychiatric causation was suggested repeatedly, but careful clinical analysis has not borne this out Once developed, the disability persists in varying degrees of severity, even after long periods of inactivity of the affected part In monkeys, Byl and colleagues found that sustained, rapid, and repetitive highly stereotypical movements greatly expanded the area of cortical representation of sensory information from the hand These authors hypothesized that degradation of sensory feedback to the motor cortex was responsible for excessive and persistent motor activity, including dystonia Such an enlargement of the area of cortical response to magnetic stimulation has also been found by a number of investigators in patients with writer s cramp Other theories pertaining to the physiology of the focal dystonias have been reviewed by Berardelli et al Treatment A high degree of therapeutic success has been obtained by injections of botulinum toxin into speci cally involved muscles of the hand and forearm (Cohen et al, Rivest et al) and this is now the most widely used form of therapy It has been claimed that the patient can be helped by a deconditioning procedure that delivers an electric shock whenever the spasm occurs or by biofeedback, but these forms of treatment have not been rigorously tested
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