Seminars in Dysphagia 190patterns autosomal recessive, autosomal dominant, and X-linked . . Phenomenology and clinical features of cranio-cervical dystonias . . Blepharospasm "lepharospasm "SP is a form of focal dystonia characterized clinically by involuntary periocular spasms resulting in forceful eye closure [ , , ]. "SP is characterized by tonic, phasic or combined involuntary tonic-phasic contractions of the orbicularis oculi muscles, producing repeated and frequent blinking and persistent forceful closure of the eyelids with various degrees of functional blindness. The characteristic features of "SP include sensory tricks that patients use to relieve their symptoms geste antagoniste and a high frequency of ocular symptoms starting before or at the onset of the spasm [ , , ]. "SP may be associated with inhibition of the levator palpebrae muscle apraxia of eyelid opening or involuntary movements in the lower face or jaw muscles [ , , ]. "praxia of eyelid opening may in turn be associated with other neurological conditions, such as progressive supranuclear palsy and corticobasal degeneration [ , ].
. . Oromandibular dystoniaOMD refers to involuntary spasms of masticatory, lingual, and pharyngeal muscles that result in jaw closing JC , jaw opening JO , jaw deviation JD , or a combination of these abnormal movements [ , ]. When OMD is associated with blepharospasm, the combination is referred to as cranial dystonia or, less appropriately,
. . Lingual dystoniaDystonic involvement of the tongue is a well-recognized feature of tardive dystonia as well as OMD, both primary and secondary, although primary focal lingual dystonia PFLD has only rarely been described. PFLD presents as an action dystonia during speech or in paroxysmal episodic lingual dystonic spasms [ ]. " rare disorder, it can be severe enough to affect speech, swallowing and breathing. Tardive lingual dystonia secondary to dopamine-receptorblocking drugs may manifest as a relatively isolated problem. "lthough severe tongue protrusion, particularly during eating, is characteristic of neuroacanthocytosis, it can also be
. Treatment of craniocervical dystonias with BoNTWhile "oNT treatment remains the treatment of preference for most focal dystonias, pharmacological and neurosurgical treatments are also important in the treatment algorithm. Treatment with "oNT in properly adjusted doses is known to be effective and safe for cranial and cervical dystonia, but not OMD. In recent years, long-term studies on the efficacy and safety of "oNT-" have been published, a new "oNT-" formulation has been marketed and new studies on "oNT-" have been carried out [ ]. Systematic reviews and guidelines recommend that "oNT injections should be offered as a treatment option for CD for which it has been proven to be effective and can be offered for blepharospasm, focal upper extremity dystonia and adductor laryngeal dystonia for which it is probably effective [ , ].The first study on the use of "oNT for CD was a single-blind study with patients and used electromyography...