[Meige's syndrome or segmental craniocervical dystonia: terminology, history and contemporary view].

The term "Meige's syndrome" or "Breughel's syndrome" is used by specialists for the description of blepharospasm with the involuntary movements in the lower part of the face and/or masseter muscle. These eponyms "Meige's syndrome" and "Breughel's syndrome" make some mess in the terminology. The term "segmental craniocervical dystonia" joins different blepharospam-plus phenotypes and reflects contemporary perceptions about its genetic and pathophysiological community. Botulinotherapy is in fact the only way of symptomathic treatment of craniocervical dystonias.

Oromandibular dystonia: A dental approach

Oromandibular dystonia consists of prolonged spasms of contraction of the muscles of the mouth and jaw. Primaryidiopathic forms and secondary forms exist. Secondary dystonia develops due to environmental factors;some cases of cranial dystonia after dental procedure have been reported, but the causal relationship betweenthese procedures and dystonia remains unclear. Traumatic situations in the mouth, such as poor aligned denturesor multiple teeth extractions may cause an impairment of proprioception of the oral cavity, leading to subsequentdevelopment of dystonia. The clinical characteristics of oromandibular dystonia are classified according to theaffected muscles. The muscles involved may be the muscles of mastication, muscles of facial expression, or themuscles of the tongue. At present, there is no known cure for OMD. The mainstay of treatment for most focaldystonia is botulinum toxin injections. It is important for the dentist to be familiar with oromandibular dystonia,as it can develop after dental treatment and is often misdiagnosed as a dental problem (AU)

No disponible

Oromandibular dystonia: a dental approach.

Oromandibular dystonia consists of prolonged spasms of contraction of the muscles of the mouth and jaw. Primary idiopathic forms and secondary forms exist. Secondary dystonia develops due to environmental factors; some cases of cranial dystonia after dental procedure have been reported, but the causal relationship between these procedures and dystonia remains unclear. Traumatic situations in the mouth, such as poor aligned dentures or multiple teeth extractions may cause an impairment of proprioception of the oral cavity, leading to subsequent development of dystonia. The clinical characteristics of oromandibular dystonia are classified according to the affected muscles. The muscles involved may be the muscles of mastication, muscles of facial expression, or the muscles of the tongue. At present, there is no known cure for OMD. The mainstay of treatment for most focal dystonia is botulinum toxin injections. It is important for the dentist to be familiar with oromandibular dystonia, as it can develop after dental treatment and is often misdiagnosed as a dental problem.

Brueghel syndrome: its distinction from Meige syndrome.

A critical historical evaluation of the cranial dystonias supports the separation of the dystonia of the motor trigeminal nerve producing a widely opened mouth (Brueghel syndrome) from the more common facial dystonias with blepharospasm (Meige syndrome). In a patient with Brueghel syndrome, paroxysmal hyperpnea coincided with dystonic gaping; the finding of upbeating nystagmus suggests pontine localization in the pathogenesis of this rare disorder.

Prevalence of focal dystonias in the western area of Tottori Prefecture in Japan.

We evaluated the prevalence of focal dystonias in the western area of Tottori Prefecture in Japan. The population of the area was 244,935 on October 1, 1992. Because four patients with blepharospasm and three patients with writer's cramp did not visit any hospitals or clinics in 1993 and did not reply to our question letter, we could not confirm their present condition: with or without focal dystonia in 1993. Four patients with facial dystonia including blepharospasm and oromandibular dystonia, seven with spasmodic torticollis, and four with writer's cramp were observed. The prevalence of focal dystonias was 6.12 per 100,000 persons, which may be lower than that in western countries. Although the reasons for this difference are still unclear, a genetic factor may be one implication.