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Meige Syndrome Secondary to Basal Ganglia Injury: A Potential Cause of Acute Respiratory Distress

Published online by Cambridge University Press:  24 February 2017

C. Adam Kirton  and
Richard J. Riopelle
C. Adam Kirton*
Affiliation:
Division of Neurology, Queen's University, Kingston, ON Canada
Richard J. Riopelle
Affiliation:
Division of Neurology, Queen's University, Kingston, ON Canada
*
c/o Montreal Neurological Institute, 3801 University St. Montreal, Quebec, H3B 2A4
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Abstract:

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Background:

Meige syndrome is a movement disorder that includes blepharospasm and oromandibular dystonias. Its etiology may be idiopathic (primary) or it may arise secondary to focal brain injury. Acute respiratory distress as a feature of such dystonias occurs infrequently. A review of the literature on Meige syndrome and the relationship between dystonias and respiratory compromise is presented.

Methods:

A 60-year-old woman suffered a cerebral anoxic event secondary to manual strangulation. She developed progressive blepharospasm combined with oromandibular and cervical dystonias. Neuroimaging demonstrated bilateral damage localized to the globus pallidus. Years later, she presented to the emergency department in intermittent respiratory distress associated with facial and cervical muscle spasms.

Results:

Increasing frequency and severity of the disorder was noted over years. The acute onset of respiratory involvement required intubation and eventual tracheotomy. A partial therapeutic benefit of tetrabenazine was demonstrated.

Conclusions:

This case highlights two interesting aspects of Meige's syndrome: (1) Focal bilateral basal ganglia lesions appear to be responsible for this patient's movement disorder which is consistent with relative overactivity of the direct pathway from striatum to globus pallidus internal and substantia nigra pars reticularis; (2) Respiratory involvement in a primarily craniofacial dystonia to the point of acute airway compromise.

Résumé:

RÉSUMÉ:Introduction:

Le syndrome de Meige est un désordre du mouvement qui comporte un blépharospasme et de la dystonie oromandibulaire. Son étiologie peut être idiopathique (primaire) ou il peut survenir secondairement à une lésion cérébrale focale. La détresse respiratoire aiguë est une manifestation rare de ce type de dystonie. Nous présentons une revue de la littérature sur le syndrome de Meige et la relation entre les dystonies et l'atteinte de la fonction respiratoire.

Méthodes:

Une femme âgée de 60 ans a présenté un événement cérébral anoxique secondaire à une strangulation manuelle. Elle a développé un blépharospasme progressif associé à de la dystonie oromandibulaire et cervicale. La neuroimagerie a montré un dommage bilatéral au niveau du globus pallidus. Plusieurs années plus tard, elle s'est présentée à l'urgence en détresse respiratoire intermittente, avec des spasmes musculaires faciaux et cervicaux.

Résultats:

Une fréquence et une sévérité accrues de la symptomatologie ont été notées au cours des années. Le début brusque de la symptomatologie respiratoire a nécessité l'intubation et éventuellement la trachéotomie. Un bénéfice thérapeutique partiel a été obtenu avec la tétrabénazine.

Conclusions:

Ce cas illustre deux aspects intéressants du syndrome de Meige: (1) des lésions focales bilatérales du noyau lenticulaire, du noyau caudé, de l'avant-mur ou du noyau amygdalien semblent être responsables du désordre du mouvement chez cette patients, ce qui est en accord avec l'hyperactivité relative de la voie directe provenant du striatum vers le globus pallidus interne et la zone réticulée de la substance noire; (2) l'atteinte respiratoire allant jusqu'à l'obstruction aiguë des voies respiratoires chez un cas de dystonie crâniofaciale.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 28 , Issue 2 , May 2001 , pp. 167 - 173
Copyright
Copyright © The Canadian Journal of Neurological 2000

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