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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

References

REFERENCES

1. Nutt, J. Epidemiology of dystonia in Olmstead county. Presented at Second International Dystonia Symposium 1986; New York.Google Scholar
2. Marsden, CD. Blepharospasm-oromandibular dystonia syndrome (Brueghel’s syndrome). J Neurol Neurosurg Psychiatr 1976; 39:1204–1209.Google Scholar
3. Jankovic, J, Ford, J. Blepharospasm and orofacial-cervical dystonia: clinical and pharmacological findings in 100 patients. Ann Neurol 1983; 13: 402–411.CrossRefGoogle ScholarPubMed
4. Tolosa, E. Clinical features of Meige disease (idiopathic orofacial dystonia): a report of 17 cases. Arch Neurol 1981; 38: 147–151.Google Scholar
5. Gilbert, JG. Brueghel’s syndrome: its distinction from Meige syndrome. Neurology 1996; 46:1767–1769.CrossRefGoogle ScholarPubMed
6. Meige, H. Les convulsions de la face, une forme clinique de convulsion faciale, bilateral et mediane. Rev Neurol 1910; 10: 437–443.Google Scholar
7. Grau, JM, Escartin, A, Kulisevsky, J. Distonias faciales. Revision de una serie de 21 casos. Arch Neurobiol 1986; 49: 201–212.Google Scholar
8. Nutt, JG, Hammerstad, JP. Blepharospasm and oromandibular dystonia (Meige syndrome) in sisters. Ann Neurol 1981; 9: 201–212.CrossRefGoogle ScholarPubMed
9. Tolosa, E, Marti, MJ. Blepharospasm-oromandibular dystonia syndrome (Meige syndrome): Clinical aspects. Adv Neurol 1988; 49: 73–84.Google Scholar
10. Tolosa, E, Lai, C. Meige disease. Striatal dopaminergic preponderance. Neurology 1979; 29: 1126–1130.CrossRefGoogle ScholarPubMed
11. Jankovic, J, Orman, J. Blepharospasm: demographic and clinical survey of 250 patients. Ann Opthalmol 1984; 16: 371–376.Google Scholar
12. Goldstein, JE, Cogan, DG. Apraxia of lid opening. Arch Opthalmol 1965; 73: 155–159.Google Scholar
13. Lepore, FE, Duvoisin, RC. Apraxia of eyelid opening: an involuntary levator inhibition. Neurology 1985; 35: 423–427.CrossRefGoogle ScholarPubMed
14. Cramer, H, Otto, K. Meige syndrome. Clinical findings and therapeutic results in 50 patients. Neuro-opthalmol 1986; 6: 3–15.Google Scholar
15. Sandyk, K. Melatonin secretion and the pathophysiology of Meige disease (idiopathic orofacial dystonia): a hypothesis. Funct Neurol 1990; 5(2):165–170.Google Scholar
16. Kraft, SP, Lang, AE. Cranial dystonia, blepharospasm and hemifacial spasm: clinical features and treatment, including the use of botulinum toxin. CMAJ 1988; 139: 837–844.Google Scholar
17. Jankovic, J. Blepharospasm associated with basal ganglia lesions. Arch Neurol 1986; 43: 866–868.Google Scholar
18. Weiner, WJ, Nausieda, P, Glanz, RH. Meige syndrome after long term neuroleptic therapy. Neurology 1981; 31: 1555–1556.Google Scholar
19. Weiner, WJ, Nausieda, P. Meige syndrome during long-term dopaminergic therapy in Parkinson’s disease. Arch Neurol 1982; 39: 451–452.Google Scholar
20. Zeman, W, Whitlock, CC. Symptomatic dystonia. In: Vinken, PJ, Bruyn, GW, eds. Handbook of Neurology, vol.6. Co. 1968; Amsterdam: North Holland, 544–566.Google Scholar
21. Palakurthy, PR, Iyer, V. Blepharospasm accompanying hypoxic encephalopathy. Mov Disord 1987; 2: 131–135.Google Scholar
22. Keanes, JR, Young, JA. Blepharospasm with bilateral basal ganglia infarction. Arch Neurol 1985; 42: 1206–1208.Google Scholar
23. Demierre, B, Rondot, P. Dystonia caused by putameno-capsulo-caudate vesicular lesions. J Neurol Neurosurg Psychiatr 1983; 46: 404–409.Google Scholar
24. Grimes, J, Hassan, M, Quarrington, A, D’Alton, J. Delayed onset post-hemiplegic dystonia: CT demonstration of basal ganglia pathology. Neurology 1982; 32: 1033–1035.Google Scholar
25. Pettigrew, L, Jankovic, J. Hemidystonia: a report of 22 patients and a review of the literature. J Neurol Neurosurg Psychiatr 1985; 48: 650–657.Google Scholar
26. Perlmutter, JS, Stambuck, MK, Markham, J, et al. Decreased [18F] spiperone binding in putamen in idiopathic focal dystonia. J Neurosci 1997; 17(2): 843–850.Google Scholar
27. Bhatia, K, Marsden, CD. The behavioural and motor consequences of focal lesions of the basal ganglia in man. Brain 1994; 117: 859–876.Google Scholar
28. Krauss, J, Mohadjier, M, Braus, D, et al. Dystonia following head trauma: a report of nine patients and review of the literature. Mov Disor 1992; 7: 263–272.Google Scholar
29. Lee, M, Rinne, J. Dystonia after head trauma. Neurology 1994; 44: 1374–1378.CrossRefGoogle ScholarPubMed
30. Marsden, C, Obeso, J, Zarranz, J, Lang, AE. The anatomical basis of symptomatic hemidystonia. Brain 1985; 108: 463–483.Google Scholar
31. Schneider, S, Feifel, E, Ott, D, et al. Prolonged MRI T2 times of the lentiform nucleus in idiopathic spasmodic torticollis. Neurology 1994; 44: 846–850.CrossRefGoogle ScholarPubMed
32. Molho, ES, Factor, SA. Basal ganglia infarction as a possible cause of cervical dystonia. Mov Disord 1993; 8: 213–216.Google Scholar
33. Berardelli, A, Rothwell, JC, Hallett, M, et al. The pathophysiology of primary dystonia. Brain 1998; 121: 1195–1212.Google Scholar
34. Vitek, JL, Chockkan, V, Zhang, J-Y, et al. Neuronal activity in the basal ganglia in patients with generalized dystonia and hemiballismus. Ann Neurol 1999; 46: 22–35.Google Scholar
35. Jankovic, J, Svendsen, CN, Bird, ED. Brain neurotransmitters in dystonia. N Eng J Med 1987; 316: 278–279.Google Scholar
36. Casey, DE. Pharmacology of blepharospasm-oromandibular dystonia syndrome. Neurology 1980; 30: 690–695.Google Scholar
37. Stahl, SM, Davis, KL, Berger, A. The neuropharmacology of tardive dyskinesia, spontaneous dyskinesias and other dystonias. J Clin Psychopharmacol 1982; 2: 321–328.Google Scholar
38. Tanner, CM, Glantz, RH, Klawans, HL. Meige disease: acute and chronic cholinergic effects. Neurology 1982; 32(7):783–785.Google Scholar
39. Jankovic, J, Patel, SC. Blepharospasm associated with brain stem lesions. Neurology 1985; 33:1237–1240.Google Scholar
40. Powers, JM. Blepharospasm due to unilateral diencephalic infarction. Neurology 1985; 35: 283–284.Google Scholar
41. Sandyk, R, Gillman, MA. Blepharospasm associated with communicating hydrocephalus. Neurology 1984; 34: 1522–1523.Google Scholar
42. Filloux, FM, Adair, J, Narang, N. The temporal evolution of striatal dopamine receptor binding and mRNA expression following hypoxia-ischemia in the neonatal rat. Brain Res Dev Brain Res 1996; 94(1): 81–91.Google Scholar
43. Johnson, M, Hanson, GR, Gibb, JW, et al. Effect of neonatal hypoxiaischemia on nigro-striatal dopamine receptors and on striatal neuropeptide Y, dynorphin A, and substance P concentrations in rats. Dev Brain Res 1994; 83: 109–118.Google Scholar
44. Przedborski, A, Kostic, V, Jackson-Lewis, V, et al. Effect of unilateral perinatal hypoxic-ischemic brain injury in the rat on dopamine D1 and D2 receptors and uptake sites: A quantitative autoradio-graphic study. J Neurochem 1992; 57: 1951–1961.Google Scholar
45. Jordan, DR, Anderson, RL, Digre, KB. Apraxia of lid opening in blepharospasm. Ophthalmic Surg 1990; 21(5):331–334.Google Scholar
46. Nicolelis, MAL, Katz, D, Krupa, DJ. Potential circuit mechanisms underlying concurrent thalamic and cortical plasticity. Rev eurosci 1998; 9: 213–224.Google ScholarPubMed
47. Chase, TN, Oh, JD, Blanchet, PJ. Neostriatal mechanisms in Parkinson’s disease. Neurology 1998; 51(suppl 2): S30–S35.CrossRefGoogle ScholarPubMed
48. O’Hollaren, MT. Masqueraders in clinical allergy: laryngeal dysfunction causing dyspnea. Ann Allerg 1990; 65: 351–356.Google Scholar
49. Altrocchi, PH, Alto, P. Spontaneous oral facial dyskinesia. Arch Neurol 1972; 26: 506–512.Google Scholar
50. Juverra, G, Bettoni, L, Lechi, A. Meige syndrome: a clinical and EMG study. Euro Neurol 1981; 20: 103–109.Google Scholar
51. Kakigi, R, Shibasaki, H, Kuroda, Y, et al. Meige syndrome associated with spasmodic dysphagia. J Neurol Neurosurg Psychiatr 1983; 46(6): 589–590.Google Scholar
52. Tolosa, E, Kulisevsky, J, Fahn, S. Meige syndrome: primary and secondary forms. Adv Neurol 1989; 50: 509.Google Scholar
53. Gerhardt, C. Studien und beobachtungen uber stimmbandlahmung. Virchow’s Arch 1863; 27: 68–99.Google Scholar
54. Hartman, DE, Abbs, JH, Vishwanat, B. Clinical investigations of adductor spastic dysphonia. Ann Otol Rhinol Laryngol 1989; 97: 247.Google Scholar
55. Marion, MH, Klap, P, Perrin, A, Cohen, M. Stridor and focal laryngeal dystonia. Lancet 1992; 339: 457–458.Google Scholar
56. Lagueny, A, Burbaud, P, Le Masson, G, et al. Involvement of respiratory muscles in adult-onset dystonia: a clinical and electrophysiological study. Mov Disord 1995; 10: 708–713.Google Scholar
57. Kang, SV, Burke, RE, Fahn, S. Tardive dystonia. Adv Neurol 1988; 50: 415–429.Google Scholar