Case-control study of Meige's syndrome. Result of a pilot study

A pilot case-control study was conducted to identify possible risk factors for Meige's syndrome. Patients with Meige's syndrome and age- and sex-matched controls suffering from other neurological diseases were recruited from the Movement Disorders Clinic and Neurology Outpatient Department of the All India Insititute of Medical Sciences. All participants were interviewed and information regarding psychiatric and medical illnesses, use of medications, exposure to fumes, dust and pets, characteristics such as marital status, socio-economic status, alcohol, tea/coffee use, tobacco use, betel nut chewing and family history of neurodegenerative diseases among first-degree relatives was ascertained. We found that betel nut with tobacco chewing was a significant predictor for Meige's syndrome (adjusted odds ratio 7.4, 95% confidence interval = 1.0-59. 82). The role of local irritation or the effect of some chemicals in tobacco and betel nuts needs further evaluation of the pathogenesis of Meige's syndrome.     

Melatonin secretion and the pathophysiology of Meige's disease (idiopathic orofacial dystonia): a hypothesis

Meige's disease is a form of focal dystonia characterized by symmetric dystonic spasms of facial muscles, sometimes associated with dystonic movements of other midline muscle groups. The etiology and pathophysiology of the disease have not been established. There is evidence that Meige's disease may result from striatal dopaminergic preponderance coupled with cholinergic overactivity. Several authors have noted a high prevalence of depression in patients with Meige's disease, suggesting a common neurochemical abnormality. Depression, in turn, is associated with decreased pineal melatonin secretion. We propose, based on studies demonstrating that melatonin regulates dopaminergic, cholinergic and GABA-ergic functions, that alterations in melatonin functions may enhance the development of the disease. This hypothesis may open new avenues toward understanding the pathophysiology of the disease and developing newer therapeutic strategies.     

Treatment of Meige's syndrome with ECT.

A 62-year-old woman with Meige's syndrome failed to respond to several pharmacologic interventions. Her dystonias improved significantly after treatment with bilateral electroconvulsive therapy (ECT). However, the effect was not durable, lasting < or = 72 h. ECT is an effective treatment for many movement disorders including dystonias of differing etiologies. Its efficacy for Meige's syndrome is questionable.     

Complete Remission of Neuroleptic-Induced Meige's Syndrome by Botulinum Toxin Treatment: A Case Report

Abstract: A botulinum A toxin injection has beneficial effects on patients suffering from facial and cervical spastic disorders. However, its effect almost completely disappears within three months. We have reported a case of a 23-year-old schizophrenic patient with severe neuroleptic-induced Meige's syndrome in whom botulinum toxin treatment exerted a marked effect which lasted more than 15 months after the final injection of botulinum toxin in spite of continuous neuroleptic medication. It is concluded that botulinum can be recommended as a treatment of choice in neuroleptic-induced Meige's syndrome.     

Meige's syndrome during long-term neuroleptic treatment

Two patients developed difficulties in eyelid opening following long-term neuroleptic treatment of more than 6-8 years. Tardive dyskinesia and dystonia apart from the face were not found in either case. The symptoms fluctuated in their severities on a daily basis and were easily aggravated by various stimuli, e.g., stress, walking, reading and watching television. Electromyographic studies of their faces clearly indicated that the symptoms resulted from spontaneous blepharospasm and were analogous to idiopathic Meige's syndrome. Therefore, the patients' difficulties in opening their eyes were considered to be the so-called drug-induced Meige's syndrome and/or facial tardive dystonia. It must be stressed that this syndrome is extremely distressing to patients and is a severe complication accompanying a long-term neuroleptic treatment.     

The relationship between eye-winking tics, frequent eye-blinking and blepharospasm.

A family is reported in which three generations were affected with eye-winking tics and/or blepharospasm. The proband developed eye-winking tics in childhood and then developed excessive blinking progressing to blepharospasm by the age of 21 years. His mother presented with Meige's syndrome and spasmodic torticollis at the age of 59 years; his uncle had blinked excessively from his early forties. His eldest son developed an eye-winking tic with facial grimacing at the age of 8 years, and in another son, a self-limiting period of eye-blinking occurred at the age of 4 years. The recovery cycle of the blink reflex was abnormal in all three generations. Three other children with eye-winking tics have a parent or close relative with frequent eye-blinking or blepharospasm. Five patients with adult-onset blepharospasm or Meige's syndrome are also described who had excessive eye-blinking dating back to childhood. It is suggested that eye-winking tics, frequent blinking and blepharospasm may share common pathophysiological mechanisms; the clinical expression may be age-related.     

Meige's syndrome associated with neuroleptic treatment

Meige's syndrome is characterized by blepharospasm and oromandibular dystonia. Three cases are presented; two were associated with long-term neuroleptic administration. This drug-induced syndrome may be a variant of tardive dystonia, and prompt discontinuation of neuroleptic treatment may be therapeutic.     

Meige's Syndrome during Long-Term Neuroleptic Treatment

Abstract: Two patients developed difficulties in eyelid opening following long-term neuroleptic treatment of more than 6–8 years. Tardive dyskinesia and dystonia apart from the face were not found in either case. The symptoms fluctuated in their severities on a daily basis and were easily aggravated by various stimuli, e.g., stress, walking, reading and watching television. Electromyographic studies of their faces clearly indicated that the symptoms resulted from spontaneous blepharospasm and were analogous to idiopathic Meige's syndrome. Therefore, the patients' difficulties in opening their eyes were considered to be the so-called drug-induced Meige's syndrome and/or facial tardive dystonia. It must be stressed that this syndrome is extremely distressing to patients and is a severe complication accompanying a long-term neuroleptic treatment.     

Substantial improvement in a Meige's syndrome patient with levetiracetam treatment.

We report on a woman with idiopathic Meige's syndrome whose dystonia improved with the use of levetiracetam (LEV, Keppra, UCB Pharma, Smyrna, GA). This report and data from an animal model of paroxysmal dystonia suggest that LEV might be helpful in the treatment of dystonia.     

Meige's syndrome: clinical, pharmacological and radiological observations

The clinical pharmacological, and neuroradiological observations in six patients with spontaneous blepharospasm-oromandibular dystonia (Meige's) syndrome are recorded. This group consisted of five males and one female, mean age at onset being 50.3 years. The duration of symptoms ranged from three months to 12 years, three patients having had symptoms for over four years. The dyskinesia was arrhythmic and asymmetrical in the orbicularis oculi and masseter muscles electrophysiologically. Pharmacological studies evinced no consistent response to parenteral physostigmine, no response to oral levodopa and no significant improvement in the dyskinesia following oral haloperidol. Lumbar air encephalogram was done in five patients, and showed frontal cortical atrophy without ventricular dilation in three. It is concluded that Meige's syndrome is a distinct nosological entity, and that physostigmine test is unlikely to be helpful in the differential diagnosis from neuroleptic-induced tardive dyskinesia. Neurotransmitter imbalance in the basal ganglia in this disorder remains to be established, and at present there is no satisfactory drug treatment for this progressively disabling movement disorder.