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PubMed1997

Botulinum A toxin treatment for blepharospasm and Meige syndrome: report of 100 patients.

One hundred patients (9.09 per cent) with blepharospasm from a grand total of 1,100 patients (at the Movement Disorders Clinic at Siriraj Hospital) who had been treated with botulinum A toxin (BTX-A) injection between 1989 and 1996 were analysed. The 100 patients comprised 65 females and 35 males with a female to male ratio of 1.86:1. Their mean age was 53.3 years (S.D. 12.03). Sixty patients had idiopathic blepharospasm, 31 patients were diagnosed with Meige syndrome (blepharospasm plus oromandibular dystonia) and nine patients with segmental dystonia (Meige syndrome plus cervical dystonia). The mean duration of suffering was 39.22 months (S.D. 44.83). Each patient received 30-50 IU of BTX-A injections according to the standard Siriraj injection pattern. Nine patients were lost to follow-up. The results of BTX-A injection were classified as: excellent result (an improvement of more than 75 per cent) in 83.51 per cent; a good response (an improvement of 50-75 per cent) in 13.19 per cent; a moderate response (an improvement of 25-50 per cent) in 2.20 per cent; and minimal or no response in 1.10 per cent. The complications of BTX-A injection were transient minimal ptosis (9.89 per cent), transient double vision (1.10 per cent) and excessive lacrimation (1.10 per cent). The efficacy of BTX-A injection lasted one to two months in 1.10 per cent, two to three months in 23.08 per cent, three to four months in 45.05 per cent, four to five months in 16.48 per cent, five to six months in 4.40 per cent and more than six months in 9.89 per cent. Botulinum A toxin injection is a simple and effective out-patient treatment for patients with blepharospasm, causing no systemic side-effects and minor transient local complications. The only drawback of this treatment is its high cost (100 IU cost 300 US dollars).

Adolescent↗

PubMed1988

The effect of omitting botulinum toxin from the lower eyelid in blepharospasm treatment.

We randomly selected 26 patients with essential blepharospasm to receive either botulinum toxin or saline injection in their lower eyelids to evaluate the necessity of lower eyelid injection to relieve blepharospasm. As diplopia may occur from botulinum toxin injections for blepharospasm, most commonly from injection of the lower eyelid, and surgical relief of blepharospasm is often achieved by excision of only the upper eyelid protractors, omission of toxin from the lower eyelid seemed both desirable and possible. All patients received botulinum toxin in the upper eyelids, above the eyebrows, across the glabella, and near the lateral canthus. Thirteen of 15 patients who received saline in their lower eyelids had relief of spasm, with the same spasm-free interval as those who received toxin. We recommend avoiding injection of toxin in the medial two thirds of the lower eyelid in order to diminish the likelihood of diplopia from inferior oblique muscle paresis.

Blepharitis↗

PubMed2005

Staged deep brain stimulation for refractory craniofacial dystonia with blepharospasm: case report and physiology.

OBJECTIVE AND IMPORTANCE: We report the intraoperative results, subsequent course, and 1-year follow-up evaluation of a patient with medication-refractory craniofacial dystonia for whom we planned bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) implantation but delayed the left GPi DBS implantation because of robust intraoperative effects of right GPi DBS. CLINICAL PRESENTATION: A 47-year-old patient had a 5-year history of progressively severe, bilateral craniofacial dystonia with blepharospasm (Meige's syndrome) that was refractory to medications and to botulinum toxin (A and B) injections. Blepharospasm interfered with his ability to perform his duties as a Special Forces soldier and ended his military career. INTERVENTION: Under stereotactic guidance (magnetic resonance imaging and computed tomographic image fusion, Cosman-Roberts-Wells frame, and University of Florida surgical navigation software) and with detailed microelectrode mapping (four microelectrode passes), a DBS electrode was implanted in the right posteroventral GPi. Microelectrode recordings were taken to document electrophysiological activity of neurons in the region, and intraoperative macrostimulation was performed. The patient was followed up for 6 months with right unilateral GPi DBS, and later a left GPi DBS electrode was placed. CONCLUSION: Although DBS for primary generalized dystonia is commonly performed by simultaneously implanting bilateral GPi electrodes, it may be reasonable in cases of refractory blepharospasm and/or craniofacial dystonia to use a staged procedure for implantation in selected patients. Additionally, the physiology, especially that encountered in the striatum, may help to elucidate the pathophysiological basis for refractory blepharospasm and Meige's syndrome. More cases will be needed to determine the significance of the results reported in this article.

Deep Brain Stimulation↗

PubMed2005

Efficacy and safety of a new Botulinum Toxin Type A free of complexing proteins in the treatment of blepharospasm.

NT 201 is a new development of Botulinum Toxin Type A free of complexing proteins. In this double-blind Phase III trial, we compared the efficacy and safety of NT 201 and BOTOX in patients suffering from blepharospasm. Of 304 enrolled patients, 300 patients received study medication (intent-to-treat population), and 256 patients completed the study as planned (per-protocol population). At baseline, patients received a single injection of NT 201 or BOTOX (<or=35 units per eye). No significant differences were found between NT 201 and BOTOX for all efficacy and safety variables three weeks after injection. Both the NT 201 and the BOTOX group showed a decrease in the Jankovic Rating Scale (JRS) sum score signifying an improvement in the symptoms of blepharospasm during this time period. These data show that NT 201* is an effective and safe treatment for patients suffering from blepharospasm.

Adult↗

PubMed1977

Signs of essential blepharospasm: a motion-picture analysis.

Motion pictures of 15 patients with essential blepharospasm were studied. Previously unrecognized signs indicated multiple cranial nerve involvement. These signs include impersistence of gaze, lid retraction, tongue thrust, head tilts, head jerks, vertical gaze spasms, and asymmetry. The sugns were also observed in a patient with bilateral blepharospasm who had a history of Bell's palsy suggesting facial nerve injury as a possible factor in blepharospasm. The presence of these signs can be explained by known neural pathways, but the site, or sites, of the lesion remains obscure. These signs may be important in assessing severity and in treatment evaluation.

Adult↗

PubMed1987

Blepharospasm accompanying hypoxic encephalopathy.

Severe bilateral blepharospasm with left hemiplegia occurred in a 50-year-old woman, who developed cardiac arrest following ventricular aneurysmectomy. The blepharospasm was present constantly, even during sleep, and increased during attempts at voluntary eye opening. CT scan revealed multiple hypodense areas involving basal ganglia bilaterally and the right perisylvian area. The blepharospasm improved gradually over a 6-month period, although the left hemiplegia remained unchanged.

Basal Ganglia Diseases↗

PubMed1987

Blepharospasm and autoimmune diseases.

We studied two patients, one with systemic lupus erythematosus and the other with myasthenia gravis, both of whom had coexistent blepharospasm. The blepharospasm occurred during exacerbations of the autoimmune diseases and improved with immunosuppressive therapy. These cases illustrate the possible association of blepharospasm and autoimmune disorders.

Adult↗

PubMed1989

Torticollis and blepharospasm in systemic lupus erythematosus.

A case of torticollis and blepharospasm as the major manifestation of neurological involvement in systemic lupus erythematosis (SLE) is presented. A 56-year-old woman presented with blepharospasm in February 1985 and with torticollis in April 1985. She was diagnosed as having SLE in 1966, on the basis of positive LE cells, when she presented with fever and joint pains. There was no evidence that she had ever been on major tranquilizers. An attempt has been made to correlate the level of antibody titres and exacerbation of these movements with response to steroids. The possibility of an association between blepharospasm, torticollis, and autoimmune disorders has recently been considered.

Blepharospasm↗

PubMed1993

Reflex blepharospasm associated with bilateral basal ganglia lesion.

A patient with a bilateral striatal lesion secondary to anoxia presented reflex blepharospasm associated with parkinsonism and dystonia in the limbs. The blink reflex excitability curve was enhanced and the R-2 response prolonged as in patients with essential blepharospasm. The findings in this patient support the notion that blepharospasm may be secondary to basal ganglia dysfunction through abnormal facilitation of reticular formation neurons controlling facial nucleus motoneuron excitability.

Adolescent↗

PubMed1992

The management of blepharospasm and hemifacial spasm.

The aetiology of blepharospasm and hemifacial spasm is different, but both produce involuntary eye closure and facial movements which do not respond to systemic drug treatment. The introduction of therapeutic focal muscle weakening with botulinum toxin injections in the early 1980s appeared to offer great promise in the management of these conditions. In this paper the results of botulinum toxin treatment of 234 patients with blepharospasm and 73 patients with hemifacial spasm over a 7-year period have been analysed. Most patients receive sustained benefit from repeated injections whilst side-effects become less frequent. A clinically recognisable subgroup of patients with blepharospasm respond poorly and may be better treated surgically.

Blepharoptosis↗

PubMed2002

Comparison of preseptal and pretarsal injections of botulinum toxin in the treatment of blepharospasm and hemifacial spasm.

Although the beneficial effect of subcutaneous injections of botulinum toxin type A (BTX-A) is well known in both blepharospasm and hemifacial spasm, the position of the injection sites around the orbicularis oculi may influence the effectiveness and side effects. Here we report results of preseptal and pretarsal BTX-A injections in 53 patients (25 blepharospasm and 28 hemifacial spasm) in whom we used both injection techniques successively. Pretarsal injections were used in 102 out of 186 treatments in blepharospasm group and in 84 out of 202 treatments in hemifacial spasm group. Pretarsal BTX-A treatment produced significantly higher response rate and longer duration of maximum response in both patient groups. This technique was also associated with a lower frequency of major side effects such asptosis. We concluded that injections of BTX-A into the pretarsal, rather than the preseptal portion of the orbicularis oculiis more effective for treatment of involuntary eyelid closure due to contractions of this muscle.

Adult↗

PubMed1984

Biofeedback therapy of essential blepharospasm.

Eight patients with essential blepharospasm were given five consecutive sessions of electromyogram biofeedback from cutaneous electrodes placed over the frontalis muscle. Four of the eight patients demonstrated a 60% or greater reduction in blepharospasm frequency. Posttreatment electromyogram significantly predicted improvement of blepharospasm. These data suggest that electromyogram biofeedback may provide a therapeutic alternative to the treatment of this disabling condition.

Aged↗

PubMed1996

Electrophysiological studies in patients with blepharospasm before and after botulinum toxin A therapy.

Botulinum toxin (BTX) is the treatment of choice for blepharospasm. To investigate if the locally injected BTX influences the central nervous system and in particular the brain stem, amplitude and latency of compound muscle action potential (CMAP) of orbicularis oculi, blink reflex and brain stem auditory evoked potential were studied in 12 blepharospasm patients before BTX therapy, 9 patients after BTX therapy and 9 age and sex matched healthy controls. The mean amplitude of orbicularis oculi in patients was significantly lower before BTX therapy (p < 0.05) and after BTX therapy (p < 0.02). The amplitudes of R1, R2 and contralateral R2 (CR) was higher in patients than controls but did not reach statistical significance. Following BTX therapy there was a reduction in amplitude of R1, R2 and CR (p < 0.001) and prolongation of latency of R2 and CR (p < 0.05). The ratio of blink reflex amplitude/CMAP amplitude of orbicularis oculi showed a significant decline after BTX therapy (p < 0.01). This small study suggests the involvement of brain stem pathways following BTX therapy in patients with blepharospasm.

Action Potentials↗

PubMedDate not supplied

Essential blepharospasm and related dystonias.

Essential blepharospasm is an idiopathic disorder of progressive involuntary spasms of the orbicularis oculi and upper facial (corrugator, procerus) muscles. Blepharospasm literally means spasm of the eyelids; however, most patients with blepharospasm also have or will develop squeezing in the lower face and neck muscles (Meige's syndrome, orofacial dystonia, or oromandibular dystonia). Some patients develop dystonic, uncontrolled movements in areas outside the facial nerve distribution (segmental cranial dystonia or craniocervical dystonia). Chronic, forceful squeezing by the periocular muscles becomes debilitating for the patient and leads to functional and cosmetic eyelid deformities. Treatment has included a variety of modalities and oral medications that are of limited efficacy. Botulinum-A toxin injections have delivered the best temporary relief from this disorder, while the periorbital myectomy operation has been shown to give the best long-term results.

Blepharospasm↗

PubMed2004

A randomized, placebo-controlled, crossover clinical trial of super blue-green algae in patients with essential blepharospasm or Meige syndrome.

PURPOSE: To evaluate the effectiveness of super blue-green algae (SBGA) supplements on the severity of essential blepharospasm treated with botulinum toxin A injections. DESIGN: Double-masked, placebo-controlled, two-period, crossover randomized trial. PATIENTS AND METHODS: The study was carried out in patients with essential blepharospasm or Meige syndrome undergoing routine treatment with botulinum toxin A injections. INTERVENTION: Patients were randomly assigned to either SBGA capsules or placebo. After 6 months of treatment, patients underwent a 6-month washout period with no treatment, then were administered the alternate treatment for an additional 6 months, thus serving as their own controls. MAIN OUTCOME MEASURES: Video documentation of blink rate and involuntary facial movements, time between botulinum toxin A injections, and patients' subjective assessment of the impact of blepharospasm on functioning were obtained at the beginning and end of the first and second (crossover) treatment periods. RESULTS: A total of 24 patients (10 men, 14 women; aged 42 to 83 years) completed both treatment periods. Mean within-patient difference in blink rate between SBGA and placebo periods was -2.1 blinks per 2 minutes (95% confidence interval [CI]: -20.8-+31.9), not statistically different from zero (P =.83). Mean within-patient difference in time between injections between SBGA and placebo periods was 4.6 days (95% CI: -13.3-+22.5), not statistically different from zero (P =.62). The lack of statistical significance may have been because of small sample size. There were no significant differences in severity of involuntary movement between SBGA and placebo treatment periods. However, patients were more likely to report limitation in function during the period they took SBGA than during the period they took placebo (odds ratio, 0.2; P =.03). CONCLUSION: Overall, we found no evidence of a beneficial effect of SBGA as an adjunct to botulinum toxin A injections; however, a few patients, all younger than 60 years, did appear to benefit from SBGA.

Adult↗

PubMed2006

Supramaximal doses of botulinum toxin for refractory blepharospasm.

PURPOSE: To investigate the response to supramaximal doses of botulinum toxin in patients with refractory blepharospasm. DESIGN: Prospective, nonrandomized, open-label interventional case series. PARTICIPANTS: Eight consecutive patients with blepharospasm requiring injections every 2 months despite receiving 100 U of botulinum toxin per session. INTERVENTION: Increasing the dose of botulinum toxin per session above the conventional maximum. MAIN OUTCOME MEASURES: Duration of treatment effect and patients' subjective response to treatment. RESULTS: Supramaximal dosages were well tolerated. Seven of 8 patients had a prolonged interval between injections relative to that associated with their previous dosing regimen. Four of the patients elected to continue with the new dosage. CONCLUSION: In select patients with essential blepharospasm who are refractory to standard treatment regimens, increasing the dosage of botulinum toxin above 100 U per session may decrease the interval between injections, improve the patient's quality of life, or both.

Aged↗

PubMed1998

Spontaneous remission in patients with essential blepharospasm and Meige syndrome.

PURPOSE: To determine the frequency of remission in patients with essential blepharospasm and Meige syndrome. METHOD: Retrospective analysis of patients by means of questionnaire in an institutional setting. RESULTS: Among 238 patients diagnosed in a single institution as having essential blepharospasm or Meige syndrome, 27 (11.3%) claimed to be symptom free without curative surgery. The average time between onset and resolution in the patients who claimed remission was 4.85 years (range, 3 months to 22 years). Among these patients, the duration of remission averaged 6.33 years (range, 1 year to 14 years). CONCLUSION: Patients with essential blepharospasm or Meige syndrome have a small but definite potential for spontaneous remission of symptoms, particularly within the first 5 years after onset of symptoms.

Blepharospasm↗

PubMed2000

Psychiatric disorders in patients with blepharospasm - a reactive pattern?

OBJECTIVE: The purpose of this study was to assess the life-time prevalence of all major psychiatric disorders in patients suffering from blepharospasm. METHOD: A total of 31 consecutive patients with blepharospasm attending the Department of Neurology were interviewed at the Department of Psychiatry at the University of Vienna. Patients had been submitted to standard neurological diagnostic procedures, psychiatric diagnoses were made with the help of the SCID, functional impairment was assessed by the General Assessment of Functioning Scale (GAF). RESULTS: A current or life-time psychiatric diagnosis was made for 22 patients (71%). The most frequent disorders were depressive disorders, mainly major depression (five patients, 16.1%), secondary dysthymia (six patients, 19.3%), and recurrent major depression (five patients, 16.1%). A diagnosis of simple phobia was made for seven patients (22.5%), for obsessive-compulsive disorder in three patients (9.6%). The mean GAF score of our sample was 63.1%. CONCLUSION: In contrast to previously published results, we did not find a high rate of a single specific disorder or patterns in our study sample, though by the inclusion of life-time diagnostic criteria, the majority of patients fulfilled criteria for at least one diagnosis. This might indicate the considerable negative impact of blepharospasm on the patients' lives.

Aged↗