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PubMed2001

Quality of life in patients with blepharospasm.

OBJECTIVES: Administration of botulinum neurotoxin A (BONT/A) is a common and effective treatment of blepharospasm. There is, however, no information regarding the emotional and social well-being of patients with blepharospasm and patient acceptance of BONT/A therapy. The purpose of this study was to investigate aspects of quality of life of patients with blepharospasm and level of patient satisfaction with treatment. MATERIAL AND METHODS: Fifty-one patients with blepharospasm who had been treated with BONT/A for years completed a questionnaire providing information about quality of life. RESULTS: Results revealed reductions in social and emotional well-being of patients but, nonetheless, good acceptance of BONT/A therapy. The positive effects of BONT/A therapy were, however, accompanied by fear of a decreasing effect of BONT/A injections. CONCLUSION: Although the objective findings following BONT/A injections in the treatment of blepharospasm are appreciated by the patients, their well-being is affected by fears and depression.

Aged↗
PubMed1999

Positron emission tomography in patients with benign essential blepharospasm.

PURPOSE: To identify possible abnormalities in regional cerebral glucose metabolism in patients with benign essential blepharospasm or Meige syndrome using positron emission tomography. METHODS: Ten patients with benign essential blepharospasm and one patient with Meige syndrome were examined using positron emission tomography with deoxyglucose labeled with fluorine 18. The severity of muscle spasm at the time of positron emission tomography was recorded in each patient. In five patients, positron tomography was performed on two occasions (once before and again 1 to 2 weeks after botulinum injection) to look for reproducibility of the findings on positron emission tomography also to see if there was any correlation between the severity of symptoms and positron emission tomography findings. RESULTS: The mean reference ratio for fluorodeoxyglucose F18 metabolism was significantly elevated in the striatum compared with the frontal, temporal, or parietal regions. Glucose metabolism was also elevated in the thalami. There was no correlation between the severity of blepharospasm and the degree of hyperactivity in the striatum. In the patients who underwent positron emission tomography scanning on two occasions, there was no significant difference between the two studies in any of the regions analyzed. CONCLUSIONS: The authors' findings support the hypothesis that benign essential blepharospasm and Meige syndrome may be associated with overactivity of the striatum and the thalamus. Positron emission tomography may prove to be a useful research tool and a possible adjunct diagnostic technique for benign essential blepharospasm and Meige syndrome.

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PubMed1992

Blepharospasm and obsessive-compulsive disorder.

Although essential blepharospasm is considered to be a form of focal dystonia, many patients with blepharospasm have been noted to have concomitant depression, anxiety, phobias, hypochondriasis, and other emotional and behavioral disorders, suggesting a psychiatric component to the disease that is phenomenologically similar to obsessive-compulsive disorder (OCD) in terms of the repetitive, perseverative, and persistent nature of the symptoms. The Maudsley OCD questionnaire was administered to 21 patients with blepharospasm and 19 normal controls. The blepharospasm patients scored significantly higher than the controls (p less than .01). Although preliminary, the current study does support at least a phenomenological link between OCD and blepharospasm.

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PubMed1994

Efficacy and side effects of botulinum toxin treatment for blepharospasm and hemifacial spasm.

PURPOSE: To analyse the effectiveness and rate of side effects of botulinum toxin treatment for blepharospasm and hemifacial spasm. METHODS: In a prospective trial, 81 patients with blepharospasm and 70 with hemifacial spasm were treated with botulinum toxin A in the neuroophthalmology clinic at St Vincent's Hospital, Melbourne. Some 989 treatments were given and the mean follow-up time was 28.7 months. RESULTS: The duration of action was longer for patients with hemifacial spasm than for those with blepharospasm (median 12.0 weeks compared with 7.0 weeks, P < 0.0001). There was no change in the duration of effect over time with repeated treatments of the same dose (F = 0.4, P > 0.05). Once an effective dosage was reached, increasing the dose further did not prolong the duration of effect. There were no systemic side effects, but there were a number of local transient side effects. The most significant side effect was ptosis, which occurred in 12% of treatments given to those patients with blepharospasm and hemifacial spasm. CONCLUSION: Botulinum toxin is an effective treatment for blepharospasm and hemifacial spasm, but there are a number of side effects, the most significant being ptosis.

Blepharoptosis↗
PubMed1989

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.

Adolescent↗
PubMed2005

Safety, effectiveness, and duration of effect of BOTOX after switching from Dysport for blepharospasm, cervical dystonia, and hemifacial spasm dystonia, and hemifacial spasm.

BACKGROUND: Local injection of botulinum toxin type A is first-line treatment of blepharospasm, cervical dystonia, and hemifacial spasm; however, there is uncertainty about the optimal dose of toxin for each indication as well as dose-conversion ratios that should be used when switching products in clinical practice. OBJECTIVE: The goal of this study was to compare the safety, effectiveness, and duration of clinical effect of BOTOX and Dysport and Dysportdagger after drug switching (Dysport to BOTOX) among patients with movement disorders. METHODS: A total of 48 patients diagnosed with blepharospasm (n = 27), cervical dystonia (n = 12), or hemifacial spasm (n = 9) were evaluated during a single-arm, crossover-design study in which each patient was his/her own historical control using a 5:1 or 4:1 conversion ratio of Dysport to BOTOX units. Patients were assessed (using Jankovic, Visual Analog Scale, general pain scale, Toronto Western Spasmodic Torticollis Rating Scale [TWSTRS] scores) after the last injection of Dysport and the first injection of BOTOX. Moreover, each patient kept a diary during treatment to track onset and duration of therapeutic effect. RESULTS: Although BOTOX and Dysport were both clinically effective, this effect was more significant with BOTOX compared to Dysport based on TWSTRS for cervical dystonia (p = 0.012), and Jankovic scores for blepharospasm (p = 0.006). Longer duration of effect also was noted with BOTOX than with Dysport (blepharospasm, 62.2 days vs 47.4 days (p = 0.001); cervical dystonia, 64.3 days vs 44.6 days (p = 0.014); hemifacial spasm, 65.1 days vs 41.8 days (p < 0.014), respectively). Of the 48 patients, 19 experienced at least one adverse drug reaction (ADR) during Dysport treatment, with the most commonly reported ADRs being ptosis for blepharospasm and hemifacial spasm and neck weakness for cervical dystonia. No patient reported an ADR during BOTOX treatment. CONCLUSIONS: Results suggest therapeutic effectiveness is enhanced with BOTOX compared to Dysport at a dosing ratio between 5:1 and 4:1 (Dysport:BOTOX). Safety and duration of therapeutic effect also are enhanced with BOTOX. Further research is needed.

Adult↗
PubMed1999

Diminished regional cerebral blood flow response to vibration in patients with blepharospasm.

OBJECTIVE: To determine whether patients with blepharospasm have abnormal sensorimotor processing similar to patients with writer's cramp. BACKGROUND: Blepharospasm is a focal dystonia manifest by involuntary, excessive blinking and squeezing of the eyes. Altered sensorimotor processing may contribute to the development of dystonic movements. Previously the authors demonstrated decreased vibration-induced cortical blood flow responses in hand primary sensorimotor area (PSA) in patients with hand dystonia. METHODS: In this prospective, case-control study, seven patients with blepharospasm were compared with seven normal subjects. PET measurements of regional blood flow were obtained using bolus administration of H(2)15O at rest or during sequential vibration of either the left or the right hand or side of the mouth. RESULTS: PSA activation decreased significantly in the patients with blepharospasm both ipsilateral (-68%; p = 0.0004) and contralateral to the side of facial stimulation (-56%; p = 0.0009). Patients had a 31% lower mean contralateral PSA response to hand vibration and a 51% smaller right supplementary motor area response to left-hand vibration than normal subjects, but these differences did not reach statistical significance. CONCLUSIONS: Patients with blepharospasm have abnormal sensorimotor processing in response to lower face vibration. They may also have abnormal brain responses to stimulation of clinically uninvolved parts of the body, but this requires confirmation.

Aged↗
PubMed1993

Botulinum a toxin treatment of hemifacial spasm and blepharospasm.

We studied the effects of botulinum A toxin in 101 patients with hemifacial spasm and 11 patients with blepharospasm in an open trial and double blind manner. All patients in the open trial and 6 patients in the double blind trial improved after the first injection of botulinum toxin. There was no improvement with placebo. The peak effect ranged from one to 6 days after injection and mean peak effect was 3.6 days in blepharospasm, and 4 days in hemifacial spasm. Of 144 treatments, 98.6% had excellent results, (below grade I). The duration of beneficial effect ranged 11 to 40 weeks (mean 16.5 weeks) in hemifacial spasm and 9 to 30 weeks (mean 14.2 weeks) in blepharospasm. Complications were encountered in 63.4% in hemifacial spasm and 72.7% in blepharospasm. The common side effects were dry eyes, mouth droop, ptosis and lid edema in order of frequency. These side effects were mild and resolved spontaneously in 1 to 3 weeks. Botulinum A toxin therapy is effective and convenient, and the treatment of choice for patients with hemifacial spasm and blepharospasm.

Adult↗
PubMed2000

Clozapine in the treatment of neuroleptic-induced blepharospasm: a report of 4 cases.

BACKGROUND: Blepharospasm, the forcible closure of eyelids, is an infrequent consequence of neuroleptic treatment that, when severe, can interfere with the ability to walk, drive, or work. Like tardive dyskinesia, blepharospasm can be disfiguring and aesthetically distressing, contributing to the increased stigmatization of patients. CASE REPORTS: We report 4 patients with DSM-IV schizoaffective disorder, paranoid schizophrenia, or chronic undifferentiated schizophrenia who developed neuroleptic-induced blepharospasm. In all patients, blepharospasm remitted without the reemergence of psychosis within 3 to 5 months of treatment with clozapine, 100-200 mg/day. CONCLUSION: The results suggest that clozapine may successfully treat neuroleptic-induced blepharospasm without the reemergence of psychosis in patients with schizophrenia, schizoaffective disorder, or schizophreniform disorder.

Adult↗
PubMed1999

Use of botulinum toxin A in the treatment of hemifacial spasm and blepharospasm.

BACKGROUND: Hemifacial spasm and blepharospasm are both dystonic disorders. They may seriously affect individuals' lifestyle and social activities. In 1990, the Food and Drug Administration of the USA approved botulinum toxin A as a therapeutic agent in the treatment of hemifacial spasm and blepharospasm. We present a therapeutic review of botulinum toxin A in 80 patients in Taiwan. METHODS: Fifty-eight patients with hemifacial spasm and 22 with blepharospasm. Botulinum toxin A was prepared and injected into the facial and eyelid muscles. Patients were monitored every two weeks and classified into four groups (excellent, moderate, mild and no improvement) according to the clinical improvement scale. Complications were also recorded. RESULTS: A total of 86.2% of hemifacial spasm patients and 81.8% of blepharospasm patients had excellent improvement on the spasm intensity scale, while 6.8% of hemifacial spasm and 9.0% of blepharospasm patients had moderate improvement. The complication rate was low and included transient mild facial weakness (5%), ptosis (3.8%), eyelid swelling and/or ecchymosis (3.8%), nausea/vomiting (2.5%) and transient severe facial weakness (1.3%). CONCLUSION: Botulinum toxin A is an excellent therapeutic agent to improve spasm intensity and has a low complication rate.

Adult↗
PubMed1998

[Blepharospasm and hemifacial spasm treated with botulinum A toxin injection].

OBJECTIVE: To study the efficacy of botulinum A toxin (BTA) injections for the treatment of blepharospasm and hemifacial spasm (HFS). METHODS: Twelve patients with blepharospasm and thirty eight patients with HFS were treated with BTA local injections (2.5 U to 5 U for each injection). RESULTS: Of the fifty patients, ten (86.7%) in blepharospasm and thirty two (84.2%) in HFS were completely relieved, two (13.3%) and five (13.2%) were remarkably relieved respectively, zero and one (2.6%) were partial relieved respectively. All patients experienced relief from spasm. The duration of the effect in blepharospasm were 10 to 24 weeks (mean, 18 weeks) and in HFS 14 to 32 weeks (mean, 22 weeks). The local side effects were transient and mild, including minor facial paralysis in eight patients, ptosis in four patients and tearing in four patients. No systemic adverse and allergic reactions were noted. CONCLUSION: Botulinum A toxin local injection is a safe, effective and simple means for the treatment of blepharospasm and HFS.

Adult↗
PubMed2003

[Blepharospasm in a patient with thymoma and positive anti-acetylcholine receptor antibody].

We report a rare case of a 70-year-old woman diagnosed as having blepharospasm with positive anti-acetylcholine receptor antibody. Blepharospasm developed in November, 2000, and increased in frequency, and worsened toward the evening. She complained of difficulty in her eyelid opening from October, 2002. Neurological examinations revealed blepharospasm and mild ptosis in both eyes. Tensilon test was negative. Waning and waxing were not detected in bilateral orbicularis oculi muscles by Harvey-Masland test. However, anti-acetylcholine receptor antibody was positive and thymoma in the anterior mediastinum was also found by the computed tomography of the chest. After the thymectomy, frequency of blepharospasm decreased. When blepharospasm worsened toward the evening, the co-existence of myasthenia gravis should be borne in mind.

Aged↗
PubMedDate not supplied

Cannabinoid agonists in the treatment of blepharospasm--a case report study.

The benign essential blepharospasm is a subliminal form of primary torsion dystonia with still uncertain aetiology. It is characterized by involuntary convulsive muscle contractions of the M. orbicularis occuli, accompanied by unbearable pain of the cornea, eye bulb and the muscle itself. It has been suggested that blepharospasm is neurobiologically based on a dysfunction of the basal ganglia and an impairment of the dopamine neurotransmitter system. Therefore, therapy of blepharospasm contains administration of anticholinergic- and tranquillizing drugs as well as botulinum toxin as neuromuscular blocking agent. However serious side effects can be observed as well as failure of therapy. In the brain a dense co-localisation of cannabinoid (CB1) and dopamine (D2)-receptor was identified which had been associated with the influence of cannabinoids on the dopaminergic reward system. Additionally, it has been demonstrated that cannabinoids may have an impact on the central GABAergic and glutaminergic transmitter system and thus might be involved in the influence of movement control. In the present case we administered the cannabinoid receptor agonist Dronabinol (Delta-9-Tetrahydrocannabinol) to a woman suffering from severe blepharospasm. Multiple treatments with botulinum toxin did not reveal a long-lasting beneficial effect. By contrast, treatment with 25 mg Dronabinol for several weeks improved the patients' social life and attenuated pain perception remarkably. This case study demonstrates that the therapy with a cannabinoid agonist may provide a novel tool in the treatment of blepharospasm and maybe of other multifactorial related movement disorders.

Analgesics, Non-Narcotic↗
PubMed1993

Botulinum toxin in the treatment of paralytic strabismus and essential blepharospasm.

As an alternative to conventional medical and surgical modalities that have met little success in the treatment of paralytic strabismus and essential blepharospasm, we explored the use of botulinum toxin as a treatment of choice in these two disorders. We used botulinum toxin in three patients with paralytic strabismus and in nine patients with essential blepharospasm. In three patients with paralytic strabismus, the botulinum toxin was injected into the ipsilateral antagonist of the paralysed muscle. The preinjection deviations ranged from 18 to 60 prism diopters. Two of these three patients achieved orthotropia around the thirtieth day and thereafter maintained it. The third patient became orthotropic on the eighteenth day, but deviation recurred and therefore required another injection of toxin. In nine patients with essential blepharospasm, botulinum toxin was injected into the orbicularis oculi muscles. Both objective and subjective improvement occurred in all nine patients within seven days and the effect lasted 12 to 15 weeks. Further injection of the toxin produced extremely beneficial results. However, the only significant complication that we encountered in both groups of strabismus and blepharospasm was ptosis, which was usually partial and temporary. From our experience, we advocate the use of botulinum toxin in the treatment of essential blepharospasm.

Adult↗
PubMed1998

Blepharospasm: past, present, and future.

To investigate causes, associations, and results of treatment with blepharospasm, 1,653 patients were evaluated by extensive questionnaires to study blepharospasm and long-term results of treatment with the full myectomy operation, botulinum-A toxin, drug therapy, and help from the Benign Essential Blepharospasm Research Foundation (BEBRF). The percent of patients improved by the BEBRF was 90%, full myectomy 88%, botulinum-A toxin 86%, and drug therapy 43%. The patient acceptance rate for the BEBRF was 96%, full myectomy 82%, botulinum-A toxin 95%, and drug therapy 57%. Blepharospasm is multifactorial in origin and manifestation. A vicious cycle and defective circuit theory to explain in origin and direct treatment rather than a defective specific locus is presented. All four forms of therapy evaluated are useful and must be tailored to the patient's needs. Mattie Lou Koster and the BEBRF have helped blepharospasm sufferers more than any other modality, and all patients should be informed of this support group. The full myectomy is reserved for botulinum-A toxin failures, and the limited myectomy is an excellent adjunct to botulinum-A toxin.

Blepharospasm↗
PubMed1998

Sensory modulation of the blink reflex in patients with blepharospasm.

OBJECTIVE: To measure the effects of a prepulse stimulus on the blink reflex responses elicited by an electrical stimulation of the supraorbital nerve in patients with blepharospasm with and without an effective sensory trick. DESIGN: Blink reflexes to supraorbital nerve stimulation were preceded in test trials by a prepulse electrical stimulus to the third finger at various leading intervals. SETTING: Ambulatory patients were treated regularly with botulinum toxin in the Neurology Department of the Hospital Clinic in Barcelona, Spain. SUBJECTS: Seventeen patients with dystonic blepharospasm and 11 age-matched control subjects. Eight of the patients with dystonic blepharospasm used a sensory trick to alleviate spasms and 9 did not. MAIN OUTCOME MEASURES: We measured amplitude of R1 and area of R2 responses elicited by the supraorbital electrical stimulus and determined the percentage of facilitation or inhibition induced by the prepulse. RESULTS: Prepulse facilitation occurred in the R1 response at intervals of 60 to 100 milliseconds and was normal in all patients. Prepulse inhibition occurred in the R2 response at intervals between 50 and 200 milliseconds and was abnormally reduced in 11 patients (64.7%), including all 9 patients who did not use a sensory trick and 2 of the 8 patients who did use a sensory trick. There was a positive correlation between absence of sensory trick and abnormality of the prepulse effects (chi(2)= 23.8; P < .001). CONCLUSIONS: Prepulse inhibition of the trigeminofacial reflex is abnormal in a percentage of patients with blepharospasm, and this abnormality occurs more frequently in patients who do not use a sensory trick. This sensory derangement may contribute to the maintenance of the dystonic spasms by reducing the amount of physiological gating from peripheral nerve inputs on trigeminofacial reflexes.

Aged↗
PubMed1983

Blepharospasm and orofacial-cervical dystonia: clinical and pharmacological findings in 100 patients.

We evaluated prospectively 100 patients, the largest reported series, with blepharospasm and orofacial-cervical dystonia, or Meige syndrome. The mean age at onset was 51.7 years, and 81% presented between the ages of 40 and 70. Women outnumbered men three to two. Blepharospasm was the initial symptom in 58 patients, but only 23 had involuntary movements localized to the orbicularis oculi. Sixty-one patients had the complete syndrome, blepharospasm and oromandibular dystonia, and 60 had neck or generalized dystonia in addition to the orofacial movements. Twenty-one patients with spasmodic dysphonia were included; in 12 of these patients, spasmodic dysphonia was part of the complete (Meige) syndrome, and 16 of these patients had neck or generalized dystonia or essential tremor. An organic cause of Meige syndrome is supported by a high correlation with essential tremor and other movement disorders and by positive family history in some patients. Response to medication was inconsistent, but 69% of patient trials resulted in some improvement; in 22% the benefit was marked and persistent. Tetrabenazine, lithium, and trihexyphenidyl were most useful for the treatment of oromandibular dystonia, and clonazepam was useful in some patients with blepharospasm.

Adult↗
PubMed1997

Blepharospasm and hemifacial spasm. Randomized trial to determine the most appropriate location for botulinum toxin injections.

PURPOSE: The purpose of the study is to analyze the effectiveness and side effects of botulinum toxin using four different treatment site applications to determine the most successful treatment regime with the least side effects. METHODS: In a prospective trial, 92 patients (50 blepharospasm and 42 hemifacial spasm) were assigned randomly to 1 of 4 different treatment groups (standard [S], brow [B], inner orbital [IO], or outer orbital [OO]). Each treatment group had a different pattern of injection sites in the orbicularis. A total of 285 treatments were given, and the mean follow-up time was 16.4 months. RESULTS: In the blepharospasm group, patients assigned to the standard group had a significantly longer duration of effect than for those in the brow, inner orbital, and outer orbital groups (8.1 weeks compared with 4.5, 4.2, and 3.1 weeks, respectively; P < 0.001). In the hemifacial spasm group, patients in the outer orbital group had significantly shorter duration of effect than those in standard, brow, or inner orbital group (7.2 weeks compared with 12.6, 12.8 and 10.4 weeks, respectively; P < 0.001). The four major complications of botulinum toxin treatment were epiphora, ocular irritation, ptosis, and diplopia. The inner orbital treatment produced significantly more episodes of ptosis (13% of treatments). However, the standard treatment produced the most epiphora and ocular irritation (18% of treatments). CONCLUSIONS: The position of the injection sites around the orbicularis influences the effectiveness and side effects of botulinum toxin treatment for patients with blepharospasm and hemifacial spasm. The further the treatment is away from the eyelid margin, the lower the risk of ocular side effects. The standard treatment produces the longest duration of effect in the blepharospasm group but with the most transient ocular irritation and epiphora. In the hemifacial spasm group, the brow treatment has an equally long duration of effect as that of the standard treatment with fewer side effects.

Aged↗