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PubMed2005

Bell's palsy-induced blepharospasm relieved by passive eyelid closure and responsive to apomorphine.

OBJECTIVE: We describe the case of a woman with Bell's Palsy-induced blepharospasm (BPIB) of the right eye that appeared simultaneously with a complete left facial nerve palsy. The involuntary spasm was relieved by passive lowering of the upper eyelid on the paretic side. METHODS: The recovery curve of the blink reflex was evaluated on the non-paretic side in baseline conditions, after subcutaneous apomorphine and placebo administration and 8 months later, at recovery from the palsy. RESULTS: We found increased recovery of the test-R2 responses at short interstimulus intervals at baseline, which was normalised by apomorphine but not by placebo. At recovery the blink reflex R2 recovery curve returned to normal. CONCLUSIONS: This report demonstrates for the first time a response of BPIB to a dopamine agonist. SIGNIFICANCE: Our findings are in agreement with an animal model of blepharospasm that suggests a combined role of weakness of the orbicularis oculi muscle and a dysfunction of the dopaminergic system in the pathogenesis of this disorder.

Apomorphine↗
PubMed1992

Effects of eyelid protractor excision for the treatment of benign essential blepharospasm.

Data were analyzed from 37 patients with essential blepharospasm who had undergone upper eyelid protractor excision with brow suspension, and who had a median follow-up of 40 months (range, 12 to 83.5 months) after surgery. Of the 37 patients, 15 (40.5%) had sufficient relief of their spasms at their last follow-up; 22 patients (59.5%) had functionally significant recurrent or residual spasms after upper eyelid surgery. Of these 22 patients, 12 (55%) had recurrences more than six months after surgery, and four patients (18%) had recurrences more than one year after surgery. Patients with Meige syndrome were no more likely to experience recurrent spasms after upper eyelid surgery than patients who lacked a lower facial spasm component. Recurrence of symptoms was primarily the result of lower eyelid spasms in 11 patients (50%), upper eyelid spasms in one patient (5%), and all eyelids in six patients (27%). The location of recurrent spasms could not be ascertained in four patients (18%), with whom we had only telephone contact. Seven of 12 patients (58%) undergoing secondary procedures had relief from their spasms at the end of this study. Excluding those patients who chose to decline further surgery, 22 of 27 patients (81.5%) received satisfactory relief of symptoms after upper eyelid and, if needed, lower eyelid and repeat upper eyelid protractor excision. These long-term results are comparable to those reported previously for differential section of the facial nerve. Because the side effects of eyelid protractor excision are generally more acceptable, we recommend that when surgery is determined to be necessary for essential blepharospasm, upper eyelid protractor excision is the preferred surgical approach.

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PubMed1985

Myectomy for essential blepharospasm.

Essential blepharospasm is a progressive, bilateral, involuntary facial spasm. In this study, we conducted a prospective analysis of myectomy for the treatment of this condition in 21 patients. The procedure consisted of extirpation of the muscles that close the eyelids (protractors), which include the pretarsal, preseptal, and orbital components of the orbicularis oculi, as well as the procerus and corrugator supercilii muscles of the brow. In addition, the muscles that open the eyelids (retractors) were strengthened (by resection of the frontalis muscle and repair of disinsertion of the levator aponeuroses). The most common symptoms that necessitated myectomy were difficulty with driving (20 patients), watching television (17 patients), reading (15 patients), and eating (5 patients). Overall, the 21 patients had a 70% mean decrease in symptoms postoperatively. Five patients required an additional operation for management of residual blepharospasm.

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PubMed1986

The use of botulinum toxin in the medical management of benign essential blepharospasm.

Twenty-eight patients with mild or moderate cases of benign essential blepharospasm were treated with botulinum toxin Type A. Average follow-up was six months. The injection technique used on these patients is illustrated. The treatment was effective in virtually all patients treated, although transient; the mean interval of relief of spasm was approximately two and one-half months. Potential side effects include ptosis and epiphora. Botulinum toxin as an initial treatment or as an adjunct in postsurgical residual blepharospasm shows promise in this preliminary study.

Adult↗
PubMed1988

Long-term results and complications of botulinum A toxin in the treatment of blepharospasm.

The authors review their long-term results and complications with the use of botulinum A toxin in the treatment of facial dystonias. Two hundred thirty-two patients in three diagnostic groups--essential blepharospasm, hemifacial spasm, and Meige's syndrome--were treated with botulinum A toxin. A total of 1044 treatments were given over a 4-year period. A reduction in orbicularis spasm intensity was noted in 1012 (96.9%) treatments (mean duration, 13.3 weeks). There was no clear relationship between toxin dose and the amount of spasm reduction or duration of response, and average duration of beneficial effect remained constant from the first through the twelfth injections. Complications occurred in 236 (22.6%) treatments. In most cases, these were local and transient. Symptomatic dry eye was the most common side effect, noted in 7.5% of cases. Ptosis was reported in 7.3% of treatments and photophobia in 2.5%. Diplopia involving the inferior oblique or lateral rectus muscles was seen in less than 1% of cases. There were no differences in degree of response or in complications among the three diagnostic groups, although there was a slight difference in duration of effect. Patients who had undergone previous eyelid surgery for blepharospasm did not respond differently from those without prior surgery.

Adult↗
PubMed2001

The stimulus intensity modifies the blink reflex recovery cycle in healthy subjects and in blepharospasm.

OBJECTIVE: The excitability of human brainstem interneurons is measured by the blink reflex recovery cycle and is abnormal in blepharospasm. We wondered whether the results of this paradigm depend on the stimulus intensity. METHODS: We investigated the blink reflex recovery cycle in 13 healthy control subjects and in 13 patients with blepharospasm (7 of them treated with botulinum toxin) using 4 levels of stimulus intensity (5, 12, 19 and 26 mA) and two interstimulus intervals (ISIs, 150 or 250 ms). RESULTS: In all groups the inhibition of the second R2 response was reduced with strong stimulus intensities: In controls, the inhibition of the second R2 decreased significantly (e.g. young controls, ISI 150 ms, from 89.6+/-15.6% at 5 mA to 21.9+/-49.7% at 26 mA, mean+/-standard deviation). In patients the R2 inhibition found at 5 mA was converted in an R2 facilitation at 26 mA, irrespective of the status of treatment. In addition, the patients' results at 5 mA did not differ significantly from the controls' results at 26 mA. CONCLUSIONS: The R2 inhibition and its sensitivity to detect abnormal interneuronal excitability depend on the stimulus intensity, which may act by modifying the excitability of the R2 interneurons.

Adult↗
PubMed1987

Botulinum toxin for benign essential blepharospasm, hemifacial spasm and age-related lower eyelid entropion.

Purified botulinum A exotoxin was used in the treatment of forty seven patients with benign essential blepharospasm, 11 patients with hemifacial spasm and 2 patients with age-related entropion. The treatment was effective in all three groups for an average of 3-4 months when symptoms recurred and repeated chemodenervation with toxin was needed. The commonest complication was transient ptosis with an overall frequency of 7.8%. This incidence increased to 11.1% with toxin doses higher than 25 units per orbicularis. The treatment was well accepted by the patients, who were subsequently able to return to pre-blepharospasm lifestyles.

Adult↗
PubMed1990

[The trigemino-facial reflex in patients with essential blepharospasm. A contribution to the etiology of the disease picture].

We conducted electrophysiological investigations in altogether 11 patients with an essential blepharospasm. In all patients the blink reflex - ipsi- and contralateral - was measured. We found reproducible pathologic changes (direct R1/R2 transition, contralateral early component with and without direct transition into the R2-component, increase of R2-duration). These findings point to a central genesis of blepharospasm in some cases.

Blepharospasm↗
PubMed1987

[The disease picture of severe blepharospasm, the cause and a new kind of treatment with long-term results].

The etiology of essential blepharospasm is extraordinarily manifold and still not clarified in many instances. The therapeutic possibilities are equally manifold and not always satisfactory. A new method of surgical intervention consists in the dissection of the zygomaticotemporal nerve shortly after its separation from the stem of the facial nerve. In 10 cases with particularly severe blepharospasm, a permanent therapeutic result which was satisfactory in every respect was achieved in collaboration with an otorhinolaryngologist. For the ophthalmologist and otorhinolaryngologist a knowledge of the various therapeutic possibilities is important in order to adjust treatment accordingly.

Blepharospasm↗
PubMed1986

[Acupuncture for pain in the cranial region and for blepharospasm without organic cause].

In patients with functional ophthalmological disturbances classical acupuncture therapy was compared to a technique of inserting the needles close to superficial nerves. Seventy-five patients with migraine, 47 patients with functional blepharospasm, 18 with trigeminal neuralgia, and 9 with posttraumatic pain syndromes were treated. Treatment comprised seven sessions at four-day intervals. Of 62 patients undergoing classical acupuncture, 21 (33.9%) were subsequently free of complaints and 33 (53.2%) felt a great improvement. Nerve points were stimulated in 83 patients, 31 (38%) of whom were completely free of symptoms after treatment, and 45 of whom (54%) felt much better. Hence, there was no marked difference between the two methods as regards therapeutic effect. These results support the concept of acupuncture therapy being a combination of dermal stimulation and suggestion. The therapeutic approach to pain or blepharospasm by use of the mainly suggestive effects of acupuncture is recommended only if underlying organic diseases can be ruled out.

Acupuncture Therapy↗
PubMed1993

A possible feline model for human blepharospasm.

'Benign essential blepharospasm' is a human eyelid disorder of unknown aetiology characterized by involuntary, bilateral, and disabling spasmodic contracture of the orbicularis oculi muscle. Treatments are frequently disappointing. Here we report what might be a first step toward developing an animal model for exploring mechanisms of the disorder and potential treatments. We surgically implanted stimulating electrodes into brain areas known to supply input to the lateral division of the facial nerve nucleus to induce blinking by electrical stimulation. Single-pulse stimuli at or near the facial, parabrachial, red, and interstitial nuclei produced consistent stimulus-induced eyelid contractions. Responses were ipsilateral to stimulation, except for the interstitial nucleus where contralateral responses occurred. Little or no other movements of the face, head, or body occurred at eye-blink threshold voltages. When these sites were stimulated with pulse trains, eyelid closures followed stimulus frequency and tended to fuse into constant closure. Thresholds at each stimulus site remained constant during three days of testing. Drug treatments produced no consistent effect on eye-blink threshold from any stimulation site, even when general behaviour was affected. We conclude that these input pathways to the facial nucleus may contribute to blepharospasm and that future neurochemical and electrophysiological study of these pathways may produce a suitable animal model for understanding this disorder.

Animals↗
PubMed2000

Anatomical reasons for problems after neurectomy for blepharospasm: a study in cadavers.

The purpose of this study is to clarify the mechanisms of the problems that develop after neurectomy for blepharospasm. The left facial nerves in 10 Japanese cadavers were dissected under a surgical microscope. The temporal, zygomatic, and buccal branches innervated to the orbicularis oculi muscle. These three groups formed a well-communicating plexus posterior to the orbicularis. The most inferior buccal branch curved in the deep layer in the lower part of the cheek. In the cheek, both the buccal and the temporal branches had ramifications of other facial muscles. The information given in previous anatomical textbooks did not specifically define the denervation of the orbicularis. When neurectomy was done in the past, the inferior buccal branch may have been kept intact, or other facial muscles as well as the orbicularis may have been denervated which caused the blepharospasm to recur and complications to develop after neurectomy.

Adult↗
PubMed1994

Abnormal eye movements in blepharospasm and involuntary levator palpebrae inhibition. Clinical and pathophysiological considerations.

We report on four patients with involuntary eyelid closure and eye movement disorders. Three were healthy until the onset of their illness and one had a mild generalized choreoathetosis and dystonia due to kernicterus. Electromyographic recording revealed solely blepharospasm in two patients and blepharospasm in combination with involuntary levator palpebrae inhibition in the other two. The eye movement abnormalities were clinically characterized by inability to fix gaze and short or prolonged episodes of uncontrollable eye deviations accompanied, in two patients, by diplopia in horizontal or vertical directions. These episodes occurred independently of a disorder of eyelid movement. Eye movement recordings with a double magnetic induction technique showed saccadic intrusions in horizontal directions. They consisted of highly frequent square wave jerks in three and sporadic macro-square wave jerks in two patients. There were also episodes of extraocular muscle dystonia, commonly known as oculogyric crises, resulting in involuntary upward eye deviation in all patients and lateral deviation in three patients. In one patient, nasal-ward deviations were sometimes restricted to one eye. We conclude that these abnormal eye movements do not necessarily point to a symptomatic form of dystonia and that they may limit the beneficial effect of botulinum toxin or surgical intervention in the therapeutic management of involuntary eyelid closure. We suggest that either basal ganglia, especially substantia nigra pars reticularis and the brainstem structures, especially the paramedian pontine reticular formation, or both, may be involved in the pathogenesis of these abnormal movements.

Adult↗
PubMed1986

Progressive supranuclear palsy: report of a case with torticollis, blepharospasm, and dysfluency.

A 76-year-old man is reported with advanced progressive supranuclear palsy (PSP) who developed a persistent, gradually progressive torticollis over a period of several months. Blepharospasm and dysfluency of the extrapyramidal type antedated the torticollis. This first report of torticollis in PSP reinforces previous notions that torticollis is related to pathologic changes in the striatum and brainstem. In addition, the combination of torticollis and blepharospasm in our patient supports the previous concept that these two "focal dystonias" have a common pathophysiologic mechanism. This also suggests that dysfluency in PSP may be an expression of a focal dystonia involving the muscles of articulation.

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PubMed1984

Psychological aspects of essential blepharospasm.

Evaluation of 20 essential blepharospasm patients by using structured interview and psychological testing (MMPI, Rorschach) revealed characteristic pathological personality patterns in all cases. Recent losses or life change, marital conflict, and specific precipitating events were common. Test findings indicated unexpressed hostility, denial, and repression in a majority of women. Three of five men showed evidence of depression. This initial systematic investigation of psychological factors in blepharospasm supports the hypothesis that neurological and psychogenic factors may interact etiologically. The data suggest that personality, recent stress, and precipitating events warrant both clinical assessment and controlled research to determine the specificity and prevalence of these findings.

Adult↗
PubMed1995

Blepharospasm and related facial movement disorders.

The variable clinical features and the relatively good response of blepharospasm to botulinum-toxin type A are now well established. The etiology and pathophysiology of blepharospasm and related facial movement disorders are still poorly understood. Genetic and histopathologic studies over the last year have contributed to our understanding of this disease. The most significant progress has been made in the electromyographic studies of the the levator palpebrae and orbicularis oculi muscles. Subclassification based on the electromyographic abnormalities of these two muscles have begun to improve our understanding of the variable responses to botulinum-toxin type A. Further electromyographic studies may help identify the best sites of injection for optimal response and differentiate patients requiring limited or complete myectomy. The development of the limited myectomy has provided excellent functional and cosmetic results with quick recovery times in selected patients.

Blepharospasm↗
PubMedDate not supplied

Clozapine for blepharospasm, obsessive-compulsive symptoms, and psychotic symptoms in a patient of old brain infarction.

Blepharospasm, psychotic symptoms, and obsessive-compulsive symptoms can result from brain infarction. However, a presentation of these 3 symptoms simultaneously is rare. This report describes a 65-year-old woman who had old brain infarction and presented with these 3 symptoms for 2 years. The patient recovered after receiving a regimen of clozapine 125 mg/d for 3 months. No recurrences of symptoms were noted during a 6-month follow-up. This is the first case report demonstrating that clozapine is effective for a patient with blepharospasm, obsessive-compulsive symptoms, and psychotic symptoms presenting simultaneously. This report also reminds physicians of the possible organic causes of unusual presentations in elderly patients.

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PubMed2006

Use of high-dose botulinum A toxin in benign essential blepharospasm: is too high too much?

BACKGROUND: Botulinum toxin (Botox) is the mainstay treatment for benign essential blepharospasm. Current treatment practice appears restricted by several reports demonstrating adverse effects and resistance to high-frequency, higher-dose therapy. This study aimed to explore whether high-dose, high-frequency treatments could be used without developing secondary resistance and without significant side-effects in patients refractory to conventional Botox doses. METHODS: From a cohort of 120 patients being treated with Botox therapy for benign essential blepharospasm and idiopathic hemifacial spasm, case notes from six patients were retrospectively examined. In these patients, therapy had exceeded the recommended 50 units per side for a duration greater than 12 months and at less than 3 monthly intervals. Patterns in subjective severity grading and percentage of improvement as well as reported side-effects were analysed. RESULTS: All patients described greater than 60% improvement and 0-2 severity grading over a 3- to 15-year period with no evidence of secondary resistance. Side-effects were minor, transient and less frequently reported at higher doses. CONCLUSION: In a select group of patients, Botox therapy can be used effectively at doses higher than recommended over long periods with minimal side-effects and little evidence of secondary resistance.

Aged↗