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Treatment of blepharospasm with botulinum neurotoxin type A: long-term results.

PURPOSE: To describe the long-term efficacy and side effects of treatment of blepharospasm with botulinum neurotoxin type A (Botox). METHODS: A total of 178 patients with blepharospasm were treated by injections of botulinum toxin in the Eye Clinic of the University of Naples from 1980 to 2001. The severity of spasm for each patient was graded on a four-point scale. Duration of improvement was assessed and reported in months. RESULTS: Of 178 cases, 10 were lost to follow-up; of the remaining patients, 93% reported improvement after treatments. The mean duration of improvement was 3.6 months. Twelve patients (76%) who underwent more than 14 treatments maintained stable relief. Three patients (1.7%) had a total remission of spasms. Side-effects were local; none of the 168 patients experienced any systemic or toxic reaction. CONCLUSIONS: Botulinum toxin therapy for blepharospasm can provide long-lasting relief and reduction of spasms in the majority of patients. This therapy has the advantages of being safe, simple, and repeatable.

Adult↗

Controlling blepharospasm effect during applanation tonometry using the Volk SuperField Lid Lens Adapter.

PURPOSE: To describe a practical clinical technique that can be used to control the effect of blepharospasm during applanation tonometry. METHODS: The Volk Superfield NC Lid Lens Adapter is used to keep the eye open and prevent the squeezing effect of orbicularis oculi in blepharospasm. RESULTS: This helpful tool can be used when blepharospasm becomes a problem that interferes with accurate intraocular pressure measurement. CONCLUSIONS: The technique is potentially useful and relatively easy to perform.

Blepharospasm↗

Stretch reflex blepharospasm.

We studied a patient with blepharospasm provoked by stretching the orbicularis oculi muscles. With the eyes closed, EMG of the orbicularis oculi at rest revealed spontaneous rhythmic muscle activity that was not visible. When she tried to open her eyes, repetitive jerking blepharoclonus and tonic blepharospasm rendered her functionally blind. Passive stretching of the orbicularis oculi evoked a burst of EMG activity with two components. Touch or pinprick did not elicit such reflex activity. Anesthesia of the supraorbital nerves abolished muscle responses to stretch and improved the blepharospasm.

Aged↗

Swallowing disorders in patients with blepharospasm.

Blepharospasm is a focal dystonia characterized by involuntary eye closure due to abnormal contraction of orbicular eyelid muscles. When blepharospasm is associated to the presence of involuntary oromandibular movements, it is termed Meige syndrome. The aim of this study was to investigate the presence of deglutition alterations in patients with concurrent blepharospasm and Meige syndrome. Twenty consecutive patients were studied by video fluoroscopy using a barium technique. The 4 stages of deglutition were investigated. Ninety percent of patients (18 cases) presented deglutition disorders. The more commonly found alterations were premature food drop, 15 cases (83%) and vallecuale residuals, 14 cases (78%). Sixty seven percent of abnormal findings occurred in the third stage of deglutition. Eighty-nine percent of patients (16) presented more than one swallowing alteration. There was a positive and significant correlation between the number of alterations and patient's age or disease duration. Prevalence of swallowing disorders in the healthy elderly population is reported to be 44%. In our series it reached 90%, suggesting that our findings might be related not only with age but also with a more widespread dystonia exceeding the orofacial muscles.

Adult↗

[Surgical treatment of blepharospasm: results of a study of 138 patients using an improved disability scale].

We applied a new blepharospasm clinical disability scale to the evaluation of blepharospasm surgery in a group of 138 patients. This scale analyzes six parameters of daily life, namely reading, watching television, household tasks, walking, employment, and driving. The patients had either essential blepharospasm or pretarsal dystonia. Frontalis suspension of the eyelid gave slightly better functional results than orbicularis resection in some patient subgroups.

Activities of Daily Living↗

Facial dystonia, essential blepharospasm and hemifacial spasm.

Movement disorders, or dyskinesias, in the facial region may be categorized in several ways. Dystonic movement disorders in the cranial-cervical region, including essential blepharospasm, Meige syndrome and spasmodic torticollis, are characterized by uncontrollable squeezing movements in the face and neck. These disorders typically present in the fifth and sixth decades of life. Essential blepharospasm is particularly debilitating, as the involuntary eyelid closure that accompanies this condition may result in functional blindness with an otherwise normal visual pathway. Hemifacial spasm is an intermittent, unilateral, spasmodic contraction of the muscles innervated by the facial nerve. This disorder usually presents in the third or fourth decade and has a different underlying pathophysiology than the dystonias. Botulinum A toxin therapy has largely supplanted surgical intervention in the treatment of essential blepharospasm and hemifacial spasm.

Blepharospasm↗

Blepharospasm associated with olivopontocerebellar atrophy.

We report two cases of cranial dystonia (blepharospasm) associated with olivopontocerebellar atrophy (OPCA). The pathophysiology of blepharospasm appears to involve an increased excitability of the interneurons of the blink and corneal reflexes. It is hypothesized that blepharospasm associated with OPCA might be due to rostral brainstem lesions disrupting central dopaminergic and cholinergic pathways, resulting in disinhibition of brainstem reflexes or denervation supersensitivity of the facial nuclear complex.

Blepharospasm↗

[Treatment of blepharospasm and facial hemispasm by injection of botulinum toxin].

13 patients with hemifacial spasm and 19 patients with blepharospasm (essential blepharospasm or Meige syndrome) were treated with injection of botulinum A toxin in the eyelids. The effectiveness of this therapy is constant and the treatment offers relief to almost all patients. The response times for repeated treatment was 6 months for blepharospasm and 7 months for hemifacial spasm. Repeated injections were performed in several patients and gave the same improvement. Transient ptosis or diplopia were observed in 11 patients. Possible systemic side effects (respiratory trouble) were documented in two patients. Botulinum toxin injection is an effective treatment but the relief is usually temporary and repeated treatments are necessary.

Adult↗

Treatment of blepharospasm with medication, surgery and type A botulinum toxin.

A total of 39 patients with essential blepharospasm and 2 patients with hemifacial spasm were treated with one or more forms of therapy. All patients underwent neurologic and ophthalmic assessment to rule out ocular causes of blepharospasm. Thirty-six patients were given a trial of various medications. Only one patient was successfully treated: her condition was markedly improved with pimozide after benztropine mesylate, clonazepam and amantadine hydrochloride had failed to help. Patients who did not respond to drug therapy were offered the option of undergoing eyebrow-eyelid muscle stripping surgery. The six patients who underwent surgery showed considerable improvement; however, side effects such as frontal anesthesia, exposure keratitis, lagophthalmus, scarring and eyelid malposition occurred, and three of the six had residual spasm. At this point type A botulinum toxin became available. A total of 27 patients (26 who did not respond to drug therapy, including the 3 with residual spasm after surgery, and 1 previously untreated patient) received type A botulinum toxin injections. Most experienced rapid relief from their spasms. The beneficial effects lasted weeks to months, and there were no major side effects. Treatment with type A botulinum toxin appears to be a safe and effective means of temporarily relieving blepharospasm. The long-term results with repeated injections are yet to be determined.

Baclofen↗

Management of blepharospasm.

From 1950 through 1984, 123 patients underwent surgical treatment of blepharospasm at the Mayo Clinic. During this period, four different operations (proximal and distal neurectomy and two types of myectomy) were used. Significant recurrent or residual blepharospasm was observed more frequently (P less than 0.01), and need for subsequent operations was greater (P less than 0.01), among patients who had undergone distal neurectomy than among those who had had myectomy. These data support the view that myectomy is a more effective procedure than neurectomy. Initial results with botulinum toxin injection seem to indicate that it is an effective short-term treatment for blepharospasm. However, its long-term efficacy and safety need further study, as does the role it should play in combination with myectomy.

Adult↗

Blepharospasm.

Blepharospasm is a cranial nerve dysfunction in which involuntary and uncontrollable forcible lid closure occurs. Surgical treatment is far from ideal, but the condition can be improved by sectioning the branches of the facial nerve plexus which supply the orbicularis oculi, the frontalis and corrugator muscles, and the midfacial muscles. Not all patients with blepharospasm should be operated upon, since they may expect too much or may enjoy the attention their invalidism has given them. Ectropion of one or both lower lids may occur, and the most severe of all complications is recurrence. This may happen despite a careful microscopic search for the nerve fibers and their division and avulsion. Blepharospasm patients often require considerable psychological support.

Adult↗

[Botulinum toxin type A (BOTOX) for treatment of blepharospasm: an open label, dose-response study].

The clinical effect of Botulinum Toxin Type A (BOTOX) were studied in patients with blepharospasm. Clinical symptoms were evaluated with Jankovic's rating scale, widely used for blepharospasm. To see dose response, eyelid muscle force of the patients was measured with a device recently developed for measurement of eyelid muscle force. The results showed significant improvement (p = 0.0000) on Jankovic's rating scale in all patient groups after effective dose injections of 0.5, 1.25, and 2.5 U/site. Particularly the number of patients with marked improvement (decrease of six points or more in total score on Jankovic's rating scale) increased with higher dose injections. The eyelid muscle force also decreased by 33.2 +/- 28.1%, 41.7 +/- 25.1%, and 69.6 +/- 5.0% in patients groups after effective dose injections of 0.5, 1.25, and 2.5 U/site, respectively. The decrease of the eyelid muscle force showed significant dose response (p = 0.0254). Mean duration of effect was 12.9 weeks in patients after effective dose injections of 1.25 U/site, which was significantly longer (p = 0.0205) than that of 9.6 weeks in patients after effective dose injections of 0.5 U/site. No severe adverse effects were observed. We concluded that BOTOX injections of 1.25 U/site or more are a safe and effective treatment of blepharospasm.

Adult↗

Botulinum toxin A in blepharospasm and hemifacial spasm.

We report the first Indian experience of botulinum toxin A in the treatment of blepharospasm and hemifacial spasm. Sixteen patients, 7 with essential blepharospasm, 5 with Meige syndrome and 4 with hemifacial spasm received botulinum toxin A injection. One patient received 3 courses of injections, 2 received 2 courses and the rest received only one course. The effect was observed after a latent period of less than 48 hours in all patients and lasted for a mean of 16.65 weeks. More than 70% improvement occurred after 17/20 injections (85%). Poor response was more often seen when blepharospasm was associated with oromandibular dystonia (2/5 injections). Though the duration of response and subjective score of improvement was best in patients with hemifacial spasm, the numbers were very small for any statistical evaluation. The side effects were local, transient, mild and well tolerated. The commonest side effect was blepharoptosis.

Adult↗

A comparative study of tear secretion in blepharospasm and hemifacial spasm patients treated with botulinum toxin.

In the neuro-ophthalmology clinic at St. Vincent's Hospital, Melbourne, 57 patients with blepharospasm and 50 patients with hemifacial spasm were treated with botulinum toxin. Schirmer tear tests were conducted on all the patients prior to each treatment and at 1 week following treatment where possible. The results were compared with a control group of 107 patients selected by age and sex. The blepharospasm patients were found to have a significantly lower tear secretion than that of the control group, using the Mann-Whitney test (median = 3.5 mm, compared with median-11.0 mm, p < .0001). This did not improve following treatment. The patients with hemifacial spasm did not have significantly different tear secretion from that of the control group (t = 1.0, p > .05). To investigate whether there was any relationship between the symptoms and the result of the Schirmer test, a survey was also conducted on the patients with blepharospasm and hemifacial spasm regarding symptoms, frequency, and type of drops/ointment used.

Aged↗

[Blepharospasm caused by bilateral paramedian thalamic infarction].

A 67 year-old woman developed a small infarct in the superficial anterior territory of the left middle cerebral artery, then 2 other successive infarcts in the left then right thalamic paramedian territories. Left thalamic infarct was associated with right hemiparesis and speech disorders of the subcortical type. The occurrence of the additional right thalamic infarct was associated with stupor, left hemiparesis and oppositional behavior. A severe blepharospasm and paralysis of convergence and vertical eye movements were also noted. Blepharospasm has been rarely reported with structural central nervous system lesions. An extensive literature review disclosed 2 other cases associated with thalamic paramedian infarcts and 2 additional cases described with other thalamic lesions. In all these cases, blepharospasm seems to have been due to lesions of the intralaminar and dorsomedian nuclei.

Aged↗

Pathophysiology of blepharospasm and oromandibular dystonia.

The pathophysiology of reflexes mediated by the fifth and seventh cranial nerves has been studied in 16 patients with blepharospasm and oromandibular dystonia compared with normal age-matched subjects. The EMG activity of the dystonic spasms in the periocular and jaw muscles was similar to that described in other muscles in patients with generalized torsion dystonia. The latency of the R1 and R2 components of the blink reflex and of the corneal reflex was normal. However, the amplitude and the duration of the R1 and R2 and the duration of the corneal reflex were increased. In some patients the R1 component was also present on the side contralateral to the stimulus, while in normal subjects it was present only on the ipsilateral side. The excitability cycle of recovery of the R2 component of the blink reflex after a prior conditioning shock was enhanced in the patients. There were no EEG potentials preceding blepharospasms in the patients, although a Bereitschaftspotential was seen beginning some 500 ms prior to voluntary blinks in the same individuals. Exteroceptive suppression in the contracting masseter and orbicularis oculi muscles was absent in 40 to 50 per cent of the patients. The jaw jerk was present in all the patients with normal latency. These results indicate that the neuronal arcs of the facial reflexes in blepharospasm and oromandibular dystonia are normal. However, there is an abnormal excitatory drive, perhaps from the basal ganglia, to the facial motoneurons and the interneurons which mediate the facial reflexes in the brainstem.

Adult↗

Botulinum toxin in the management of blepharospasm.

Seventy-seven patients with blepharospasm unresponsive to other forms of therapy were treated with botulinum A toxin. The drug was injected into the orbicularis oculi muscle of both upper and lower eyelids and, in some patients, into the brows and upper part of the face as well. A total of 192 treatment sessions were given, with follow-up ranging from four weeks to 18 months. Orbicularis muscle spasms decreased markedly over the first five days following injection, both in patients with essential blepharospasm and in those with hemifacial spasm. Beneficial effect was temporary, however, and repeated injections were required for sustained relief. Fifty-two patients received multiple treatments, ranging from two to eight injections each, with a mean duration of benefit of 11.8 weeks. Results of subsequent injections were similar to the first in rapidity of onset, degree of benefit, and duration of effect. Complications were local, mild, and transient, and no systemic side effects were encountered.

Adult↗

Treatment of essential blepharospasm. II. A modification of exposure for the muscle stripping technique.

The refinement of a muscle excision technique for the correction of essential blepharospasm has been a major contribution to treatment. In this procedure, incisions are made directly above the eyebrow to excise the brow muscles, often resulting in adherent scars and poor brow position. The bicoronal scalp flap has been used by many other surgical subspecialities for exposure of the frontal bone area, eg, in neurosurgical procedures, sinus surgery, and cosmetic forehead lifts. The use of the coronal flap exposure for excision of the corrugator and procerus muscles has allowed them to be more easily excised and has avoided the problems associated with the direct brow incisions. The Anderson muscle stripping technique, combined with the coronal flap exposure for the brow muscles, provides the best correction for the spasms in patients who have essential blepharospasm. The frequency of complications is low, and patient acceptance is high.

Blepharospasm↗