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PubMed1984

Treatment of blepharospasm with botulinum toxin. A preliminary report.

The effects of botulinum A toxin injections for the treatment of facial spasm were analyzed for 22 patients. Sixteen patients had unoperated on essential blepharospasm, three had essential blepharospasm with residual spasm following previous surgical treatment, and three had unoperated on hemifacial spasm. Treatment was effective for most patients, but transient, with the mean interval of relief of spasm after the first injections being ten weeks. The injection of botulinum toxin reduced the maximum lid force by about 10%. While side effects were common, they were generally mild and well tolerated. No cumulative effect of botulinum toxin was evident in those receiving three series of injections. There is no significant difference in either the interval free of spasm or the rate of lid-force recovery following any of three sequential injections of increasing doses of botulinum toxin.

Adult↗
PubMedDate not supplied

Botulinum toxin for blepharospasm: challenges for rehabilitation nurses.

Blepharospasm is a chronic, progressive, involuntary spasmodic closure of the eyelids associated with abnormal facial and oromandibular movements. It is a neurologic disorder whose cause is unknown and whose pathophysiology is poorly understood. Without appropriate treatment, it can result in functional blindness and other disabilities. In the last decade, botulinum toxin has been found to be effective therapy for most individuals. The drug, which is given by local injection, has a denervation effect. It relieves symptoms for several months, allowing patients to resume their former lifestyles between treatments. This new therapy modality challenges rehabilitation nurses to bridge the gap between disabled persons in the community and this new technology. Casefinding, referrals, and patient education are among the interventions that can help meet this challenge. The major purpose of this article is to inform rehabilitation nurses about how to recognize the symptoms of neurologic blepharospasm and how to intervene to prevent disabilities that could result.

Blepharospasm↗
PubMed2002

Intracortical excitability in the hand motor representation in hand dystonia and blepharospasm.

We sought to determine the activity of inhibiting and facilitating cortical circuits in areas surrounding a hand muscle motor representation in focal dystonia and in controls. In 15 patients with hand dystonia, 16 patients with blepharospasm, and age-matched controls, we applied suprathreshold transcranial magnetic stimuli with a figure-eight coil over the optimal representation of the relaxed abductor digiti minimi muscle of the dominant hand. Additional conditioning stimuli were given through a second figure-eight coil that was held either above the test coil or 2 cm or 4 cm apart in the anterior, posterior, lateral, or medial direction. We measured intracortical excitability in each of the nine positions of the conditioning coil. Intracortical inhibition was reduced in both patient groups at all conditioning coil positions. With both coils centered, the intracortical facilitation did not differ between patients and controls. After shifting the conditioning coil, the intracortical facilitation tended to be less diminished in patients than in controls, this difference between patients and controls was significant for the anterior, posterior, and medial 4-cm conditioning coil shift. Our results demonstrate decreased intracortical inhibition in the cortical hand muscle representation not only in patients with hand dystonia, but also in patients with blepharospasm. In addition, our findings in both patient groups show a trend toward a relatively increased intracortical facilitation in surrounding motor areas.

Adult↗
PubMed2003

Familial blepharospasm is inherited as an autosomal dominant trait and relates to a novel unassigned gene.

Blepharospasm (BSP) is a common form of primary torsion dystonia (PTD). Although most cases are sporadic, an increased familial incidence of BSP has been reported. Precisely how blepharospasm is inherited remains unclear. We report on two Italian families with adult-onset focal BSP inherited as an autosomal dominant trait with reduced penetrance. None of the affected family members had the 3-bp (GAG) or the 18-bp deletion in the DYT1 gene. In one family, linkage analysis allowed us to exclude segregation of the disease with the known PTD loci (DYT1, DYT6, DYT7, and DYT13). These findings suggest that primary familial adult-onset BSP is a distinct entity among inherited PTD and is caused by a novel, unmapped gene.

Adolescent↗
PubMed2004

Improvement of blepharospasm with Zolpidem.

Zolpidem (ZLP) is an imidazopyridine that binds to GABA receptors. We report on improvement of blepharospasm in 3 patients treated with ZLP. The GABAergic action of this drug on the output structures of the basal ganglia could explain the improvement of blepharospasm in these patients.

Aged↗
PubMed2007

Assessing the role of DRD5 and DYT1 in two different case-control series with primary blepharospasm.

Primary blepharospasm is a common adult-onset focal dystonia. Polymorphisms of the genes encoding TorsinA (DYT1) and the D5 dopamine receptor (DRD5) have previously been associated with lifetime risk for focal dystonia. We describe here experiments testing common variability within these two genes in two independent cohorts of Italian and North American patients with primary blepharospasm. We have failed to identify a consistent association with disease in the two patient groups examined here; however, analysis of the Italian group reveals an association with the same risk genotype in DYT1 as previously described in an Icelandic population. We have also found global significant DYT1 haplotype differences between patients and controls in the Italian series. These data suggest that further examination is warranted of the role genetic variability at this locus plays in the risk for primary dystonia.

Blepharospasm↗
PubMed2007

Asynchronous blepharospasm, facial and cervical dystonia, and bilateral asynchronous hemifacial spasm.

We present a patient with a facial movement disorder that has characteristics of both blepharospasm and bilateral asynchronous hemifacial spasm. Because of the increased incidence of blepharospasm in patients with hemifacial spasm, our patient's clinical presentation is probably not a chance occurrence, but rather a manifestation of some predisposition for these two movement disorders. This unusual constellation of signs and symptoms challenges the current diagnostic criteria and suggests that some of these facial movement disorders may lie on a spectrum, rather than represent distinct entities.

Aged↗
PubMed1994

Exteroceptive reflexes in dystonia: a study of the recovery cycle of the R2 component of the blink reflex and of the exteroceptive suppression of the contracting sternocleidomastoid muscle in blepharospasm and torticollis.

The recovery cycle of the R2 component of the blink reflex and the exteroceptive suppression of EMG activity in the contracting sternocleidomastoid muscle produced by electrical stimulation of the supraorbital nerve were studied in normal subjects and in patients with either blepharospasm or torticollis. The latencies of the reflexes were normal, suggesting that the neural structures that mediated them were intact. However, the recovery of the R2 component of the blink reflex was enhanced in patients with either blepharospasm or torticollis. Also, the size of the exteroceptive suppression of the sternocleidomastoid muscle was reduced in both groups of patients. Our results are indicative of abnormalities of interneurons mediating exteroceptive reflexes in patients with craniocervical dystonia that are not restricted to the systems controlling the muscle involved in the dystonia.

Adult↗
PubMed1998

Asymmetric predominantly ipsilateral blepharospasm and contralateral parkinsonism in an elderly patient with a right mesencephalic cyst.

A 66-year-old woman presented with a 3-year history of predominantly right-sided blepharospasm and a 1-year history of progressive predominantly left-sided hemiparkinsonism manifested by a left upper extremity resting tremor and left-sided bradykinesia. Magnetic resonance imaging of the brain revealed a large right mesencephalic cyst with mass effect. Positron emission tomography revealed bilateral striatal hypometabolism consistent with nigrostriatal dopaminergic dysfunction. The association of predominantly ipsilateral blepharospasm and predominantly contralateral hemiparkinsonism is very rare, and its association with a posterior fossa space-occupying lesion has been reported only once. This is the second report of such an association and the first description of adult-onset symptomatology.

Aged↗
PubMed1990

Saccadic eye movements in essential blepharospasm.

To provide evidence of an organic pathology for essential (idiopathic) blepharospasm, reflex saccadic eye movements in response to randomly stepped visual targets were assessed in seven affected patients and seven age-matched controls using the magnetic scleral search coil technique. The results indicate a significant prolongation in latency and a reduction in gain of horizontal saccades, and an increase in latency and reduction of peak velocity of large downward saccades. These findings suggest an organic component to the aetiology of blepharospasm, the pathology of which also appears to involve the oculomotor system. The pattern of the oculomotor disorder does not allow specific localisation but is consistent with the underlying pathology being localised in the basal ganglia.

Adult↗
PubMed1978

[Idiopathic blepharospasm. Clinical and electrophysiological investigations in 27 patients (author's transl)].

Idiopathic blepharospasm is reexamined and the differential diagnosis discussed on the basis of the signs and symptoms of 27 unpublished cases. As in tardive dyskinesia, middle-aged and elderly women are mostly affected. Contrary to general opinion, spontaneous improvement occurred in about one-fifth of the patients. The habituation of the orbicularis oculi reflexes (investigated electromyographically) was diminished in 52% of the patients. These and other symptoms indicate an organic cerebral lesion as the main cause of the disease. Similarities existing between blepharospasm and torticollis spasticus are discussed.

Basal Ganglia Diseases↗
PubMed1997

Frontalis suspension in the treatment of essential blepharospasm unresponsive to botulinum-toxin therapy: long-term results.

Thirty-one patients with essential blepharospasm or lid opening disorder of the levator-inhibiting type, unresponsive to treatment with botulinum toxin, underwent frontalis suspension. Twenty-eight patients received bilateral surgery (three patients with bilateral complaints of different severity were operated on the more affected side; these patients were not included in the statistical analysis). The mean age was 62.4 years +/- 8.52 (range 42-80 years). The individual improvement of complaints was assessed by the patients using a percentage scale (0% = no improvement; 100% = no complaints). Objective and subjective improvement was achieved in 26 of 28 patients. The mean subjective improvement was 57.7% +/- 31.4. In 23 cases an additional treatment with botulinum toxin was administered. During follow-up period (mean 22.1 months +/- 11.6; range 5-40 months) the effect of surgery remained stable. There were no serious complications, in a 5 of 56 operated eyes suture granuloma had developed. Unlike other surgeries for treatment of blepharospasm (excision of the orbicularis muscle, resection of facial nerve branches) frontalis suspension can be considered as a minimally invasive, but very effective and (if desired) reversible procedure. Moreover, additional treatment with botulinum toxin can bring about further improvement.

Adult↗
PubMed1985

Blepharospasm: a case study comparison of trihexyphenidyl (Artane) versus EMG biofeedback.

Blepharospasm is a chronic condition characterized by involuntary spasmodic closure of the eyelid(s). The present investigation empirically evaluated the relative efficacies of a current pharmacological and a psychological approach in the treatment of blepharospasm. An A-B-A-C-A single-case design was used to compare trihexyphenidyl [Artane] with EMG biofeedback. EMG activity in the frontalis muscle and number of eyeblinks were the dependent variables. The results indicated that Artane had no effect on either dependent measure and that biofeedback had a dramatic treatment effect on both dependent variables. Treatment gains were maintained at a 42-week follow-up. These results are discussed in light of the methodological considerations, the efficacy of psychological intervention, and the need for future research.

Biofeedback, Psychology↗
PubMed1988

Beneficial effect of botulinum A toxin in blepharospasm: 16 months' experience with 16 cases.

After introducing the problem of blepharospasm, we report our experience on treatment with purified botulinum A toxin in 16 cases of blepharospasm, symptomatic in two and essential in 14, than had not responded to drugs. The changes in intensity and frequency of spasm after treatment were evaluated on a clinical scale and by review of videotapes. The beneficial effect appeared within a week in most patients, lasting from 6 to 28 weeks (mean 13), and reached the maximum at the third-seventh week. Mild spasms and female patients responded better. Repeated injections were followed by better response to the drug. Complications, exclusively local, were represented by transient corneal exposure, ptosis, lacrimation or diplopia.

Aged↗
PubMed1990

Botulinum A toxin injection in patients with blepharospasm, torticollis and hemifacial spasm.

Botulinum A toxin was injected into the affected muscles in 20 patients with blepharospasm, 8 with torticollis and 12 with hemifacial spasm. In all cases blepharospasm and hemifacial spasm was abolished or markedly reduced. The only side effect was transient ptosis and diplopia. Patients with torticollis had a mild to moderate improvement of the dystonic posture and pain; dysphagia was the most troublesome side effect. Botulinum A toxin is an effective therapy in patients with focal dystonia and spasms.

Blepharospasm↗
PubMed2006

Long-term follow-up of patients with frontalis sling operation in the treatment of essential blepharospasm unresponsive to botulinum toxin therapy.

OBJECTIVE: Botulinum toxin is the treatment of choice in patients with essential blepharospasm, but about 4% of patients show no sufficient effect. Many of these patients try to open their eyes by innervating their frontalis muscle. This led to the idea of performing frontalis suspension, normally used for certain types of ptosis. We set out to evaluate the long-term results, complication rates and patient acceptance of this intervention. METHODS: Frontalis sling operation was carried out on 252 eyes of 132 blepharospasm patients between 1992 and 2004. In all patients botulinum toxin treatment was administered before surgery with no or only brief and incomplete effect even with increasing toxin doses. In 120 patients surgery was performed under local anaesthesia, while 12 patients were operated upon under general anaesthesia (mostly bilateral). Silk sutures were employed in the first 14 eyes, and in all others we used Gore-Tex suture material. RESULTS: The duration of follow-up was 3-154 months; 60 patients were followed up for at least 5 years. Seventy-three per cent of patients reported an improvement after surgery. Long-term subjective improvement showed a median of 50% on a scale ranging from 0%=no improvement to 100%=no complaints. No serious corneal complications occurred, although slight overcorrection is desirable in the first days after surgery for a satisfactory long-term result. Seven per cent of operations had to be revised due to suture granulomas or extruded suture material. The effect of surgery generally remained stable over the years, with most patients needing additional treatment with botulinum toxin. In cases of decreasing effect (5% of eyes), the sutures were tightened under local anaesthesia. CONCLUSION: Frontalis suspension can be considered as a minimally invasive but very effective and even reversible procedure in "poor responders" to botulinum toxin, with good long-term effect and good acceptance by the patients. Additional treatment with botulinum toxin is required in most patients in order to increase the desirable imbalance between the frontalis and the orbicularis muscle.

Adult↗
PubMed1985

Treatment of blepharospasm with botulinum toxin.

In 43 patients (81 eyes) with blepharospasm resistant to other forms of therapy, 149 outpatient injections of botulinum A toxin were given into the orbicularis oculi muscle. Subsequent follow-up periods ranged from ten to 210 days. Orbicularis oculi spasm, eyelid forced closure, and eyebrow spasm all decreased substantially over the first ten days following the initial injection. Patients with essential blepharospasm, hemifacial spasm, or previous surgery all responded in a similar fashion. This beneficial effect was transient, however, and additional treatment was required for sustained relief. Twenty-five patients received multiple injections with a mean interval of 65 days. Results of second and third injections were similar to the first in rapidity of onset and duration of effect. Complications were local, mild, and transient, and no systemic side effects were noted.

Adult↗
PubMed1993

Auditory brain-stem responses in blepharospasm.

The auditory brain-stem response (ABR) has been reported to detect abnormalities in both the auditory pathways and in adjacent structures. Ten of 35 consecutive patients with blepharospasm were found to have abnormal ABRs involving poor form and delayed peak latency of positive components III or V. Abnormal ABRs in approximately 30% of patients with essential blepharospasm suggest pathology in the brain-stem of a substantial proportion of patients with this form of cranial-cervical dystonia.

Acoustic Stimulation↗