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Focal dystonias are relatively rare and significantly disabling disorders. These include cervical dystonia, blepharospasm and hemifacial spasm. The spasmodic torticollis consists of tonic posturing of the head away from its neutral position or twisting of the cervical muscles. The blepharospasm is an abnormal blinking, eyelid tic or twitch resulting from any cause. The hemifacial spasm is an involuntary unilateral twitching of the facial muscle. Patients affected by focal dystonias are predominantly females, and many times psychical stress can be revealed. The pathogenesis may involve dysfunction of the basal ganglia and brain stem although the exact mechanism remains to be elucidated. The patients need to be diagnosed and treated in centers specialized in movement disorders. Although many drug treatments can be beneficial, the most effective treatment is the local Botulinum toxin injection into the affected muscles. This neurotoxin produces temporary neuromuscular blockade, which reveals the symptoms and pain. The effect of the toxin is temporary and, therefore, the injection needs to be repeated every 6-12 weeks. The most common side effects are hypersensitivity, bleeding, hematoma, ptosis, facial spasm, dysphasia or dysarthria. With the use of proper dose and injection sites these side effects can be avoided.
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PMID:[Clinical symptoms, diagnosis and treatment of focal dystonias]. 1176 Jun 45

Superior limbic keratoconjunctivitis results mechanically from blinking under prolonged unphysiological conditions. The pathogenic process is known as blink-related microtrauma. This review aimed to explore the validity of a general theory that besides superior limbic keratoconjunctivitis, there may be other diseases of the ocular surface arising from mechanical microtrauma. A review of relevant clinical and microscopic lesions in a range of ocular surface disorders with possible mechanical aetiology was conducted. New terms were selected to facilitate understanding of such new aetiology. Besides superior limbic keratoconjunctivitis, other ocular surface disorders regarded as primarily derived from blink microtrauma are: other filamentary keratitides; blepharospasm and severe ptosis; canthal/palpebral froth; affections from disordered eyelid lining; and contact lens related damage. A group of secondarily microtraumatic disorders was identified, including the example of microtrauma impacting upon interpalpebral bulbar prominences. Superior limbic keratoconjunctivitis is the archetype of diseases affecting a unique combination; namely, the ocular surface conjoined with its lacrimal fluid. It is only one among many diseases actively generated within the confines of 'a self-harming surface'.
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PMID:Blink-related microtrauma: when the ocular surface harms itself. 1278 67

Dystonias are frequently observed in Parkinson's disease or other parkinsonian syndromes. They can occur during off-periods, either in the morning (early morning dystonia) or during daily off-periods, and during on-periods. Dystonia involves more frequently the upper and lower limbs, the neck or the face. Dystonia can be painful in particular off-period feet dystonia. The mechanisms underlying dystonia are not fully understood, basal ganglia activity and levodopa levels seems to play an important role. There are several medical options to try and improve those dystonias, adjustment of levodopa doses, adding a dopamine agonist drug, anticholinergics, lithium, baclofene or clonazepam. Those options are not always very effective. Botulinum toxin injections are an alternative treatment for focal dystonia. Muscles have to be selected by observation of the dystonia. Deep muscles in particular in the legs can be injected under EMG guidance. Botulinum toxin injections are particularly helpful and safe for lower limb dystonia. They can be used also for other forms of dystonia. Upper limb dystonia can be injected, allowing more comfort and easier hygiene but not necessarily better function, weakness is the main side effect. Cervical dystonia, blepharospam and oromandibular dystonia can be managed the same way as idiopathic dystonia. The dose might be lower since the muscles are usually not as hypertrophic. Side effects are as expected dysphagia and neck weakness in case of cervical dystonia, ptosis, inocclusion and diplopia in case of blepharospasm, jaw opening difficulty with oromandibular dystonia. Basal ganglia surgery can also help dystonia in a selected population of parkinsonian patients.
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PMID:[Parkinsonian dystonia]. 1461 83

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.
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PMID:[Blepharospasm in a patient with thymoma and positive anti-acetylcholine receptor antibody]. 1465 4

Botulotoxin A (BT) was administered to 51 patients (40 patients with essential blepharospasm, 11 patients with facial hemispasm) 299 times since December 1993 till January 2002. Total doses of 20-48 U of the preparation BOTOX (Allergan Inc.) were administered bilaterally in essential blepharospasm, doses of 12-24 U were administered unilaterally in facial hemispasm. The mean duration of therapeutic effect evaluated by the patient was 12.2 weeks (3-28 weeks) in the blepharospasm group and 13.8 weeks (7-35 weeks) in the hemispasm group. No general side effects were noted. Local complications in the blepharospasm group were observed in 8.83% (ptosis in 5.62%) and those in the facial hemispasm in 8.16% (ptosis in 2.04%) from the total number of BT administration in the respective groups. The therapeutic effect during the long-term administration was decreasing and required increasing doses of BT by more than 10%, as observed in 9 patients with essential blepharospasm (22.5%). The mean increase of BT dose required to maintain sufficient therapeutic effect was by 32% (14-66%). Two patients with the longest 8-year period of observation have not required increasing dosages above 16 U unilaterally over the last 5 years. The treatment with BT-A exerted the same effects in most cases even during long-term therapy lasting several years. The need to increase the doses of the administered BT was more frequent during the first two years of the treatment. Locally administered botulotoxin A is a safe and efficient remedy for the therapy of essential blepharospasm and facial hemispasm, the efficiency being unchanged in more than 80% of patients.
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PMID:[Results of long term treatment of essential blepharospasm and facial hemispasm with botulinum toxin A]. 1501 5

Although the relative potency measured by the number of units per nanogram of the toxin is different for the three preparations (BOTOX = 20 U/ng; Dysport = 40 U/ng, and CS-BOT = 15.2 U/ng), the effective dose for CS-BOT is similar to that of BOTOX (Allergan, Irvine, CA). Despite the twofold difference in potency per nanogram, it appears that the clinically observable activity of 1 U of BOTOX is roughly equivalent to 3 U of the Dysport (Inamed, Santa Barbara, CA) product. Using quantitative analysis of regional paralysis produced by local injections into the gastrocnemius muscles of mice, prior studies estimated the potency ratio between Dysport and BOTOX to be 4.2 to 1. In a single-blind, randomized comparison study of Dysport and BOTOX in 91 patients with blepharospasm or hemifacial spasm, it was found that 4:1 dose ratio produced similar benefits. A similar 4:1 Dysport:BOTOX ratio was found to produce equivalent beneficial effects in a double-blind study in patients with blepharospasm, but the frequency of side effects, particularly of ptosis, was lower in the BOTOX group. In a study of 73 patients with cervical dystonia treated either with Dysport or BOTOX, it was concluded that a 3:1 ratio provides equivalent results. But a recent study concluded that the appropriate conversion factor between BOTOX and Dysport is less than 3. Therefore, there is some controversy about the relative potencies of the two preparations, with one study proposing that 1 unit of BOTOX corresponds to 1 unit of Dysport.
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PMID:Pharmacokinetic properties of different formulations of botulinum neurotoxin type A. 1502 57

The eyelid movements are mediated mainly by the orbicularis oculi (OO) and the levator palpebrae superioris (LPS) muscles. Dissociated upper lid functions exhibit different counterbalanced action of these muscles, and in blinking they show a strictly reciprocal innervation. The disturbance of this close LPS-OO relationship likely leads to many of the central lid movement disorders. Three groups of supranuclear motor impairment of lid movements are considered: the disorders of the lid-eye movements' coordination, the disturbances of blinking and lid "postural" maintenance, and the alteration of voluntary lid movements. Nuclei of the posterior commissure control the inhibitory modulation of LPS motor-neuronal activity and they are involved in the lid-eye coordination disorders such as lid retraction, which is observed in the Parinaud's syndrome and also in parkinsonism and progressive supranuclear palsy. Spontaneous (SB) and reflex blinking consist of two components: the inhibition of the basal tonic LPS activity, which keeps the eyes open, and the concurrent activation of the OO muscles. LPS inhibition precedes and outlasts the OO activation. This normal configuration is impaired in parkinsonism and blepharospasm (BSP). SB shows a highly interindividual rate variation (among 10-20 per minute in adults) and abnormal blink rates occur in neurological diseases related to dopaminergic transmission impairments. Lid postural abnormalities include involuntary eyelid closure, which is usually associated with inability to open the eyes. Two major disorders share these two aspects: BSP and blepharocolysis (BCO). BSP consists of an involuntary overactivity of the OO, with LPS co-contraction activity, and is expressed as frequent and prolonged blinks, clonic bursts, prolonged tonic contraction or a blend of all of them. BCO (commonly named "so-called lid opening apraxia") is an overinhibition of the LPS with no evidence of ongoing OO activity. BSP and BCO occur in many instances of idiopathic dystonias and basal ganglia diseases and, less frequently, in rostral brainstem lesions. Both may coincide in the same patient. Voluntary lid movement disorders comprise the impairment of Bell's phenomenon, the voluntary eyelid closure palsy and the so-called cerebral ptosis, all related to lesions of frontal cortical areas and/or the corticospinal system.
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PMID:Eyelid movements in health and disease. The supranuclear impairment of the palpebral motility. 1503 Jul 96

We evaluated our results of advancement of the frontalis muscle to correct brow ptosis associated with blepharospasm in five patients who had difficulty opening their eyelids as a result of ptosis even after injections of botulinum toxin. The frontalis muscle was retracted inferiorly and connected directly to the skin of the eyebrow. Postoperatively the level of the eyebrow was raised above the superior orbital rim in all cases during the observation period (4 to 15 months). Although the operation did not improve muscle spasms, it successfully shortened the duration of involuntary closure of the eyelid. The only postoperative complication was lymphoedema of the eyelids. Results of postoperative injection of botulinum toxin were satisfactory. Advancement of the frontalis muscle corrects brow ptosis without major complications, and is complementary to injection of botulinum toxin.
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PMID:Advancement of the frontalis muscle for ptosis of the brow associated with essential blepharospasm. 1520 67

This study was a Phase IV, prospective, one arm, non-comparative open trial, to investigate the efficacy and safety of Dysport (Botulinum toxin type A) in patients with idiopathic blepharospasm or hemifacial spasm. During the treatment period, patients were evaluated at baseline (week 0), week 6, and week 8, 10, or 12. Thirty two women and 16 men completed the whole course of the study. The therapeutic efficacy of Dysport became evident from 1.5 to 15 days (mean+/- SD, 6.1 +/- 2.9 days). The maximal effect appeared 12.2+/-5.0 days later. Injection of Dysport achieved 72.9 (13.0% amelioration in the spasm symptom. Dysport significantly improved the following functions, such as reading, watching TV, house work, working, driving and outing alone. At the twelfth week after Dysport injection, it was still effective in relieving blepharospasm or hemifacial spasm. The most frequent adverse event was ptosis, which was noted in 9 cases and represented 18.7% of total patients. Other adverse events were very mild, although lagophthalmos and dry eyes occurred in some patients, but none manifested any corneal complications. In conclusion, Dysport injection appears to be a safe, and effective procedure - accompanied only by minor, and transit adverse events.
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PMID:Quantitative assessment of efficacy of dysport (botulinum toxin type A) in the treatment of idiopathic blepharospasm and hemifacial spasm. 1600 64

A 6-year-old boy with Schwartz-Jampel syndrome and severe myotonia-induced blepharospasm and ptosis did not respond to botulinum toxin A injections in the orbicularis muscle. The clinical diagnosis was further supported by electromyography. Surgical management using a combination of techniques in one operation produced a satisfactory result for both function and appearance. Muscle biopsy was also done during surgery.
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PMID:Schwartz-Jampel syndrome: surgical management of the myotonia-induced blepharospasm and acquired ptosis after failure with botulinum toxin A injections. 1641 70


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