Gene/Protein Disease Symptom Drug Enzyme Compound
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One hundred patients (9.09 per cent) with blepharospasm from a grand total of 1,100 patients (at the Movement Disorders Clinic at Siriraj Hospital) who had been treated with botulinum A toxin (BTX-A) injection between 1989 and 1996 were analysed. The 100 patients comprised 65 females and 35 males with a female to male ratio of 1.86:1. Their mean age was 53.3 years (S.D. 12.03). Sixty patients had idiopathic blepharospasm, 31 patients were diagnosed with Meige syndrome (blepharospasm plus oromandibular dystonia) and nine patients with segmental dystonia (Meige syndrome plus cervical dystonia). The mean duration of suffering was 39.22 months (S.D. 44.83). Each patient received 30-50 IU of BTX-A injections according to the standard Siriraj injection pattern. Nine patients were lost to follow-up. The results of BTX-A injection were classified as: excellent result (an improvement of more than 75 per cent) in 83.51 per cent; a good response (an improvement of 50-75 per cent) in 13.19 per cent; a moderate response (an improvement of 25-50 per cent) in 2.20 per cent; and minimal or no response in 1.10 per cent. The complications of BTX-A injection were transient minimal ptosis (9.89 per cent), transient double vision (1.10 per cent) and excessive lacrimation (1.10 per cent). The efficacy of BTX-A injection lasted one to two months in 1.10 per cent, two to three months in 23.08 per cent, three to four months in 45.05 per cent, four to five months in 16.48 per cent, five to six months in 4.40 per cent and more than six months in 9.89 per cent. Botulinum A toxin injection is a simple and effective out-patient treatment for patients with blepharospasm, causing no systemic side-effects and minor transient local complications. The only drawback of this treatment is its high cost (100 IU cost 300 US dollars).
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PMID:Botulinum A toxin treatment for blepharospasm and Meige syndrome: report of 100 patients. 907 10

A double-blind study was performed on 212 consecutive patients (58 men, 154 women) with essential blepharospasm, who received one injection of Botox and one injection of Dysport in two separate treatment sessions (at the first session the patients randomly received one of the drugs, at the second the other drug was given. The patients' mean age was 66.4 years +/- 8.14 (range 39-86 years). The average dose of Botox per treatment was 45.4 IU +/- 13.3 (range 25-85 IU) and of Dysport 182.1 IU +/- 55.1 (range 100-340 IU). We used an empirical ratio Botox:Dysport of 1:4 (IU) in order to ensure equal doses. All patients had received botulinum toxin injections prior to the present study (mean 15.3 injections +/- 9.4; range 1-43 injections). The effect of Botox lasted 7.98 weeks +/- 3.8 (range 0-16 weeks), while the effect of Dysport lasted 8.03 weeks +/- 4.6 (range 0-22 weeks). Side effects (ptosis, tearing, blurred vision, double vision, hematoma, foreign body sensation) were observed with Botox in 36 of 212 (17.0%) of the treatment sessions and with Dysport in 51 of 212 sessions (24.1%). Ptosis was observed with Botox in 3 cases (1.4%) and with Dysport in 14 cases (6.6%). There was no statistically significant difference in the duration of the treatment effect between the two preparations (P = 0.42). The total number of side effects was lower with Botox than with Dysport; the significance of the difference was moderate (P < 0.05). However, the rate of occurrence of ptosis was significantly lower with Botox (P < 0.01). The bioequivalence, which varies between 1:3 and 1:6 (Botox:Dysport) in the literature, was found to be 1:4 in this study.
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PMID:Comparison of two botulinum-toxin preparations in the treatment of essential blepharospasm. 914 85

In a series of 33 blepharospasm patients who had the side effect of ptosis following therapeutic botulinum toxin type A (Botox: Allergan, Inc., Irvine, CA, U.S.A.) injection, we administered 41 injections of human botulinum immune globulin (IG) following injections of the toxin to test the dosage and timing of IG injection and its effectiveness in limiting or avoiding ptosis. An IG dose of 3.2 x 10(-3) international units (IU) per unit of Botox was effective in blocking toxin effect when injected into the same tissue site within 4 hours. An IG dose of 1.6 x 10(-2) to 3.2 x 10(-2) into the levator of the eye having more frequent ptosis in 19 patients reduced the incidence of ptosis to 11%. The fellow (control) eye had a ptosis incidence of 37%. No orbital hemorrhage or other adverse effect occurred from the IG or its injection.
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PMID:Preventing ptosis after botulinum treatment. 918 90

Blepharospasm is form of focal dystonia characterized by involuntary and repetitive contractions involving the orbicular oculi muscle. Fourteen patients with blepharospasm were submitted to botulinum toxin type A injections. Of these 14 cases, 6 were male and 8 female. The mean age was 58 years (range: 33 to 76 years) with illness duration ranging from 1 to 25 years (average: 7.3 years). All patients have been treated with conventional therapies with poor results, but presented a good response to botulinum toxin type A. The average latent period was 3 days (range: 0 to 10); maximum effect of treatment was observed after 16 days. The mean duration of maximum response was 90 days. The main side effects of botulinum toxin injections were ptosis and lagophtalmos.
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PMID:[Blepharospasm: treatment with botulinum toxin]. 923 94

This investigation was designed to extend our present knowledge of the supraorbital n. (SO n.) distal to the supraorbital notch. It is based on 40 dissected hemi-faces and the position of the notch and the periosteal and frontalis cutaneous branches of the SO n. were studied. The notch was 33.05 mm from the midline on the right side and 30.70 mm on the left. The periosteal branch arises from the lateral frontalis cutaneous branch. Its ascends in an oblique direction laterally and ends in two terminal branches. The frontalis cutaneous branch, after a very short trunk, divides into two branches, medial and lateral. The medial or deep branch enters the corrugator supercilii m. between its fibers. Most frequently, it passes under the inferior fasciculus and superficial to the middle and superior ones. Leaving the corrugator m., it ascends medially into the frontalis m., supplying the median cutaneous frontalis region. The lateral or superficial branch crosses superficial to the corrugator supercilii m. to penetrate the frontalis m. in an ascending and lateral direction, supplying the lateral frontalis region. The two branches enter the frontalis m., displaying a zigzag pattern in order to adapt its length during expressive movements. They cross the frontalis region together with the SO a. and two veins supplying the nerve and the frontalis m. These anatomic data may explain some of the complications after surgery for ptosis and blepharospasm.
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PMID:Anatomic study of the distal supraorbital nerve. 947 12

A case that combines cerebral eyelid ptosis and blepharospasm secondary to cerebral hemisphere infarction is shown. EMG recording from the facial and eyelid muscles revealed lack of the levator palpebrae superioris, orbicularis oculi and corrugator muscles activity. Any voluntary opening attempt lead to a simultaneous contraction of the three muscles. Blink reflex responses were normal although showed facilitation features on the right side and mild inhibition characteristics from the left side. Median nerve SEP revealed a loss of precentral components (P22-N30) as well as a delay and amplitude decrease of N20. Transcranial magnetic stimulation disclosed a complete lesion of corticospinal pathway for right upper limb. In this case, a right hemisphere lesion caused an unusual eyelid motor abnormality: cerebral eyelid ptosis and blepharospasm induced by the voluntary eyelid opening.
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PMID:[Palpebral ptosis and blepharospasm secondary to hemispheric cerebral infarction]. 952 84

Botulinum toxin A is the more efficient therapy of focal dystonias and hemifacial spasm. Our experience with botulinum toxin A injections in 115 patients is reported. Marked or total improvement was achieved in all 45 patients with hemifacial spasm, in 70% of 20 patients with essential blepharospasm and in 71.4% of 14 patients with Meige's syndrome. In 65.2% of 23 patients with cervical dystonia marked but no total improvement was obtained. The worse results were seen in the 6 patients with hand dystonia (writers cramp), in whom marked improvement was obtained in just two. Mild and transient complications occurred in up to 24.4%, eyelid ptosis and eyelid weakness being the most frequent. One patient with Meige's syndrome had an aspiration pneumonia following dysphagia. Our results are in agreement with others, showing that botulinun toxin A is a useful and safe treatment for these conditions.
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PMID:[Botulinum toxin A: experience in the treatment of 115 patients]. 962 4

Schwartz-Jampel syndrome is a rare disorder with prominent palpebral fissure changes. These changes are complex and have been described as ptosis, blepharophimosis, and blepharospasm. Two new cases of the Schwartz-Jampel syndrome and the results of elevating the upper eyelid margin with levator aponeurosis surgery are presented, along with a review of the literature.
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PMID:Levator aponeurosis surgery in Schwartz-Jampel syndrome. 970 Jul 36

Twenty seven patients with hemifacial spasm (HFS) and sixteen patients with blepharospasm (BS) having mean Jankovic disability rating scale score of 2.56+0.58 SD and 2.81+0.54 SD, respectively, were treated with botulinum toxin A (BTX-A) injections. The total number of injection sessions were ninety one with relief response in 98.91%. The mean improvement in function scale score was 3.78+0.64 SD and 3.29+1.07 SD respectively, in HFS and BS groups. The clinical benefit induced by botulinum toxin lasted for a mean of 4.46+3.11 SD (range 2 to 13) months in HFS group and 2.66+1.37 SD (range 1 to 6) months, in BS groups. Transient ptosis was seen in 4.39% of total ninety one injection sessions. These findings show that local botulinum toxin treatment provides effective, safe and long lasting relief of spasms.
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PMID:Botulinum toxin treatment of hemifacial spasm and blepharospasm: objective response evaluation. 1051 80

A 66-year old man with blepharospasm and ptosis of the brow was treated with a combined procedure in which the branches of the facial nerve were excised at the margin of the orbicularis, and the periorbital area was reconstructed simultaneously. The condition improved after the treatment with no occurrence of either oral complications or facial anaesthesia.
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PMID:Superselective neurectomy with periorbital primary reconstruction for blepharospasm. Case report. 1102 Sep 26


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