Epiteliopatía en limpiaparabrisas en pacientes con blefaroespasmo o espasmo hemifacial

ObjectiveTo evaluate the presence of wiper epitheliopathy in patients with blepharospasm and/or hemifacial spasm before and 4 weeks after routine treatment with botulinum toxin.MethodsProspective study comprising 31 eyes of 20 patients with neurological diagnosis of hemifacial spasm (9 eyes of 9 patients) and essential blepharospasm (22 eyes of 11 patients). Various ocular surface parameters were assessed before and 4 weeks after infiltration with botulinum toxin using the OSDI questionnaire, Schirmer's test, tear break-up time, fluorescein and lissamine green staining assessed with the Oxford test and the degree of involvement of the palpebral wiper.Results100% of the patients had palpebral wiper involvement before (30% mild and 70% moderate) and after toxin treatment (100% mild). 75% of patients had mild-normal OSDI before treatment, after treatment it was 80%. The tear break-up time was 7.2 ± 0.2 sg before and 7.5 ± 0.7 sg after treatment. Schirmer's test was 11.4 ± 5.5 and 12.5 ± 5.5 mm before and after treatment. The Oxford test was initially pathological in 69.3% of patients, after 4 weeks it was pathological in only 54%.ConclusionWiper epitheliopathy is present in 100% of patients with blepharospasm and/or hemifacial spasm. The main pathophysiological mechanism that triggers it in these patients is the increase in the coefficient of friction, as tear volume and stability are normal.     

Sensitization to d-amphetamine after its repeated administration: evidence in EEG and behaviour

We investigated the efficacies and potencies of two commercial preparations of botulinum neurotoxin type A (BoNt/A) reputed to differ in potency. Tests were conducted in vitro using the mouse phrenic nerve-hemidiaphragm which is an approved tool for measuring clostridial toxicity. In addition, in a double-blind trial on volunteers, varying amounts of one product were injected into the Musculus extensor digitorum brevis of the left foot, while equal amounts, i.e. units, of the other preparation were injected into the same muscle of the right foot. Compound muscle action potentials (CMAPs) were recorded before and at various points in time after the injections. As opposed to wide-spread anecdotal reports, no difference in effectiveness was found. The dose-response curves obtained from the mouse organ preparation with both commercial products equalled one another in potency (number of units) and corresponded to previous toxicity tests in mice conducted elsewhere. Dose-response curves from volunteers were also identical for both commercial preparations. The time course of paralysis and recovery of muscle function did not differ either. At lower concentrations of toxin, however, restoration of muscle function was more rapid than at higher concentrations. Since the results obtained from man and the animal organ preparation are in excellent accord, we conclude that 1 unit of Botox corresponds to 1unit of Dysport. Received: 26 September 1996 / Accepted: 11 November 1996     

Relationship between various clinical outcome assessments in patients with blepharospasm

The objective was to analyze the metric properties of the Jankovic Rating Scale (JRS) and a self‐rating patient response outcome scale, the Blepharospasm Disability Index (BSDI©), in blepharospasm patients. Data from a randomized, double‐blind, active‐control clinical trial in 300 patients with blepharospasm treated with either botulinum toxin type A (Botox®) or NT201 (Xeomin®) were used to evaluate the metric properties of the JRS and the BSDI compared with the Patient Evaluation of Global Response (PEGR) and Global Assessment Scale (GAS). The internal consistency of the BSDI was high, Cronbach's Alpha = 0.88, and the retest reliability of the BSDI single items was adequate, Spearman's rank coefficient = 0.453 < r < 0.595. The correlation between JRS sum score and BSDI weighted mean score was r = 0.487 (baseline) and r = 0.737 (control visit), respectively. Using the GAS and PEGR, the results suggest that a change of 2 points in the JRS and of 0.7 points in the BSDI are clinically meaningful. JRS and BSDI are objective indicators of clinical efficacy as suggested by their good validity when compared with physicians' and patients' rating scales. Both, JRS and BSDI, can be used to reliably assess blepharospasm in treatment trials. © 2008 Movement Disorder Society     

Surface electromyographic study of idiopathic cranial dystonia focused on the orbicularis oculi muscles

We studied 115 Japanese patients with idiopathic cranial dystonia (Meige disease), using surface electromyographu (EMG) focused on the orbicularis oculi muscles to classify the findings of the abnormal involuntary movements of this disease and to evaluate the pathophysiology of blepharospasm (BS). Surface EMGs at rest and at voluntary eyelid opening after eyelid closing were investigated. We found 62 (53.9%) patients exhibiting the overblinking type, 37 (32.2%) the tonic BS type, and 16 (13.9%) the normal type of behavior, considering the frequency of spontaneous blinking and presence of spasms. The present results suggest that BS is not a summation of blinking but a spatial and temporal extension of the orbicularis oculi muscle activity engaging in blinking, and the classification of the present study can support the investigation of the temporal characteristics of patients with this disease.     

Facial dystonia: clinical features, prognosis and pharmacology in 31 patients

The natural history and response to different treatments were assessed in 31 consecutive patients with blepharospasm (BS) and/or oromandibular dystonia (OMD). The mean age at onset was 52.4 years and there was a female preponderance of 2.5 to 1. Ocular symptoms preceded the onset of blepharospasm in more than 50% of the affected patients, whereas psychiatric and dental problems prior to the onset of focal dystonia were found in 10% and 13% of the cases respectively. Dystonia elsewhere, mainly in the craniocervical area, was found in 23% of patients and appeared to follow a somatotopic progression. The first 2–3 years of history were crucial for the spread of dystonia to other face and body parts. When OMD was the first symptom, a lower tendency of dystonia to progress elsewhere was observed. A putative cause was found in 14% of patients who showed clinical and radiographic evidence of basal ganglia or rostral brainstemdiencephalon lesions. The response to different drugs was inconsistent although transient improvement was induced by haloperidol in 6 patients, by L-Dopa plus deprenyl in 3 patients, by trihexyphenidyl in 2 patients and by clonazepam in 2 patients. One, apparently spontaneous, remission was observed. Botulinum A toxin was iniected in the orbicularis oculi of 8 patients affected by BS: moderate to marked improvement lasting 5 to 30 weeks (mean 14.5 weeks) was achieved in all cases; transient ptosis, lasting 1 to 3 weeks, occurred in 3 cases.

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Sommario La storia naturale e la prognosi della distonia faciale sono state valutate in una serie di 31 consecutivi pazienti con blefarospasmo (BS) e/o distonia oromandibolare (OMD) (età di esordio: 19–75 anni; durata di malattia: 1–15 anni; rapporto maschi/femmine: 2.5/1). Sintomi oculari precedevano l'insorgenza del BS in oltre il 50% dei pazienti, mentre anomalie dentali e problematiche psichiatriche comparivano come prodromi nel 10% e nel 13% dei casi rispettivamente. La sintomatologia distonica diffondeva, con andamento somatotopico, oltre il distretto cranio faciale nel 23% dei casi. Evidenze cliniche o radiologiche di lesioni dei gangli della base, della parte rostrale del tronco dell'encefalo o del diencefalo erano presenti solo nel 13% dei casi. Un lieve ma transitorio miglioramento della sintomatologia distonica era indotto da aloperidolo in 6 pazienti, da 1-dopa+deprenyl in 3, da triesifenidile in 2 e da clonazepam in 2. Solo 1 paziente andava incontro a remissione apparentemente spontanea della sintomatologia distonica dopo un anno di malattia. L'iniezione di tossina botulinica di tipo A negli orbicolari delle palpebre di 8 pazienti con BS induceva un sensibile miglioramento della sintomatologia distonica che persisteva per 5–30 settimane (media 14.5 settimane) con ridotti effetti collaterali locali (transitoria ptosi in 3 casi).

     

Comparison of preseptal and pretarsal injections of botulinum toxin in the treatment of blepharospasm and hemifacial spasm

Although the beneficial effect of subcutaneous injections of botulinum toxin type A (BTX-A) is well known in both blepharospasm and hemifacial spasm, the position of the injection sites around the orbicularis oculi may influence the effectiveness and side effects. Here we report results of preseptal and pretarsal BTX-A injections in 53 patients (25 blepharospasm and 28 hemifacial spasm) in whom we used both injection techniques successively. Pretarsal injections were used in 102 out of 186 treatments in blepharospasm group and in 84 out of 202 treatments in hemifacial spasm group. Pretarsal BTX-A treatment produced significantly higher response rate and longer duration of maximum response in both patient groups. This technique was also associated with a lower frequency of major side effects such as ptosis. We concluded that injections of BTX-A into the pretarsal, rather than the preseptal portion of the orbicularis oculi is more effective for treatment of involuntary eyelid closure due to contractions of this muscle. Received: 15 January 2001, Received in revised form: 11 May 2001, Accepted: 17 May 2001     

9 Mb deletion including chromosome band 3q24 associated with unsuspicious facial gestalt, persistent ductus omphaloentericus, mild mental retardation and tic

9 Mb deletion including chromosome band 3q24 associated with unsuspicious facial gestalt, persistent ductus omphaloentericus, mild mental retardation and tic.     

BOTULINUM TOXIN — THE RATIONALE OF ITS USE

Treatment with Oculinum, (Botulinum toxin, Type A), may induce three possible effects. These are a pharmacologic inhibitory effect, a mechanical effect and a sensory effect.     

A型肉毒毒素注射治疗锥体外系疾病合并眼睑痉挛的疗效观察

目的:探讨A型肉毒毒素(Bo NT/A)注射治疗锥体外系疾病合并眼睑痉挛(伴或不伴睁眼性失用)的疗效。方法:30例锥体外系疾病(19例帕金森病、5例多系统萎缩、6例进行性核上性麻痹)合并眼睑痉挛的患者局部注射Bo NT/A,在治疗前和治疗后1个月采用Jankcovic评定量表(JRS)、眼睑痉挛残疾指数(BSDI)评估眼睑痉挛改善效果,视觉模拟评分法(VAS)让患者自评疗效,自评改善大于50%定义为治疗有效。结果:治疗后1个月患者JRS-F(1.43±0.68)分、JRS-S(1.23±0.94)分、JRS-T(2.53±1.46)分、BSDI(1.33±0.91)分,均较治疗前[JRS-F(2.47±0.90)分、JRSS(2.70±0.88)分、JRS-T(5.30±1.68)分、BSDI(1.95±0.83)分]明显降低,差异均有统计学意义(均P     

Alcohol with xylocaine for treatment of eyelid dystonia.

Eyelid dystonia represents a form of idiopathic torsion dystonia, and it is considered as a rare disorder. Although botulinum toxin injections into the eyelids and eyebrows are considered by many neurologists as the treatment of choice for this, its cost limit its use in our community. A study was conducted at the King Hussein Medical Center between January 1995 and January 1998 using 1-2 ml of 0.5% xylocaine added to 99.5% ethanol in one-tenth of the volume of xylocaine, made injectable into the eyelids and eyebrows muscles in patients with blepharospasm. The treatment was aimed at reducing muscle spindle afferent activity. Twenty-one patients were given this modality of treatment in at least 8-12 separate sessions, with two weeks elapsing between any two sessions. Mild to moderate improvement was observed in 47.6% of our patients. The average latency from the time of the injection to the onset of improvement was 30-60 min and the average duration of improvement was 5-7 days. The commonest encountered side-effect was bruising at the site of injection that resolved spontaneously in less than 5 days. It is obvious that the usefulness of this treatment is limited by the substantially shorter duration of benefit, thus it cannot be considered as an alternative to Botox treatment. It may be useful to investigate whether a higher alcohol concentration is more effective.