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PubMed1998

Long-term efficacy of local doxorubicin chemomyectomy in patients with blepharospasm and hemifacial spasm.

OBJECTIVE: This study examines the long-term follow-up of all patients treated with doxorubicin injections in the eyelids. DESIGN: Nonrandomized clinical trial. PARTICIPANTS: Eighteen patients with blepharospasm (12 female; 6 male) and nine patients with hemifacial spasm (4 female; 5 male). INTERVENTION: Eyelids were repeatedly injected at intervals of 10 or more weeks until the spasms were ameliorated or the patient requested discontinuation. MAIN OUTCOME MEASURE: Clinical "cure" defined as sufficient symptomatic relief to defer further paralytic treatment. All patients have been followed for more than 1 year since the last injection. RESULTS: Nine of 18 patients with blepharospasm completed the full course of treatment and are considered "cured" for more than 1 year (median, 3 years; maximum, 6 years). Six of nine patients with hemifacial spasm completed treatment. Five of these six patients are considered "cures," lasting for more than 4.5 to 6 years. Two additional patients, one with blepharospasm and one with hemifacial spasm, had significant amelioration and were untreated for more than 3 years after the last doxorubicin injection, but occasionally request botulinum toxin supplementation. The minimum effective dose per treated eyelid ranged from 1.0 to 4.2 mg (median, 2.25 mg). The treatment-related discontinuations and complications were related to skin inflammation. Four of the 14 "cured" patients required some surgical "touch-up" on 1 eyelid. However, all the patients who completed treatment are either cured or have had significant amelioration of symptoms. CONCLUSIONS: Doxorubicin chemomyectomy is an evolving technique and an effective treatment for essential blepharospasm and hemifacial spasms symptomatically localized to the eyelids. Sixteen (59%) of the initial series of 27 patients completed the treatment. Of these, all are apparently cured or their symptoms significantly ameliorated. In the future, an even higher proportion would be expected to complete the treatment due to improvements in the selection criteria and treatment protocols developed during this 8-year trial. While the treatment appears to be reasonably safe compared with surgical myectomy in its present form, the authors are continuing to explore and introduce additional cotreatments to minimize the acute skin changes and maximize the long-term effectiveness of the myectomy.

Aged↗
PubMed1988

The significance of ophthalmological symptoms in idiopathic blepharospasm.

Two hundred and seventy-two patients with idiopathic blepharospasm were reviewed to establish the role of local eye disease in their illness. The majority of patients (57%) had symptoms at the onset of their illness such as dryness of the eyes, grittiness, irritation or photophobia suggesting eye lid or ocular surface disease. Detailed ophthalmological examination at the time of presentation had been carried out in 170 of the 272 cases; 64% of these patients had ocular symptoms, and 40% had demonstrable ocular surface or eye lid pathology. Such pathology was usually bilateral, chronic and resistant to local treatment. Blepharospasm developed in these patients after a long latent period, often of many years. Unilateral pathology was acute, normally responded well to local treatment, but was followed by the development of bilateral blepharospasm usually within six months. Amongst all 272 patients, those without ocular symptoms at presentation rarely developed them subsequently; if they did, there were no abnormal signs. The data suggest that ophthalmological disorders may trigger idiopathic blepharospasm in a substantial proportion of cases predisposed to develop this condition.

Adolescent↗
PubMed1994

Electromyographic features of levator palpebrae superioris and orbicularis oculi muscles in blepharospasm.

Electromyographic (EMG) recording was performed synchronously from the levator palpebrae superioris (LP) and the orbicularis oculi (OO) muscles in 28 patients referred to us for treatment of blepharospasm with botulinum A toxin. At the time of this study, 19 patients were under the treatment with botulinum, four started treatment shortly after the EMG recording and five patients had not yet been treated. Based on the EMG patterns, we were able to classify five major groups of abnormalities. Group 1 (blepharospasm): consisted of 10 patients with dystonic discharges limited to OO, normal LP tonic activity, intact reciprocal inhibition between LP and OO and dense bursts of action potentials with high amplitude preceding the return of LP tonic activity, i.e. 'postinhibition potentiation' of LP, brought about by a brief contraction of OO. Group 2 (combined dystonic activities of LP and OO): seven patients belonged to this group. The EMG recording revealed alternating tremulous discharges in both LP and OO muscles, and short intervals of co-contractions due to moderately disturbed reciprocal inhibition. Group 3 (combination of blepharospasm, LP motor impersistence): the EMG patterns, observed in three patients, were characterized by a gradual cessation of LP activity, followed by a brief contraction of OO, which facilitated the return of LP activity, resulting in opening of the eyes. The EMG recordings, thus, revealed the crucial, beneficial role of postinhibition potentiation as a compensatory mechanism in this type of eyelid movement disorder. The EMG patterns were also characterized by short or prolonged periods of dystonic discharges limited to the OO muscles. Group 4 (combination of blepharospasm, involuntary LP inhibition): this group consisted of four patients. In addition to episodes of dystonic activities of OO, the EMG also showed some periods of involuntary inhibition of LP without any concomitant activities of OO. Two patients also exhibited a failure of inhibition of OO muscle activity, following the voluntary contraction of this muscle. The postinhibition potentiation was often not observed. Group 5 (involuntary LP inhibition): consisted of four patients with EMG patterns of involuntary inhibition of LP activity, without any dystonic discharges in OO. The postinhibition potentiation was not observed in this group. The response to the treatment with botulinum toxin was good in the first group and gradually worsened towards the fifth group. Application of botulinum into multiple sites of OO, especially its pretarsal portion, resulted in better response to the treatment in the second and fourth groups.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗
PubMed2006

Results of botulinum toxin: an application to blepharospasm Schwartz-Jampel syndrome.

Schwartz-Jampel syndrome (SJS) is a rare congenital disorder of continuous myotonia, causing visual and eyelid problems such as blepharospasm, acquired ptosis, and blepharophimosis. We report the management of blepharospasm in two sisters with Schwartz-Jampel syndrome with application of botulinum toxin type A (BTX-A), as an alternative to the surgical treatments, such as orbicularis oculi myectomy, levator aponeurosis resection, and lateral canthopexy as described in the literature. Three consequent doses of commercially available BTX-A (Botox-Allergan, Inc. 100 u/via) were injected to orbicularis oculi muscle in two sisters with Schwartz-Jampel syndrome. At the least, the authors waited for a six-month interval to prevent development of sensitivity to BTX-A in patients. No significant improvement was observed after the injection of first dose of BTX-A, with the total dose of 25 units of BTX-A to each orbicularis oculi muscle of the eyes. The muscle tone weakened after the second dose, which was the twice as such of the first applied total dose (50 units of BTX-A to each orbicularis oculi muscle of the eyes) and significant functional and cosmetic improvements were achieved after injection of the third dose, in which the total applied dose was the same as the one applied in second dose. The blepharospasm and eyelid alterations caused by Schwartz-Jampel syndrome should be treated to provide functional and cosmetic improvements. Application of BTX-A to orbicularis oculi muscle for the treatment of blepharospasm could be considered as an alternative to levator resection, and lateral canthopexy in Schwartz-Jampel syndrome.

Adolescent↗
PubMed1999

Follow-up of patients with essential blepharospasm who underwent eyelid protractor myectomy at the Mayo Clinic from 1980 through 1995.

PURPOSE: To determine the efficacy of eyelid protractor myectomy (subtotal excision of the orbicularis oculi, the corrugator supercilii, and the procerus muscles) for the treatment of essential blepharospasm, and to evaluate the need for and the effectiveness of botulinum toxin (BT) injections in these patients. METHODS: The medical records of all patients who underwent eyelid protractor myectomy at the Mayo Clinic (Rochester, MN) from 1980 through 1995 were reviewed. The Health Status Questionnaire was used to assess overall medical and mental health, and a questionnaire specific to eyelid spasms was developed. RESULTS: Fifty-four white patients, of whom 32 (59%) were women, underwent myectomy. The average age at diagnosis of essential blepharospasm was 64 years (median, 65 years; range, 43 to 84 years), whereas the average age at the time of myectomy was 66 years (median, 66 years; range, 51 to 85 years). Of the 14 patients who were treated with BT injections before myectomy, the average interval between the initial injection and surgery was 21 months (median, 20 months; range, 2 to 51 months). Patients who had been treated with BT injections before myectomy were more likely to receive injections postoperatively than were those patients who had not been treated with BT (p < 0.001). Twenty patients were treated with BT injections after myectomy; the overall probability of receiving BT five years after surgery was 46%. Time from myectomy to treatment with BT varied considerably; mean, 880 days; median, 659 days; range, 3 to 4221 days. Postoperative follow-up for those patients who did not receive BT after myectomy ranged from 2 to 5935 days (mean, 2354 days; median, 1722 days). Although the probability of receiving BT injections after myectomy was not associated with age or sex, there was a significant association with the time interval during which the myectomy had been performed (related to the availability of BT as an adjunctive therapy). Of the 41 patients who were alive when the study was conducted, 32 (78%) completed a follow-up survey. Thirty of those (94%) said myectomy provided short-term and long-term benefits. Of the 11 patients who received BT injections before and after myectomy, six (55%) said the toxin was more effective in ameliorating eyelid spasms after surgery and four (36%) required injections less frequently after myectomy. Results from the Health Status Questionnaire showed no significant differences between patients who underwent myectomy and control subjects. CONCLUSIONS: Eyelid protractor myectomy provides subjective benefit to patients with essential blepharospasm and decreases the long-term need for BT injections in approximately 50% of these patients. Although the probability of receiving postoperative BT paralleled its availability, patients who received both preoperative and postoperative BT perceived either increased efficacy of the toxin injections, longer-lasting effects, or both, after myectomy. Patients with severe disability from blepharospasm benefited more from myectomy than did patients with relatively mild symptoms.

Adult↗
PubMed2000

Frontalis suspension combined with blepharoplasty as an effective treatment for blepharospasm associated with apraxia of eyelid opening.

PURPOSE: Essential blepharospasm can be associated with apraxia of eyelid opening and is characterized by the inability to initiate the act of eyelid elevation even after cessation of orbicularis spasms. Current therapies such as botulinum toxin injections, orbicularis resection, or neurectomy may be unsuccessful or have undesired side effects. METHODS: Frontalis suspension was used to treat 13 consecutive patients with apraxia and blepharospasm during a 4-year interval. Follow-up ranged from 16 months to 55 months. To improve the aesthetic outcome, an upper blepharoplasty was done at the same time as the frontalis suspension in 7 cases. RESULTS: Good or excellent functional results were obtained in 10 of 13 patients. In 6 of these patients, the spasm disappeared completely. Therapy was unsuccessful in 1 patient, and in 2 patients blepharospasm recurred after 9 months. CONCLUSION: Patients with blepharospasm and apraxia of eyelid opening may benefit from a frontalis suspension operation, which can be considered minimally invasive and reversible.

Adult↗
PubMed2005

Assessment of blepharospasm surgery using an improved disability scale: study of 138 patients.

PURPOSE: To assess a new scale for grading functional disability in a series of patients operated on for blepharospasm resistant to treatment by botulinum toxin (BT) injections. METHODS: The bicentric study included 138 patients: 80 (58%) with essential blepharospasm; 46 (33%) with apraxia of eyelid opening; and 12 (9%) with intermediate forms. They had undergone orbicularis myectomy (92 cases, 67%), frontalis suspension (31 cases, 22%), or both (15 cases, 11%). Functional disability was assessed by using an original scale covering 6 daily life activities, each graded using 5 levels from 0 (no disability) to 4 (activity impossible). The score obtained was related to the total of activities actually performed to produce a functional disability score (FDS) from 0 to 100. Each patient's FDS was prospectively determined, before surgery and 3 months after surgery (M3). In patients who, after surgery, required a further course of BT injections, the FDS was assessed immediately before the third injection. RESULTS: The mean FDS fell significantly, from 78 +/- 15 (standard deviation) before surgery to 45 +/- 21 at M3 (p < 0.01%), whatever the clinical form of essential blepharospasm or surgical procedure. The mean postoperative FDS was significantly lower in the 62 patients (45%) weaned off BT than in the unweaned group, respectively (31 +/- 17 versus 56 +/- 18; p < 0.01). In the latter group, the mean FDS after BT reinjection (34 +/- 19) fell to a level comparable with that of the weaned patients. CONCLUSIONS: This accurate, easy-to-use scale makes it possible to quantify in patients with blepharospasm functional improvement as the result of surgery and, where applicable, of BT reinjection.

Activities of Daily Living↗
PubMed1990

Essential blepharospasm: nursing update.

Essential blepharospasm is a chronic, potentially disabling disorder for which there is no known cause or cure. The term blepharospasm derives from the Greek word 'blepharon' meaning eyelid and the word 'essential' implies unknown cause. In neurological literature blepharospasm is classed as a focal dystonia. Lack of recognition of the disease may cause patients to consult numerous physicians as well as acupuncturists, chiropractors, faith healers and others in an effort to find a cure to what they are sure is not just a psychological problem. Nurses who are knowledgeable about the disease entity can easily recognize the symptoms. They can also prevent disability by recommending appropriate professional help. Treatment for the control of symptoms has only recently become available. It is the purpose of this discussion to review current information about blepharospasm that has particular relevance for neuroscience nurses in their personal as well as professional lives.

Aged↗
PubMed2005

Health-related quality of life in blepharospasm or hemifacial spasm.

OBJECTIVES: Health-related quality of life (HRQL) has become an important outcome criterion of medical interventions, but was hardly studied in patients with facial spasms. MATERIALS AND METHODS: Patients with blepharospasm or hemifacial spasm treated with botulinum toxin were included. A healthy control group sociodemografically matched to patients was established. Instruments applied included the SF-36 (global HRQL), the NEI-VFQ (disease-specific HRQL) and the Blepharospasm Rating/Disability Scale, the latter to patients only. RESULTS: Thirty-one patients with blepharospasm and 21 patients with hemifacial spasm took part. The Blepharospasm Rating/Disability Scale revealed minor functional limitations, but identified some symptoms to be improved. In both patient groups global (both SF-36 Component Summaries) and disease-specific (eight of 12 subscales) HRQL were significantly impaired compared with controls. CONCLUSION: HRQL should be integrated as core outcome criterion in treatment of patients with facial spasms. Assessment by a generic and a disease-specific instrument is recommended.

Aged↗
PubMed1997

Animal model explains the origins of the cranial dystonia benign essential blepharospasm.

The current study demonstrates that combining two mild alterations to the rat trigeminal reflex blink system reproduces the symptoms of benign essential blepharospasm, a cranial dystonia characterized by uncontrollable spasms of blinking. The first modification, a small striatal dopamine depletion, reduces the tonic inhibition of trigeminal reflex blink circuits. The second alteration, a slight weakening of the lid-closing orbicularis oculi muscle, begins an adaptive increase in the drive on trigeminal sensory-motor blink circuits that initiates blepharospasm. By themselves, neither of these modifications causes spasms of lid closure, but combined, they induce bilateral forceful blinking and spasms of lid closure. A two-factor model based on these rodent experiments may explain the development of benign essential blepharospasm in humans. The first factor, a subclinical loss of striatal dopamine, creates a permissive environment within the trigeminal blink circuits. The second factor, an external ophthalmic insult, precipitates benign essential blepharospasm. This two-factor model may also be applicable to the genesis of other cranial dystonias.

Animals↗
PubMed1997

Different effects of levodopa and apomorphine on blink reflex recovery cycle in essential blepharospasm.

With the aim of assessing dopaminergic responsiveness in essential blepharospasm, we investigated the effects of oral levodopa and subcutaneous apomorphine on blink reflex recovery cycle in 7 blepharospasm patients. We found that in blepharospasm the excitability of the blink reflex recovery cycle was increased compared with control subjects. The oral administration of levodopa/carbidopa (500/50 mg) did not significantly modify the blink reflex recovery cycle. The 50 micrograms/kg dose of apomorphine decreased the amplitude of conditioned responses at 300 and 500 ms, whereas the 10 micrograms/kg dose was ineffective. We conclude that the excitability of the blink reflex recovery cycle in blepharospasm is partly under dopaminergic control. The partial normalization of the blink reflex recovery cycle excitability observed with 50 micrograms/kg apomorphine is consistent with the reported clinical efficacy of the drug in this condition.

Administration, Oral↗
PubMed1998

Higher prevalence of obsessive-compulsive symptoms in patients with blepharospasm than in patients with hemifacial spasm.

OBJECTIVE: The prevalences of obsessive-compulsive symptoms in patients suffering from blepharospasm and in those with hemifacial spasm were determined. The two conditions have similar symptoms, but only blepharospasm is etiologically linked to basal ganglia dysfunction. METHOD: After being interviewed with the Structured Clinical Interview for DSM-III-R, 13 patients with blepharospasm and 13 with hemifacial spasm completed the SCL-90-R and the Hamburg Obsession/Compulsion Inventory-Short Form. RESULTS: Patients in the blepharospasm group had significantly more obsessive-compulsive symptoms, as indicated by higher scores on the Hamburg Obsession/Compulsion Inventory-Short Form, than the patients with hemifacial spasm. SCL-90-R scores were in the normal range for nine and eight categories, respectively (out of nine). CONCLUSIONS: The findings provide additional support for the hypothesis that obsessive-compulsive symptoms are related to basal ganglia dysfunction.

Aged↗
PubMed2006

[Essential blepharospasm and hemifacial spasm: characteristic of the patient, botulinum toxin A treatment and literature review].

PURPOSE: To evaluate the characteristics of the essential blepharospasm and hemifacial spasm patients and the feasible treatment with botulinum toxin A. METHODS: Thirty-four essential blepharospasm or hemifacial spasm patients were evaluated according to gender, ocular complaint, time of disease, treatment outcome and complications. RESULTS: Age median was 63 years and the mean was 61 years, with no difference regarding sex; 66.66% of the patients had hemifacial spasm and 33.33%, essential blepharospasm. Many patients complained of dry eye associated with involuntary spasm. Botulinum toxin A showed a positive outcome in 91.30% of the treated patients and complications observed after treatment were eyelid ptosis (8.33%) and buccal angle deviation (8.33%). CONCLUSION: Essential blepharospasm and hemifacial spasm occurred in the elderly, of both sexes. Treatment with botulinum toxin A was useful, with very low complication rates.

Adult↗
PubMed2005

[Upper lid frontalis suspension as a treatment for essential blepharospasm].

BACKGROUND: Essential blepharospasm is a progressive disease of unknown etiology causing a significant decline in the quality of life of patients suffering from the disease. Currently used treatment for this condition is repeated injections of Botulinum toxin. Some of the patients' therapeutic response to Botox injections is insufficient, and some develop ocular complications. GOALS AND METHODS: This report presents our experience with sling frontalis suspension for essential blepharospasm in 4 patients with follow-up periods of six months to 2 years. A literature review is presented and discussed. RESULTS: A treatment trial with Botox injections was attempted in all four patients. Three of the patients showed insufficient treatment response. Three of the patients developed side effects of recurrent episodes of upper lid ptosis following injections. In all four patients that underwent operations significant reduction of blepharospasm was observed. In two of the patients Botulinum toxin could be discontinued, in one patient treatment intensity could be reduced, and in three--treatment complications were prevented. All four patients reported a high degree of satisfaction from treatment results. CONCLUSIONS: Our results indicate that sling frontalis suspension for essential blepharospasm is a safe and efficient treatment for essential blephrospasm with a high degree of patient satisfaction. Further large-scale studies are needed to substantiate our results.

Aged↗
PubMed2006

[The effect of botulinum A toxin repeated injection in treating blepharospasm and hemifacial spasm].

OBJECTIVE: To study the long-term efficacy of botulinum A toxin (BTXA) repeated injection for the treatment of blepharospasm and hemifacial spasm (HFS). METHOD: Eighty patients with blepharospasm and/or HFS (75 sides and 65 sides respectively) were treated with constant BTXA local repeated injection over 4 times and the dosage was 12.5-15.0 U and 22.5-32.5 U respectively in each injection. A total number of 886 injections were given. All the patients were followed up for 2-8 years. RESULT: The earliest effect was found in 2-3 days after the treatment. The effective ratios of the partial relieved, remarkably relieved and completely relieved in blepharospasm and HFS were 0, 67.7%, 32.7% and 1.4%, 72.3%, 26.3% respectively. The average duration of improvement lasted about (19.9 +/- 4.5) weeks and (20.7 +/- 4.2) weeks respectively (P>0.05), which increased with the times of injection. The local side effects were mild and transient. CONCLUSION: BTXA for the treatment of blepharospasm and HFS is a safe, effective and simple method.

Adolescent↗
PubMed1983

Prosthesis for blepharospasm.

Following several years of experience with a newly designed spectacle-supported ptosis crutch for ptosis and blepharospasm, the author has developed a new approach to these conditions. In mild to moderate blepharospasm cases, the spectacle-supported prosthesis can still produce satisfactory results. However, in moderate to severe blepharospasm, the force of the spasm moves the entire device forward and downward, making it ineffective or too uncomfortable to wear. To create greater stability and a more effective counterforce to the blepharospasm, the author has developed a simple headband-supported device which does not use a spectacle frame for support. This new prosthesis is constructed of varying diameters of spring tempered diamond drawn stainless steel orthodontic wire, partially or completely covered by Teflon tubing. A silicon rubber cylinder is placed over the center which becomes a bridge and the main support for the device. An elastic head band completes the prosthesis.

Adult↗
PubMed1994

Surgical technique for patients with the apractic type of essential blepharospasm: case report.

A frontalis suspension was carried out in a patient with an essential type of blepharospasm, characterized by difficulties in initiating the act of lid elevation, often referred to as the apractic form of blepharospasm or, as J. Elston proposed, the pretarsal blepharospasm. The patient tries to open the eyes by using the frontalis muscle or by manual traction. It is known than in this form of blepharospasm, insufficient results are seen after botulinum toxin infection. Proper examination of the skin crease of the upper eyelid and of the eyelid gives an idea of the insertion of the levator aponeurosis and of the levator muscle function. A desinsertion, due to frequent manual traction, may be found. In this case, reinsertion of the aponeurosis may relieve the symptoms. If no desinsertion is present a frontalis suspension, similar to those used in ptosis surgery, may give good results.

Blepharospasm↗
PubMed1996

Effect of photic conditioning on blink reflex recovery function in blepharospasm.

Patients with blepharospasm may have selective sensitivity to photic stimuli, for their symptoms are often aggravated after exposure to brightness. To investigate the pathophysiology underlying this phenomenon, we compared the effects of electric and photic conditioning on electrically-evoked blink reflex in 21 patients with blepharospasm and 11 age-matched control subjects. With electric conditioning, R1 was facilitated at inter-stimulus intervals (ISIs) of up to 100 ms in both groups. R2 was inhibited at all ISIs longer than 20 ms in the normals, but to a much lesser extent in the patients, suggesting increased excitability of the multi-synaptic reflex pathway. Compared to electric conditioning, photic conditioning was less potent in inhibiting R2 in the normals. In contrast, photic conditioning in the patients produced R2 inhibition as powerful as electric conditioning. These findings indicate that, in patients with blepharospasm, photic input has a relatively more profound inhibitory effect on the test blink reflex than electric conditioning as compared with normal subjects. The greater susceptibility of the brainstem interneurons to photic conditioning in blepharospasm may be related to the clinically-observed light sensitivity.

Adult↗