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Memory-contingent saccades and the substantia nigra postulate for essential blepharospasm.

Essential blepharospasm and cranial dystonia are related focal dystonias of unknown aetiology. Blepharospasm induced by acute dopamine depletion in parkinsonism restricts saccade initiation possibly via the substantia nigra pars reticulata (SNpr). If essential blepharospasm and cranial dystonia similarly restrict saccades, then a selective, somatotopically arranged pathway such as the SNpr may be involved. To test this possibility memory-contingent and visually-guided saccades were measured in patients with essential blepharospasm and cranial dystonia. The latency of both forms of saccades was either significantly prolonged or excessively variable, while the accuracy and peak velocity of these fast eye movements were similar to age-matched control subjects. Essential blepharospasm and cranial dystonia alter the initiation of saccadic eye movements. Subcortical brain regions or pathways where eyelid, saccade and cranial/cervical motor control are somatotopically approximated, such as the SNpr, may be involved in blepharospasm.

Blepharospasm

Blepharospasm and obsessive-compulsive disorder.

Although essential blepharospasm is considered to be a form of focal dystonia, many patients with blepharospasm have been noted to have concomitant depression, anxiety, phobias, hypochondriasis, and other emotional and behavioral disorders, suggesting a psychiatric component to the disease that is phenomenologically similar to obsessive-compulsive disorder (OCD) in terms of the repetitive, perseverative, and persistent nature of the symptoms. The Maudsley OCD questionnaire was administered to 21 patients with blepharospasm and 19 normal controls. The blepharospasm patients scored significantly higher than the controls (p less than .01). Although preliminary, the current study does support at least a phenomenological link between OCD and blepharospasm.

Aged

Signs of essential blepharospasm: a motion-picture analysis.

Motion pictures of 15 patients with essential blepharospasm were studied. Previously unrecognized signs indicated multiple cranial nerve involvement. These signs include impersistence of gaze, lid retraction, tongue thrust, head tilts, head jerks, vertical gaze spasms, and asymmetry. The sugns were also observed in a patient with bilateral blepharospasm who had a history of Bell's palsy suggesting facial nerve injury as a possible factor in blepharospasm. The presence of these signs can be explained by known neural pathways, but the site, or sites, of the lesion remains obscure. These signs may be important in assessing severity and in treatment evaluation.

Adult

The management of blepharospasm and hemifacial spasm.

The aetiology of blepharospasm and hemifacial spasm is different, but both produce involuntary eye closure and facial movements which do not respond to systemic drug treatment. The introduction of therapeutic focal muscle weakening with botulinum toxin injections in the early 1980s appeared to offer great promise in the management of these conditions. In this paper the results of botulinum toxin treatment of 234 patients with blepharospasm and 73 patients with hemifacial spasm over a 7-year period have been analysed. Most patients receive sustained benefit from repeated injections whilst side-effects become less frequent. A clinically recognisable subgroup of patients with blepharospasm respond poorly and may be better treated surgically.

Blepharoptosis

Clinical doxorubicin chemomyectomy. An experimental treatment for benign essential blepharospasm and hemifacial spasm.

Doxorubicin (DXR) was injected as a treatment for benign essential blepharospasm and hemifacial spasm. The other eyelids were treated concurrently with botulinum toxin (BT). No DXR-treated eyelid has maintained 0 strength (commonly achieved with BT). Two patients with benign essential blepharospasm and four patients with hemifacial spasm have achieved major improvement, sustained for more than 6 months. Eyelids have been swollen and inflamed for up to 3 months. No spontaneously irreversible complication has occurred. A single injection at the maximum safe dose (1 mg in the upper lid and 1.5 mg in the lower lid) has not proven sufficient to produce cure. Treatment of each lower eyelid of a muscular male with severe blepharospasm may require cumulative doses of up to 4.0 mg, delivered in three injection events separated by at least 2 months, with each injection no greater than 1.5 mg DXR per site. At the present time, there is no assurance that a permanent cure will result.

Aged

[Magnetic stimulation in patients with essential blepharospasm].

In 31 patients with an essential blepharospasm investigations were undertaken with transcranial cortical and cisternal magnetic stimulation of the facial nerve. Cortical stimulation was performed contralaterally, whereas cisternal stimulation was ipsilateral; the latencies and amplitudes of the potentials were determined. However, the extracranial part of the facial nerve was stimulated in the region of the stylomastoid foramen for neuromyography electrically. In most measurements the response potentials in the orbicularis oculi muscle and the levator labii muscle were determined. Cisternal stimulation yielded normal latencies, whereas cortical stimulation showed an increase of latency in some blepharospasm patients. A difference between the orbicularis oculi muscle and the levator labii muscle as known in patients with hemifacial spasm (20) could not be observed. The mean values of all latencies after cortical stimulation were normal. The method of transcranial magnetic stimulation is an important part of the complete investigation of patients with facial hyperkinesias as shown here in blepharospasm patients.

Blepharospasm

Neurophysiological observations on the effects of botulinum toxin treatment in patients with dystonic blepharospasm.

Botulinum toxin treatment improves dystonic blepharospasm by inducing transient paresis of the orbicularis oculi muscle. It is not known if it also reduces the enhanced brainstem neuronal excitability found in this disorder. We have performed conventional electromyography (EMG) and blink reflex excitability studies on fifteen patients with blepharospasm before and after botulinum toxin treatment. Denervation signs were found with needle EMG in all treated muscles. Amplitude of the facial compound muscle action potential (CMAP) and R1 response was reduced after botulinum toxin injections. In blink reflex excitability studies, the recovery of R2 response was enhanced after treatment even when patients were tested at the time of maximal benefit from botulinum toxin injections. The results suggest that there is little influence of botulinum toxin treatment upon the enhanced excitability of brainstem interneurons in patients with blepharospasm.

Adult

Facial dystonia, essential blepharospasm and hemifacial spasm.

Movement disorders, or dyskinesias, in the facial region may be categorized in several ways. Dystonic movement disorders in the cranial-cervical region, including essential blepharospasm, Meige syndrome and spasmodic torticollis, are characterized by uncontrollable squeezing movements in the face and neck. These disorders typically present in the fifth and sixth decades of life. Essential blepharospasm is particularly debilitating, as the involuntary eyelid closure that accompanies this condition may result in functional blindness with an otherwise normal visual pathway. Hemifacial spasm is an intermittent, unilateral, spasmodic contraction of the muscles innervated by the facial nerve. This disorder usually presents in the third or fourth decade and has a different underlying pathophysiology than the dystonias. Botulinum A toxin therapy has largely supplanted surgical intervention in the treatment of essential blepharospasm and hemifacial spasm.

Blepharospasm

Blepharospasm.

Blepharospasm is a cranial nerve dysfunction in which involuntary and uncontrollable forcible lid closure occurs. Surgical treatment is far from ideal, but the condition can be improved by sectioning the branches of the facial nerve plexus which supply the orbicularis oculi, the frontalis and corrugator muscles, and the midfacial muscles. Not all patients with blepharospasm should be operated upon, since they may expect too much or may enjoy the attention their invalidism has given them. Ectropion of one or both lower lids may occur, and the most severe of all complications is recurrence. This may happen despite a careful microscopic search for the nerve fibers and their division and avulsion. Blepharospasm patients often require considerable psychological support.

Adult

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

[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

Effects of eyelid protractor excision for the treatment of benign essential blepharospasm.

Data were analyzed from 37 patients with essential blepharospasm who had undergone upper eyelid protractor excision with brow suspension, and who had a median follow-up of 40 months (range, 12 to 83.5 months) after surgery. Of the 37 patients, 15 (40.5%) had sufficient relief of their spasms at their last follow-up; 22 patients (59.5%) had functionally significant recurrent or residual spasms after upper eyelid surgery. Of these 22 patients, 12 (55%) had recurrences more than six months after surgery, and four patients (18%) had recurrences more than one year after surgery. Patients with Meige syndrome were no more likely to experience recurrent spasms after upper eyelid surgery than patients who lacked a lower facial spasm component. Recurrence of symptoms was primarily the result of lower eyelid spasms in 11 patients (50%), upper eyelid spasms in one patient (5%), and all eyelids in six patients (27%). The location of recurrent spasms could not be ascertained in four patients (18%), with whom we had only telephone contact. Seven of 12 patients (58%) undergoing secondary procedures had relief from their spasms at the end of this study. Excluding those patients who chose to decline further surgery, 22 of 27 patients (81.5%) received satisfactory relief of symptoms after upper eyelid and, if needed, lower eyelid and repeat upper eyelid protractor excision. These long-term results are comparable to those reported previously for differential section of the facial nerve. Because the side effects of eyelid protractor excision are generally more acceptable, we recommend that when surgery is determined to be necessary for essential blepharospasm, upper eyelid protractor excision is the preferred surgical approach.

Aged

Surgical management of essential blepharospasm.

We have reviewed the surgical management of essential blepharospasm over the last 15 years, comparing the results from facial nerve avulsion with those from orbicularis muscle stripping. After facial nerve avulsion 50% of patients remained free of troublesome spasm for 15 months after surgery, but only 25% remained so for more than two years. Following orbicularis oculi myectomy 50% of patients were free of troublesome spasms for 30 months after surgery and 55% of patients had relief from spasm for more than two years. Secondary effects of the two procedures are compared and are found to be fewer after orbicularis myectomy. There were no major complications after either form of surgery. Botulinum toxin is the treatment of first choice for this condition. If this becomes ineffective or inconvenient, surgical treatment is warranted and should not be deferred for fear of severe side effects of treatment, since these are rare. Protractor myectomy gives longer relief from blepharospasm than facial nerve avulsion and has fewer complications. However, it is technically difficult, time consuming, and has greater peroperative morbidity. Facial nerve avulsion may therefore still have a role in selected patients.

Adult

Blepharospasm-oromandibular dystonia syndrome (Brueghel's syndrome). A variant of adult-onset torsion dystonia?

Thirty-nine patients with the idiopathic blepharospasm-oromandibular dystonia syndrome are described. All presented in adult life, usually in the sixth decade; women were more commonly affected than men. Thirteen had blepharospasm alone, nine had oromandibular dystonia alone, and 17 had both. Torticollis or dystonic writer's camp preceded the syndrome in two patients. Eight other patients developed toritocollis, dystonic posturing of the arms, or involvement of respiratory muscles. No cause or hereditary basis for the illness were discovered. The evidence to indicate that this syndrome is due to an abnormality of extrapyramidal function, and that it is another example of adult-onset focal dystonia akin to spasmodic torticollis and dystonic writer's cramp, is discussed.

Adult

Botulinum toxin as a treatment for blepharospasm, spasmodic torticollis and hemifacial spasm.

Fifty-two patients affected by focal dystonia or hemifacial spasm were treated with repeated injections of botulinum toxin. A clinical improvement was observed in all patients with blepharospasm; clinical benefit had a mean duration of 10 weeks. Clinical results were less impressive, but also favorable in patients affected by spasmodic torticollis and by hemifacial spasm. In the latter, the incidence of drug-induced paresis was much higher than that observed in patients with blepharospasm, even though the doses of toxin injected were significantly lower.

Adult

Treatment of blepharospasm, hemifacial spasm and strabismus with botulinum a toxin.

Thirty patients with blepharospasm, hemifacial spasm, strabismus and entropion were treated with botulinum A toxin giving satisfactory results. Rapid spasm relief, correction of strabismus and entropion were obtained. Only mild, transient and local side-effects occurred. The patients were followed up for 4-12 weeks with no recurrence. The clinical results show that local injection of a minute dose of botulinum A toxin in treating blepharospasm, hemifacial spasm, strabismus and entropion is a safe, effective and simple method of nonsurgical therapy.

Adult

Blepharospasm associated with neurocysticercosis.

We report a case of a 48-year-old woman who had a 4 years history of bilateral blepharospasm. CT-scan showed multiple intracranial calcifications (two of them in the left thalamus) corresponding to neurocysticerosis, and a lacunar infarction in right internal capsule area. To our knowledge this case is the first one reported in the literature of blepharospasm associated with (and perhaps symptomatic of?) neurocysticercosis.

Blepharospasm

[Botulinum toxin in the treatment of blepharospasm. Initial experience].

The clinical effects of botulinum toxin A were evaluated in eight patients with idiopathic blepharospasm and hemifacial spasm. A substantial reduction or disappearance of muscle spasms were observed after the treatment. The action of botulinum toxin became noticeable 2 days after administration with a peak effect at 2 weeks. This effect diminished with muscle spasms returning about 12 weeks after administration. No serious adverse events were noted. The treatment with botulinum toxin has become method of choice in blepharospasm, it is proposed for the management of local muscle spasms of various origin.

Adult