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PubMed1987

Use of botulinum toxin in blepharospasm and other facial spasms.

Essential blepharospasm is an involuntary spasmodic closure of the eyelids. It frequently makes normal social human life such as reading, driving and even walking almost impossible. Botulinum toxin has been approved for investigational use for treatment of blepharospasm. We report the results and side-effects of botulinum A toxin treatment of the first 13 patients in Finland. Temporary control of spasms was achieved in all patients varying from 1 to 38 weeks with a mean of 11 weeks after the first injection. Most patients needed re-treatment after varying intervals. All complications were transient.

Adult↗
PubMed1987

Long-term results of treatment of idiopathic blepharospasm with botulinum toxin injections.

One hundred and one patients with idiopathic blepharospasm have been treated with injections of botulinum toxin A into the orbicularis oculi. Ten had previously had facial nerve avulsions and responded well, normal visual function being restored in the majority (7/10) for an average of 14 weeks. Without prior surgical treatment the response was more variable, but 71/91 regained normal or near normal vision. Older patients, those with a family history of the condition, and those without oromandibular dystonia responded slightly better. The severity of the blepharospasm, the length of the history, and spontaneous resolution of an episode of focal dystonia in the past had no influence on the outcome. Results were poor in the presence of an associated neurological disorder. Side effects, particularly a temporary partial ptosis, were common but were well tolerated. The average duration of improvement was eight weeks in men, nine in women, and there was no evidence of any increase in duration after multiple injections. Eighty nine patients continued with injections, 11 opted for surgical treatment, and one resumed drugs.

Adult↗
PubMed1991

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↗
PubMed1996

Treatment selections of 239 patients with blepharospasm and Meige syndrome over 11 years.

BACKGROUND: A retrospective review of 239 patients with benign essential blepharospasm and Meige syndrome was performed in order to determine patients' long term treatment preferences. METHODS: Of 239 patients evaluated, 228 received local injections of botulinum toxin, type A, into the eyelid and facial musculature over 11 years. RESULTS: Of 228 patients, 202 (72.1%) were still treated with botulinum toxin, type A. Eighteen patients (6.9%) no longer received botulinum toxin injections and sought no other treatment. Five patients (2.2%) had apparent remission of their disease after injection. Three patients (1.3%) ultimately obtained relief from orbicularis muscle extirpative surgery and required no additional treatment. Two of the 11 patients (4.6%) who chose not to receive botulinum toxin injections were successfully treated with other modalities: psychotherapy (one patient) and oral haloperidol (one patient). CONCLUSION: While botulinum toxin is the most highly effective treatment for benign essential blepharospasm and Meige syndrome over a long period of time, adjunctive oral drug therapy, including minor tranquillisers as well as eyelid surgery, may augment its effectiveness.

Anti-Anxiety Agents↗
PubMed1976

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↗
PubMed1995

Pretarsal application of botulinum toxin for treatment of blepharospasm.

The response to botulinum toxin type A was compared after two injection techniques in 45 patients with blepharospasm. Initially, patients were treated according to a triple injection technique; two injections into the upper eyelid and one injection into the lower eyelid. Subsequently, without altering the dose, the same patient group received two further injections into the pretarsal portion of the orbicularis oculi muscle of the upper lid. Triple injections were given in 227 treatments, of which 81% were successful. Mean duration of benefit was 8.5 weeks. Additional pretarsal injections were given in 183 treatment sessions. The number of successful treatments significantly increased, to 95% (P < 0.001), and the mean duration of benefit increased to 12.5 weeks (P < 0.001). Ptosis occurred significantly less often after pretarsal injections (P < 0.01). Patients with combined blepharospasm and involuntary levator palpebrae inhibition responded better to the pretarsal injection technique.

Adult↗
PubMed2000

The control of blepharospasm by essential fatty acids.

Dopamine depletion induced by administration of Ro4-1284 produces a condition of rapid and repeated eye blinking in rats. This condition mimics the human disorder, blepharospasm, which often accompanies parkinsonism and other dopamine deficiency disorders. When given a 3-week course of a compound (SR-3) developed from a specific ratio of two free polyunsaturated fatty acids - linoleic acid and alpha-linolenic acid - the eye blinking rate following administration of Ro4-1284 is reduced to saline and no drug control levels. These results suggest a favorable prospect for essential fatty acids in general, and SR-3 in particular, to provide an improved therapeutic option for the clinical management of benign essential blepharospasm.

2H-Benzo(a)quinolizin-2-ol, 2-Ethyl-1,3,4,6,7,11b-↗
PubMed2001

Orbicularis oculi and orbicularis oris reflexes in blepharospasm and torticollis spasmodica during spasm-free intervals.

To investigate possible abnormalities of the blink reflex pathways, we analyzed the latencies and amplitudes of the blink reflex responses in the orbicularis oculi (Ooculi) muscle, following supraorbital nerve stimulation, in 19 patients with blepharospasm, 16 patients with torticollis spasmodica and 22 control subjects. Furthermore, in order to examine the suprasegmental control of the responses, the reflex responses were also evoked in the orbicularis oris (Ooris) muscle after stimulation of the ipsilateral supraorbital nerve. The responses were recorded only when subjects had no contractions of the eyelid muscles, either involuntarily, voluntarily or spontaneously; this could be controlled by a sound signal. The metrics of the reflex responses in the Ooculi and Ooris muscles in patient groups were comparable to those in controls. Our data indicate that the afferent and efferent pathways of the reflex arc and the suprasegmental control of the reflex are intact in patients with blepharospasm and torticollis spasmodica, at least during spasm-free intervals. Alterations of responses may occur during spasms due to either segmental or suprasegmental changes.

Adult↗
PubMed1992

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↗
PubMed1981

Meige syndrome (blepharospasm-oromandibular dystonia) after long-term neuroleptic therapy.

Two patients developed either blepharospasm or blepharospasm-oromandibular dystonia following chronic therapy with chlorpromazine, haloperidol, or thioridazine. In one patient, appearance of the movement disorder was associated with neuroleptic withdrawal, and in the other patient, the movement disorder began while neuroleptic therapy continued. Because of the age of one patient and the severe intermittent psychosis in the other, these Meige-like symptoms were attributed to chronic neuroleptic use rather than to spontaneously occurring Meige syndrome. The symptoms occurring as part of a tardive dyskinesia suggest that dopaminergic mechanisms play a role in idiopathic Meige syndrome.

Adolescent↗
PubMed1983

Blepharospasm associated with brainstem lesions.

We studied six patients with clinical and radiographic evidence of rostral brainstem lesion and bilateral blepharospasm. Two patients also had other facial dystonic movements. Four patients suffered rostral brainstem strokes, and two had multiple sclerosis. None had been treated with antipsychotic drugs prior to the onset of blepharospasm. Medical treatment was helpful in two patients, and bilateral selective facial nerve section was used in another patient. Possible pathogenic mechanisms are discussed.

Adult↗
PubMed1987

Familial apoceruloplasmin deficiency associated with blepharospasm and retinal degeneration.

A 52-year-old woman had a newly recognized disorder of familial hypoceruloplasminemia, blepharospasm, retinal degeneration, and high-density areas in CT of the basal ganglia and liver scan. Immunofixation electrophoresis disclosed apoceruloplasmin deficiency. Kinetic, x-ray analysis, and histochemical study showed accumulation of iron in liver and brain, but not of copper. Intestinal copper absorption was reduced, but liver uptake was increased. Ceruloplasmin is involved in iron metabolism, and the findings suggest that hypoceruloplasminemia due to lack of apoceruloplasmin was causally linked to the iron deposition in basal ganglia and other organs, leading to blepharospasm and retinal degeneration.

Apoproteins↗
PubMed1996

Blepharospasm in association with a lower pontine lesion.

A patient with neurofibromatosis type I and breast carcinoma developed a bilateral but asymmetric blepharospasm and paresis of the left abducens muscle over a 2-week course. MRI disclosed a small lesion in the left dorsomedial lower pontine region. Electrodiagnostic investigations revealed bilateral R1 responses after stimulation of the left supraorbital nerve and enhancement of R1 and R2 recovery curves. We concluded that lesions in the lower pontine tegmentum may cause blepharospasm.

Blepharospasm↗
PubMed2002

A polymorphism in the dopamine receptor DRD5 is associated with blepharospasm.

Abnormalities in dopamine neurotransmission are thought to underlie the generation of dystonic movements. The authors performed a case-control allelic association study in patients with the focal dystonia blepharospasm, using polymorphisms in the dopamine receptor and transporter genes. Allele 2 of a DRD5 dinucleotide repeat was significantly associated with blepharospasm. This may indicate a pathogenic role for this receptor.

Adult↗
PubMed2005

Botulinum toxin a in the treatment of blepharospasm: a 10-year experience.

To evaluate the long-term effect of botulinum toxin type A (BTX) in the treatment of blepharospasm, a retrospective analysis was conducted from the patients seen at the Movement Disorders Clinic of the Department of Neurology, Hospital das Clínicas, University of São Paulo School of Medicine from 1993 to 2003. A total of 379 treatments with BTX were administered to 30 patients with blepharospasm. Sixty six per cent of the subjects had used oral medication for dystonia and only 15% of them reported satisfactory response to this treatment. Ninety three per cent of the patients showed significant improvement after the first BTX injection. There was no decrement in response when compared the first and the last injection recorded. Adverse effects, mostly minor, developed at least once in 53% of patients. Six patients (20%) discontinued the treatment but there was no case of secondary resistance.

Blepharospasm↗
PubMed1999

Lamotrigine-induced blepharospasm.

Movement disorders such as tremor and ataxia occur commonly during therapy with antiepileptic drugs (AEDs). Dystonias, however, are rare. Blepharospasm, although reported with neuroleptic agents, has never been reported with AEDs. Our patient developed blepharospasm during therapy with lamotrigine.

Anticonvulsants↗
PubMed1989

Blinking-blepharospasm after long-term neuroleptic treatment.

A patient is described who developed frequent blinking and blepharospasm after long-term treatment with trifluoperazine, whose condition improved dramatically after the cessation of the drug. The implications of this for our understanding of the manifestations and natural course of the late-onset side-effects of neuroleptic drugs are discussed. This case further supports the role of dopaminergic mechanisms in the aetiology of Meige's syndrome, which has blepharospasm and oromandibular dystonia as its main manifestations.

Akathisia, Drug-Induced↗