Aripiprazole in Meige Syndrome: Clinical Response and Implications for Treatment Development.

INTRODUCTION: Meige syndrome is a segmental form of dystonia where botulinum toxins are the preferred treatment option. However, its invasive nature, treatment costs, partial responsiveness, and benefit duration are some of their limitations. METHODS: Six consecutive subjects with Meige syndrome were treated only with aripiprazole. RESULTS: A dramatic response was obtained in all subjects during the first weeks of treatment. Aripiprazole mean ± SD daily dose was 7.9 ± 3.6 mg. Three subjects developed parkinsonism related to aripiprazole treatment; the former improved after reducing the dosage, without significant worsening of cranial dystonia. After a mean ± SD follow-up of 2.0 ± 0.7 years, clinical benefit persists over time, with a mean percentage reduction of Unified Dystonia Rating Score of 75.6% ± 8.4%. CONCLUSIONS: Aripiprazole should be considered as an alternative treatment option among subjects with Meige syndrome, especially in those refractory to botulinum toxin injections. The clinical response shown in our patients may lead to treatment development.

Report: A case report of Meige syndrome-like blepharospasm caused by ingestion of allopurinol.

One case of allopurinol-caused rare adverse reactions was reported. One male 51-year-old patient presented blurred vision, streaming eyes, photophobia and blepharospasm sequentially 1 week after oral administration of allopurinol. Complete remission was obtained after Botulinum toxin was locally injected. Allopurinol may cause Meige syndrome-like blepharospasm, the mechanism of which may be related to the inhibition of dopamine activity by affecting adenosine level in the brain.

Methylphenidate-induced acute orofacial and extremity dyskinesia.

Methylphenidate is a short-acting stimulant. In this article, the authors report a 7-year-old male patient who presented with orofacial and limb dyskinesia after his first dose of methylphenidate treatment for a diagnosis of attention-deficit/hyperactivity disorder; he was also receiving sodium valproate treatment for epilepsy. Orofacial dyskinesia appeared 5 hours after methylphenidate administration, persisted for 10 hours, and had completely resolved within 2 days. Although limb dyskinesia after methylphenidate is a commonly reported side effect, to the authors' knowledge this is only the second reported case to develop both orofacial and limb dyskinesia in the acute period after the first dose of methylphenidate. This case is reported to emphasize the potential side effects of methylphenidate, individual differences in drug sensitivities, and drug-receptor interactions via different mechanisms.

Long-term follow-up of cervical dystonia patients treated with botulinum toxin A.

We followed the course in 100 consecutive patients with cervical dystonia (CD) after they were initially treated with botulinum toxin (BTX) in the form of Dysport 10 to 12 years ago. A total of 4 patients had died, and 6 were lost to follow-up. Of the remaining 90 patients, 57 (63%) were still treated with BTX. In the patients treated at one centre over the whole period with Dysport, mean dose used during each treatment session was 833 (SD +/- 339) units Dysport with a cumulative dose of 20,943 (SD +/- 9462) units Dysport over a mean of 26.8 (SD +/- 8.6) treatment cycles. Secondary nonresponse was detected in 3 of the 90 patients. During follow-up, 12 patients developed blepharospasm, 13 oromandibular dystonia, and 17 patients writer's cramp. We conclude that BTX remains effective and safe for approximately 60% of CD patients for more than 10 years.

Meige syndrome with apraxia of lid opening after the discontinuation of sulpiride treatment.

We report a case of Meige syndrome with apraxia of lid opening that lasted for about seven months after discontinuation of sulpiride treatment. To our knowledge, this is the first report demonstrating that Meige syndrome with apraxia of lid opening is induced by sulpiride, and that the condition persists.

Effect of clonazepam treatment on antipsychotic drug-induced Meige syndrome and changes in plasma levels of GABA, HVA, and MHPG during treatment.

We demonstrated the effect of clonazepam (2 mg/day) on Meige syndrome in two schizophrenic patients under continuous treatment with antipsychotic drugs, and changes in the plasma levels of gamma-aminobutyric acid (GABA), homovanillic acid (HVA), and 3-methoxy-4-hydroxyphenylglycol (MHPG) in these cases. The plasma levels of HVA and MHPG during treatment with clonazepam were decreased in the responder, while not changed in the non-responder to clonazepam. A difference between the responder and the non-responder was not found in the plasma GABA levels. These results suggest that hyperactivities of the central dopaminergic and noradrenergic neurones are involved in the pathophysiology of Meige syndrome.

Tardive blepharospasm.

I report on five patients with tardive blepharospasm seen in a movement disorders clinic, out of 25 tardive dystonia patients. They were young (aged 25-50 yrs); four were men and three had a schizophrenic disorder. The onset was gradual while on maintenance neuroleptics in four and on withdrawal in the fifth. There were no significant antecedent events precipitating the disorder. The disorder was bilateral but asymmetric in two cases. Dyskinetic blinking was often an initial feature and tended to persist after the resolution of the blepharospasm. Orolingual dyskinesia was present in one case and tardive akathisia in two other cases. The symptoms fluctuated in severity with a number of exacerbating and relieving factors. Reduction of neuroleptic dose led to improvement with complete reversal in one of two patients who could be withdrawn off neuroleptic medication. These reports suggest that TB, although uncommon, can be a disabling disorder that may improve considerably with the cessation or dose reduction of the neuroleptic drugs. Its treatment and longitudinal course should be further examined.

Neuroleptic-induced Meige's syndrome following akathisia: pharmacologic characteristics.

A 52-year-old schizophrenic patient acutely showed blepharospasm and oromandibular dystonia following neuroleptic-induced akathisia. She had suffered from schizophrenia and been treated with neuroleptics for 15 years and had manifested tardive dyskinesia 5 years ago. Following a change in her neuroleptic medication, severe akathisia developed. Two days after the appearance of akathisia, blepharospasm and oromandibular dystonia appeared. After the disappearance of akathisia, the disorder continued. The frequency of blepharospasm ranged from 30 to 40 (times/min). The oral administration of trihexyphenidyl (6 mg/day), perphenazine (12 mg/day), and fluphenazine (12 mg/day) significantly decreased the frequency of blepharospasm, whereas carbamazepine (600 mg/day) and sulpiride (1200 mg/day) did not. These results suggest that overactivity of both cholinergic and dopaminergic functions in the striatum may be involved in this patient. Our patient, who showed acute onset of Meige's syndrome following neuroleptic-induced akathisia, is of interest to those studying the pathogenesis of Meige's syndrome.

Drug-associated facial dyskinesias--a study of 238 patients.

UNLABELLED: The purpose of this study was to determine whether antidepressant, antimania, antipsychotic, antihistamine, or antiparkinsonian drugs are associated with eyelid and facial dyskinesias; whether discontinuing such drugs results in improvement in the facial dyskinesias; and whether response to botulinum toxin treatment is influenced by such medications. METHODS: A retrospective review was performed on a population of 238 patients with presumed benign essential blepharospasm and Meige syndrome. Types of drugs taken before the development of disease and clinical response to botulinum toxin injections were studied. RESULTS: Fourteen of 238 patients (5.9%) with facial dyskinesias had been prescribed a variety of antidepressants, antimania medications, antipsychotics, antihistamines, antiparkinsonian drugs, or a combination of these substances before their condition developed. The onset of blepharospasm varied from 2 months to 35 years after administration of the drug. Three of seven patients who discontinued the presumed responsible drug had improvement in their facial dyskinesias. Of the 11 patients who did not improve when their drugs were stopped or whose medication could not be stopped, all but one patient had a good response to treatment with botulinum toxin A. CONCLUSIONS: Drug-induced blepharospasm should be considered in all patients who present with facial dyskinesias, and such patients should undergo withdrawal of the medication when possible. When withdrawal of medication is not possible or does not result in improvement in the facial dyskinesia, treatment with botulinum toxin injections should be initiated. The possible role in the production of facial dyskinesias of antidepressants that block reuptake of serotonin requires further evaluation.

A unique case of tardive dystonia induced by short-term therapy with perazin.

Perazin is a piperazine derivative of phenothiazine with higher affinity for the D2 than the D1 receptor. We observed a 43-year-old woman who developed blepharospasm and oral hyperkinesia as a tardive dystonic syndrome after short-term treatment with perazin. She had never taken any other neuroleptic medication and all known forms of secondary dystonia were ruled out. We were unable to find any previous reports of perazin-induced tardive dystonia.