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PURPOSE: Information is scarce regarding the comprehensive profile of patients with essential blepharospasm and hemifacial spasm in Brazil. The present study aimed to assess the clinical features of patients with these conditions, followed up in two reference centers in Brazil. METHODS: The study included patients with essential blepharospasm and hemifacial spasm, followed up at the Departments of Ophthalmology at Universidade Federal de São Paulo and Universidade de São Paulo. Apart from demographic and clinical features, past stressful events related to the first symptoms (triggering event), aggravating factors, sensory tricks, and other ameliorating factors for the eyelid spasms were assessed. RESULTS: A total of 102 patients were included in this study. Most patients were female (67.7%). Essential blepharospasm was the most frequent movement disorder [51/102 patients (50%)], followed by hemifacial spasm (45%) and Meige's syndrome (5%). In 63.5% of the patients, the onset of the disorder was associated with a past stressful event. Ameliorating factors were reported by 76.5% of patients; 47% of patients reported sensory tricks. In addition, 87% of the patients reported the presence of an aggravating factor for the spasms; stress (51%) was the most frequent. CONCLUSION: Our study provides information regarding the clinical features of patients treated in the two largest ophthalmology reference centers in Brazil.

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BACKGROUND: Neuroophthalmological phenotypical particularities of SCA3. PHENOMENOLOGY: Eyelid opening apraxia and asymmetrical blepharospasm. EDUCATIONAL VALUE: To illustrate the phenomenology for purposes of education.

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BACKGROUND AND PURPOSE: Sensory tricks are sensory, often but not exclusively tactile, stimuli usually in the body part affected by the movement disorder that produce a meaningful alleviation of dystonia. The frequency and clinical features of sensory tricks in different types of dystonia are poorly studied in the literature. There is no information regarding the presence of a similar phenomenon in HFS. METHODS: We enrolled consecutive patients who had the diagnosis of HFS (26) or the following types of dystonia: cervical dystonia (CD; 21); blepharospasm (BS; 20); and writer's cramp (WC; 10). Patients underwent a structured interview to assess the following items related to sensory trick: presence, type and effectiveness. Statistical analysis used the Verisimilitude Reason test and the significance level was set at 5%. RESULTS: Sensory trick was more frequent in the CD group (81%) when compared with HFS (38.5%; P=0.004) and WC (20%; P=0.001), but there was no statistical difference from the frequency in BS patients (55%; P=0.078). The most common sensory tricks were facial massage (60%) in HFS; to touch the head with the hands (35.3% in CD); to touch the upper eyelid with the index finger and thumb (81.8%) in BS; and to touch the hand with the contralateral hand (100%) in WC. CONCLUSIONS: Sensory tricks are common in all types of focal dystonia herein studied, although they are more frequent in CD. Patients with HFS also often display improvement of the movement disorder with tactile stimulation.

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Botulinum toxin A (BTA) is considered an effective treatment of blepharospasm and hemifacial spasm, but there are few studies to permit a comparison of its different formulations. This prospective, randomized, double-blind study compared Prosigne, a BTA of Chinese origin, with Botox to establish safety, efficacy, and equivalence of doses between those 2 formulations in blepharospasm and hemifacial spasm treatment. Fifty-seven patients participated in this study: 21 blepharospasm (from whom 11 were treated with Botox; and 10, with Prosigne) and 36 hemifacial spasm patients (17 were treated with Botox; and 19, with Prosigne). All patients were similar in age, disease time span, number of previous shots, and time elapsed since the last BTA application. Pain and burning during the injection and the result of the treatment were similar in both groups. There were no systemic adverse events, and the local ones were observed with similar intensity and frequency for both groups. The mean effect time length was similar for both blepharospasm (11.3 weeks for both toxins) and hemifacial spasm patients (12.8 weeks for Botox and 12.9 weeks for Prosigne). In both blepharospasm groups, only the 36-Item Short-Form Health Survey emotional aspects domain showed improvement from baseline after 16 weeks. There were no differences between the 36-Item Short-Form Health Survey scores before and after the treatment of all hemifacial spasm patients. Therefore, it has been concluded that Botox and Prosigne have similar efficacy, safety, and tolerability profiles, so that a dose equivalence of 1:1 may be considered for blepharospasm and hemifacial spasm treatments.

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The common belief that primary dystonia is a purely motor disorder with no anatomical substrate and no other accompanying neurological dysfunction has recently been challenged. In addition, there is increasing evidence that the basal ganglia besides motor control, plays a role in cognitive functioning. However, no systematic cognitive performance evaluation has been carried out in patients with primary blepharospasm (BS), one of the most common forms of adult dystonia. We evaluated a series of 20 patients with primary BS and a group of 17 controls matched by severity of mood symptoms, age, and sex. BS patients performed significantly worse on the Luria sequencing test, Purdue pegboard test, reciprocal coordination, tactile denomination, and reverse visuospatial span and the differences persisted after correction for age, duration of disease, severity of BS, and degree of depression. The Wisconsin card sorting test showed no statistical difference, but BS patients made more errors and more perseverative answers than expected according to population means, whereas the control group performed poorly but within normal parameters. Our findings suggest broad cortical involvement in focal dystonia that is not correlated with the severity or duration of dystonia.

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Essential blepharospasm is a facial dystonia characterized by spontaneous, spasmodic and involuntary contractions of the eyelid muscles. In advanced cases, blepharospasm patients develop severe eyelid spasms that render them functionally blind, socially reclusive, and unable to work or care for themselves. Oculoplastic surgeons frequently have to deal with patients with blepharospasm. The decrease in quality of life caused by this pathology drives all the attention to the resolution of the spasms. However, other conditions may be associated with them and must be kept in mind during the ophthalmological examination. Four patients with essential blepharospasm were diagnosed as glaucomatous during their follow-up at the Oculoplastic Service. All of them showed glaucomatous optic neuropathy and corresponding visual field defect and no clinically apparent secondary cause for their glaucoma. Forced eyelid closure may lead to intraocular pressure peaks. These patients with blepharospasm present repetitive and spasmodic eyelid contractions and the intraocular pressure rise observed during eyelid squeezing could be an additional risk factor for glaucomatous damage. Our case series suggest that patients with blepharospasm should be seriously evaluated for glaucoma.

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Essential blepharospasm is a facial dystonia characterized by spontaneous, spasmodic and involuntary contractions of the eyelid muscles. In advanced cases, blepharospasm patients develop severe eyelid spasms that render them functionally blind, socially reclusive, and unable to work or care for themselves. Oculoplastic surgeons frequently have to deal with patients with blepharospasm. The decrease in quality of life caused by this pathology drives all the attention to the resolution of the spasms. However, other conditions may be associated with them and must be kept in mind during the ophthalmological examination. Four patients with essential blepharospasm were diagnosed as glaucomatous during their follow-up at the Oculoplastic Service. All of them showed glaucomatous optic neuropathy and corresponding visual field defect and no clinically apparent secondary cause for their glaucoma. Forced eyelid closure may lead to intraocular pressure peaks. These patients with blepharospasm present repetitive and spasmodic eyelid contractions and the intraocular pressure rise observed during eyelid squeezing could be an additional risk factor for glaucomatous damage. Our case series suggest that patients with blepharospasm should be seriously evaluated for glaucoma.

Blefaroespasmo essencial é uma distonia facial caracterizada por contrações espontâneas, espasmódicas e involuntárias dos músculos palpebrais, podendo tornar os pacientes funcionalmente cegos. Tais pacientes são geralmente referidos aos médicos oculoplásticos para avaliação e tratamento. Devido à intensidade dos espasmos e ao comprometimento da qualidade de vida, toda a atenção é dirigida à sua resolução e outras condições oculares associadas podem passar despercebidas. Neste estudo, quatro pacientes com blefaroespasmo foram diagnosticados como glaucomatosos durante o seu seguimento no Departamento de Plástica Ocular. As quatro pacientes apresentavam neuropatia óptica glaucomatosa e defeito no campo visual compatível, sem que houvesse nenhuma causa secundária para o glaucoma. O fechamento palpebral forçado causa importante aumento da pressão intra-ocular e estes pacientes com blefaroespasmo, por apresentar contrações espasmódicas e repetitivas das pálpebras, poderiam estar sob risco aumentado de desenvolver glaucoma.

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PURPOSE: To determine the effect of botulinum toxin injection in the eyelid on lacrimal film in patients with facial dystonia. METHODS: Twenty-four patients with essential blepharospasm and hemifacial spasm were submitted to botulinum toxin injection and lacrimal film tests were performed before the application and after seven and thirty days. RESULTS: There was improvement in symptoms of dry eye and rose bengal test, however, the breakup time and Schirmer's test did not show significant variation between pretreatment and after 1 month of follow-up. CONCLUSION: The dry eye symptoms in patients with facial dystonia may be attenuated by botulinum toxin due to its possible inhibitory effect on the orbicular muscle leading to a decrease in lacrimal pump.

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OBJETIVO: Determinar o efeito da toxina botulínica no filme lacrimal em pacientes com distonia facial. MÉTODOS: Foram incluídos 24 pacientes portadores de blefaroespasmo essencial e espasmo hemifacial que receberam aplicação de toxina botulínica tipo A que foram submetidos à propedêutica do filme lacrimal previamente à aplicação e após, com 7 e 30 dias. RESULTADOS: Houve diminuição das queixas de olho seco trinta dias após a aplicação, entretanto, o tempo de ruptura do filme lacrimal e o teste de Schirmer não demonstraram variação significativa entre os períodos pré-tratamento e 1 mês da aplicação. Em relação ao teste de coloração com rosa bengala, todos os olhos que coraram no pré-tratamento, melhoraram na última avaliação. CONCLUSÃO: A injeção de toxina botulínica pode aliviar as queixas de olho seco nos pacientes com distonia facial pela provável ação de inibição do orbicular na sua função de bomba lacrimal.

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Blepharospasm is a focal dystonia characterized by involuntary eye closure due to abnormal contraction of orbicular eyelid muscles. When blepharospasm is associated to the presence of involuntary oromandibular movements, it is termed Meige syndrome. The aim of this study was to investigate the presence of deglutition alterations in patients with concurrent blepharospasm and Meige syndrome. Twenty consecutive patients were studied by video fluoroscopy using a barium technique. The 4 stages of deglutition were investigated. Ninety percent of patients (18 cases) presented deglutition disorders. The more commonly found alterations were premature food drop, 15 cases (83%) and vallecuale residuals, 14 cases (78%). Sixty seven percent of abnormal findings occurred in the third stage of deglutition. Eighty-nine percent of patients (16) presented more than one swallowing alteration. There was a positive and significant correlation between the number of alterations and patient's age or disease duration. Prevalence of swallowing disorders in the healthy elderly population is reported to be 44%. In our series it reached 90%, suggesting that our findings might be related not only with age but also with a more widespread dystonia exceeding the orofacial muscles.

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