Factors influencing the efficacy of initial adrenocorticotropic hormone therapy for infantile epileptic spasms syndrome / 中国当代儿科杂志

OBJECTIVES@#To study the factors influencing the short-term (28 days) efficacy of initial adrenocorticotropic hormone (ACTH) therapy for infantile epileptic spasms syndrome (IESS), as well as the factors influencing recurrence and prognosis.@*METHODS@#The clinical data were collected from the children with IESS who received ACTH therapy for the first time in the Department of Pediatric Neurology, Xiangya Hospital of Central South University, from April 2008 to January 2018 and were followed up for ≥2 years. The multivariate logistic regression analysis was used to evaluate the factors influencing the short-term efficacy of ACTH therapy, recurrence, and long-term prognosis.@*RESULTS@#ACTH therapy achieved a control rate of seizures of 55.5% (111/200) on day 28 of treatment. Of the 111 children, 75 (67.6%) had no recurrence of seizures within 12 months of follow-up. The possibility of seizure control on day 28 of ACTH therapy in the children without focal seizures was 2.463 times that in those with focal seizures (P<0.05). The possibility of seizure control on day 28 of ACTH therapy in the children without hypsarrhythmia on electroencephalography on day 14 of ACTH therapy was 2.415 times that in those with hypsarrhythmia (P<0.05). The possibility of recurrence within 12 months after treatment was increased by 11.8% for every 1-month increase in the course of the disease (P<0.05). The possibility of moderate or severe developmental retardation or death in the children without seizure control after 28 days of ACTH therapy was 8.314 times that in those with seizure control (P<0.05). The possibility of moderate or severe developmental retardation or death in the children with structural etiology was 14.448 times that in those with unknown etiology (P<0.05).@*CONCLUSIONS@#Presence or absence of focal seizures and whether hypsarrhythmia disappears after 14 days of treatment can be used as predictors for the short-term efficacy of ACTH therapy, while the course of disease before treatment can be used as the predictor for recurrence after seizure control by ACTH therapy. The prognosis of IESS children is associated with etiology, and early control of seizures after ACTH therapy can improve long-term prognosis.

Neuromielitis óptica y lupus eritematoso sistémico en un hombre: caso clínico / Neuromyelitis optica presenting concomitantly with systemic lupus erythematosus: report of one case

Neuromyelitis optica (NMO) is a severe demyelinating disease of the central nervous system, which preferentially attacks the optic nerve and spinal cord. It is associated with antibodies against aquaporin 4. Morbidity and mortality are higher than in multiple sclerosis and its treatment focuses on immunosuppressive drugs. Immunomodulators are contraindicated. We report a previously healthy 35-year-old man, presenting with NMO concomitantly with systemic lupus erythematosus. His evolution was torpid with three outbreaks in the 10 months after the diagnosis, requiring a first-line therapy with methylprednisolone and cyclophosphamide and then a second-line therapy with rituximab.

Tonic spasms are a common clinical manifestation in patients with neuromyelitis optica / Espasmos tônicos são manifestações clínicas frequentes em pacientes com neuromielite óptica

Tonic spasms have been most commonly associated with multiple sclerosis. To date, few reports of series of patients with neuromyelitis optica and tonic spasms have been published. Methods: We analyzed the characteristics and frequency of tonic spasms in 19 subjects with neuromyelitis optica. Data was collected using a semi-structured questionnaire for tonic spasms, by both retrospectively reviewing medical records and performing clinical assessment. Results: All patients except one developed this symptom. The main triggering factors were sudden movements and emotional factors. Spasms were commonly associated to sensory disturbances and worsened during the acute phases of the disease. Carbamazepine was most commonly used to treat the symptom and patients showed good response to the drug. Conclusions: Tonic spasms are a common clinical manifestation in patients with neuromyelitis optica. .

Espasmos tônicos têm sido mais frequentemente associados com esclerose múltipla. Foram publicados até agora poucos relatos de série de pacientes com neuromielite óptica e espasmos tônicos. Métodos: Foram analisadas as características e a frequência de espasmos tônicos em 19 indivíduos com neuromielite óptica. Os dados foram coletados por meio de um questionário semiestruturado para espasmos tônicos, mediante a avaliação retrospectiva dos prontuários e a análise dos dados clínicos Resultados: Todos os pacientes com neuromielite óptica exceto um apresentaram espasmos tônicos. Os principais fatores desencadeantes foram movimentos bruscos e fatores emocionais. Espasmos foram frequentemente associados a perturbações sensoriais e se agravaram durante a fase aguda da doença. A carbamazepina foi utilizada frequentemente para tratar os sintomas, com boa resposta. Conclusões: Os espasmos tônicos são manifestações clínicas frequentes em pacientes com neuromielite óptica. .

Botulinum toxin: Bioweapon & magic drug.

Botulinum neurotoxins, causative agents of botulism in humans, are produced by Clostridium botulinum, an anaerobic spore-former Gram positive bacillus. Botulinum neurotoxin poses a major bioweapon threat because of its extreme potency and lethality; its ease of production, transport, and misuse; and the need for prolonged intensive care among affected persons. A single gram of crystalline toxin, evenly dispersed and inhaled, can kill more than one million people. The basis of the phenomenal potency of botulinum toxin is enzymatic; the toxin is a zinc proteinase that cleaves neuronal vesicle associated proteins responsible for acetylcholine release into the neuromuscular junction. As a military or terrorist weapon, botulinum toxin could be disseminated via aerosol or by contamination of water or food supplies, causing widespread casualties. A fascinating aspect of botulinum toxin research in recent years has been development of the most potent toxin into a molecule of significant therapeutic utility. It is the first biological toxin which is licensed for treatment of human diseases. In the late 1980s, Canada approved use of the toxin to treat strabismus, in 2001 in the removal of facial wrinkles and in 2002, the FDA in the United States followed suit. The present review focuses on both warfare potential and medical uses of botulinum neurotoxin.

Uso de manometria computadorizada para estudo do espasmo do segmento faringoesofágico em pacientes com voz traqueoesofágica inadequada antes e após aplicação de toxina botulínica / Computerized manometry use to evaluate spasm in pharyngoesophageal segment in patients with poor tracheoesophageal speech before and after treatment with botulinum toxin

Voz traqueoesofágica (VTE) com prótese fonatória (PF) é método eficaz e reproduzível na reabilitação vocal após laringectomia total (LT), impedida pelo espasmo do segmento faringoesofágico (SFE). A manometria computadorizada (MC) é novo método objetivo e direto de avaliação do SFE. OBJETIVO: Análise objetiva do espasmo do SFE, com MC, antes e após aplicação de toxina botulínica (TB). DESENHO DO ESTUDO: Prospectivo clínico. MATERIAL E MÉTODOS: Análise de oito pacientes consecutivos submetidos à LT com VTE e PF, sem emissão vocal, com espasmo do SFE à videofluoroscopia, considerado padrão ouro para detecção de espasmo. Todos trataram o espasmo com injeção de 100 unidades de TB no SFE. Avaliação constituiu-se de videofluoroscopia e MC do SFE, antes e após aplicação de TB. RESULTADOS: Houve diminuição na pressão do SFE à MC, após injeção de TB em todos. A média de pressão do SFE à MC, nos oito pacientes, antes da aplicação de TB foi de 25.36 mmHg e após foi de 14.31 mmHg (p=0,004). Houve emissão vocal sem esforço e melhora do espasmo do SFE à videofluoroscopia após o uso da TB. CONCLUSÃO: Foi observada diminuição na pressão do SFE após injeção da TB à MC em todos os pacientes, com melhora do espasmo à videofluoroscopia.

Tracheoesophageal voice (TEV) with voice prosthesis (VP) is an efficient and reproducible method used in vocal rehabilitation after total laryngectomy (TL), prevented by spasms in the pharyngoesophageal segment (PES). Computerized Manometry (CM) is a new, direct and objective method used to assess the PES. AIM: to carry out an objective analysis of the PES, with CM, before and after the injection of botulinum toxin (BT). STUDY DESIGN: clinical-prospective. MATERIALS AND METHODS: analysis of eight patients consecutively submitted to TL with TEV and VP, without vocal emission, with PES spasms seen through videofluoroscopy, considered the gold standard for spasm detection. All had their spasms treated with the injection of 100 units of BT in the PES. The assessment was based on PES videofluoroscopy and CM, before and after BT injection. RESULTS: There was a PES pressure reduction according to the CM after BT injection in all patients. The average pressure in the PES seen through the CM in eight patients before BT injection was 25.36 mmHg, and afterwards it dropped to 14.31 mmHg (p=0.004). There was vocal emission without stress and PES spasm improvement seen through the videolaryngoscopy after BT injection. CONCLUSION: We observed a reduction in PES pressure after BT injection, seen through CM in all the patients, with spasms improvement seen through videofluoroscopy.

Síndrome de Satoyoshi: Enfermedad multisistémica con respuesta exitosa a tratamiento esteroidal / Satoyoshi syndrome: Report of one case

Satoyoshi  syndrome is a rare multisystemic disease of presumed autoimmune etiology characterized by progressive painful intermittent muscle spasms, diarrhea frequently associated with malabsorption, alopecia, skeletal abnormalities and endocrine disorders with a poor long-term prognosis due to early crippling. We report a 14-year-old Chilean girl with clinical and radiological features of the syndrome who has been successfully treated with prednisone and carbamazepine. She remarkably recovered from muscle spasms, alopecia and diarrhea. At follow up, 24 months later, she persists asymptomatic with considerable improvement in her quality of life.

Treatment of infantile spasms with sodium valproate followed by benzodiazepines.

OBJECTIVE: To review the result of the infantile spasms' treatment with sodium valproate followed by nitrazepam or clonazepam. STUDY DESIGN: Descriptive retrospective study. SETTING: Srinagarind Hospital, Department of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. MATERIAL AND METHOD: Twenty-four infantile spasms admitted between January 1994 and December 2003 were analyzed. The inclusion criteria were the patients with infantile spasms clinically diagnosed by the pediatric neurologist, having hypsarrhythmic pattern EEG, and receiving sodium valproate with or without nitrazepam or clonazepam. The patients who had an uncertain diagnosis, incomplete medical record, or that were incompletely followed up were excluded. Data were collected on sex, age at onset of seizure, type of infantile spasms, associated type of seizure, predisposing etiological factor, neuroimaging study, and the result of treatment including cessation of spasms, subsequent development of other seizure types, quantitative reduction of spasms, relapse rates of spasms, psychomotor development, and adverse effects of AEDs. RESULTS: The mean age at onset was 177 days. The male-to-female ratio was 1:1.2. There were 13 cryptogenic (54.2%) and 11 symptomatic (45.8%) infantile spasms. The most common predisposing etiological factors in symptomatic cases were hypoxic ischemic encephalopathy (45.5%) and microcephaly (36.4%), respectively. Ten patients received sodium valproate (41.7%), another 10 received sodium valproate with clonazepam (41.7%), and four received sodium valproate with nitrazepam (16.7%). Both, the complete cessation rate and the 50% reduction of spasms rate were 45.8%. The duration to complete cessation was 70 days. The relapse rate was 18.2%. The rate of delayed psychomotor development was 83.3%. The mean duration of follow-up was 49.6 months. CONCLUSION: The authors propose to use sodium valproate concomitantly with benzodiazepines, especially clonazepam, in situations such as unavailability, intolerability, or adverse effects of ACTH or vigabatrin, or in a patient who does not respond to ACTH or vigabatrin.

Botulinum toxin injection for treatment of spasmodic dysphonia: experience at Srinagarind Hospital.

BACKGROUND: Spasmodic dysphonia (SD) is a focal dystonia and adductor SD is the most common form. The standard treatment for adductor SD is EMG-guided, transcutaneous injections of botulinum toxin into the thyroarytenoid muscle. OBJECTIVE: Report the clinical presentation of SD, treatment with botulinum toxin injection, injection technique, results, and adverse effects. MATERIAL AND METHOD: A reviewed of clinical records of patients diagnosed with SD at the Voice Clinic between April 1999 and December 2004 at Srinagarind Hospital, Khon Kaen University, Thailand. Thirty-seven patients were identified but only twenty-five were treated with EMG-guided botulinum toxin injection to the thyroarytenoid muscle. RESULTS: In the presented 37 patients, SD was more common in women (89%) than men (11%). The median duration of symptoms prior to diagnosis was 12 months: all were the adductor type. The average age at onset was 46 years. The presenting symptoms included influence to the voice (84%), hoarseness (70%), strained or strangled voice (65%), decreased loudness (27%), and breathy voice (22%). A vocal tremor coexisted with dystonia in 60% of the presented patients. Treatment with botulinum toxin injection was carried out on 25 patients for 78 injections (mean, 3 per patient). The time for botulinum toxin to take effect averaged 2.3 days (peak effect, 7 days). The patients received substantial relief from their SD symptoms, an average functional improvement of 39.2% (37.6% initially vs. 76.8% finally). Patients' best voice was achieved within one week and persisted for an average of 13.6 weeks. Side effects from the injections included mild breathiness (68%) and mild choking on fluid (56%). After injection, decreased potential for volume was a common complaint, but since all of the patients experienced increased fluency, they were satisfied. Almost all of the patients returned for repeat injections when the benefit diminished. CONCLUSION: Botulinum toxin therapy has become the standard care for the treatment of SD. An acceptable and flexible treatment plan to produce a balance between decreased spasms and loss of function must be developed for each patient.

Aicardi syndrome: a report of five Indian cases.

Five Indian cases of Aicardi syndrome, aged 1-13 years and all with the classic triad of infantile spasm, corpus callosal agenesis and chorioretinal lacunae, were presented. The purpose of this report was to demonstrate the clinical, electroencephalographic (EEG) and radiological spectrum and the treatment outcome of this disorder among the Indians. None of the patient had any family history of similar illness. All had profound psychomotor impairment with no meaningful speech development. There were microphthalmia in 2, optic disc coloboma in 1, interhemispheric cyst in 1, periventricular heterotopia in 2 and thoracolumbar kyphoscoliosis in 2 cases. They all had early onset epilepsy and there were multiple types of seizures. Two cases (40%) had the characteristic dissociated burst-suppression pattern in EEG. Two cases whose antiepileptic medications included vigabatrin had complete control of seizure.

Neuroparalysis and ventilatory support in severe tetanus.

In severe form of tetanus, even with maximum dose of muscle relaxants, spasms and apnoeic spells may persist and that may be life-threatening. The aim of this study was to assess the effect of neuroparalysing the patients and then providing ventilatory support in bringing about their recovery. Forty-nine adult patients of severe tetanus (Ablett's grade IIIA--6 patients and Ablett's grade IIIB--43 patients) were studied during the period from April, 1993 to February, 1996. Mean period of onset ie, period from trismus to first spasm, in these patients was 24 hours. Patients were neuroparalysed with a bolus dose of 2-4 mg of pancuronium followed by a continuous infusion of 1-2 mg/hour and simultaneously supported with mechanical ventilation until spasms subsided. Fourteen patients (28.6%) survived and rest died. Mean duration of ventilatory support on survived patients was 14.4 days. The commonest complication encountered during ventilatory support was respiratory tract infection observed in 36 patients (73.5%). Commonest cause of death was autonomic imbalance encountered in 15 patients (30.6%). Treatment of choice in severe tetanus should be neuroparalytic ventilatory support. With use of new generation ventilators and better intensive care facility, death in severe tetanus is likely to be very less.

[Spasmolytic effect of zataria multiflora boiss leaf extract on rat uterus]

Zataria multiflora Boiss is used in traditional medicine to treat gastrointestinal disorders and menrrhalgia. The inhibitory effect of this herb on rat ileuni contractions has also been reported. The aim of this study was to investigate the effect of Zataria multiflora Boiss hydroalcoholic leaf extract [ZHLE] on isolated rat uterus in the presence of some known uterus stimulants. Pieces of virgin adult rat uterus were mounted in an organ bath containing Tyrode or De Jalon solutions. Uterus contractions were induced by KCI, oxytocin and BaC1[2] in presence and absence of ZHLE. Animals in oxytocin studies received an injection of oestradiol valerate [5mg/kg, s.c.] 24 h prior experiment. ZHLE [0.125, 0.25, 0.5, 1 and 2 mg/ml] relaxed the uterus precontracted by KC1 [60mM] in a dose-dependent manner [p<0.0001] and at 2mg/mi attenuated the BaC1[2] [4mM]-induced uterus contraction significantly [p<0.001]. The inhibitory effect of ZLHE on KC1-induced uterus contraction was unaffected by propranolol [1 micro M]. in normal IDe Jalon solution, ZHLE [0.125, 0.25, 0.5, and 1 mg/ml] reduced the oxytocin [10mU/ml]-induced contraction dose-dependently [p<0.0001] but in Ca[2+]-free Dc Jalon solution, the stimulatory effect of oxytocin was weaker and also the inhibitory effect of ZHLF was more consistent. In presence of atropine [0.5jiM], acetylcholine [0.5jiM] failed to induce contraction but KC1 [30mM]-evoked contraction and extract diminished the contractile response of KC1. The spasmolytic effect of extract [2mg/ml] on KC1-induced contraction was unaffected by naloxone [1 micro M]. From the obtained results it may be concluded that, the ZHLE may induce its inhibitory effect through blockage of the voltage dependent calcium channels and releasing calcium from intracellular stores in rat uterus smooth muscle. The ineffectiveness of propranolol and naloxone on ZLHE inhibitory effect indicates that adrenergic and opoids agonist substance[s] did not exist in the extract. it seems that there was no anticholinergic substance[s] in the extract. The results support the usage of this plant in traditional medicine

Modulatory effect of diclofenac on antispasmodic effect of pitofenone in cholinergic spasm.

Biliary, ureteric and intestinal colic are extremely common clinical conditions associated with smooth muscle spasm. In the present study, antispasmodic activity was carried out against acetylcholine (10-640 ng/ml)-induced contractions on guinea pig ileum. Acetylcholine (10-640 ng/ml) induced concentration-dependent contraction of smooth muscle. Diclofenac, in varying concentration (9.4 x 10(-5) mol/l and 14.1 x 10(-5) mol/l) shifted the concentration response curve of acetylcholine to the right without suppressing the maximal response. However, in higher concentration diclofenac (18.9 x 10(-5) mol/l) blocked the response in an unsurmountable fashion. Further, analgin (11.09 x 10(-5), 16.63 x 10(-5) and 22.18 x 10(-5) mol/l) in equimolar concentrations did not alter the concentration response curve of acetylcholine, but in higher concentration analgin (44.36 x 10(-5) mol/l) also blocked the response in an unsurmountable fashion. Pitofenone (2.5 x 10(-6) mol/l) also, shifted the concentration response curve of acetylcholine to right in a parallel fashion with no change in maximal response. The present study confirms the potent antispasmodic activity of diclofenac-pitofenone combination in comparison to analgin-pitofenone in molar equivalent concentration (in comparison to diclofenac) against acetylcholine-induced contractions of guinea pig ileum.

Tratamento do espasmo facial unilateral com toxina botulínica tipo A / Management of hemifacial spasm with botulinum A toxin

Este estudo retrospectivo apresenta a experiência no tratamento do espasmo facial unilateral com toxina botulínica tipo A. Dezenove pacientes receberam 71 aplicaçöes. O índice de sucesso foi de 94,4 por cento. A duraçäo média do efeito foi de 17,7 por cento (ñ7,2) semanas. A incidência de complicaçöes foi de 35,2 por cento e dose dependente, todas elas locais, transitórias e de grau leve a moderado

Tratamiento del espasmo hemifacial y blefaroespasmo con toxina botulínica: resultados a largo plazo / Treatment of hemifacial spasm and blepharospasm whit botulinum toxin

Se revisaron las historias clínicas de 234 pacientes con diagnóstico de blefaroespasmo (BE) (68) y espasmo hemifacial (EH) (166) que fueron infiltrados entre 1987 y 1995 en el Servicio de Neurología del Hospital de Clínicas de la ciudad de Buenos Aires. De los pacientes con EH, 55 fueron varones y 111 mujeres, con una edad promedio de 57,5 años, mientras que 17 varones y 51 mujeres presentaron BE/BE-distonía oromandibular con una edad promedio de 63 años. La edad general de comienzo fue de 56,3 años para los EH y 56,8 para los BE. Trece EH fueron postparalíticos, dos secundarios a tumores de tronco, uno secundario a una lesión vascular protuberancial y uno a enfermedad de Paget, el resto fueron diagnosticados como primarios, aunque en las IRM y TC cerebrales resultó evidente en muchos de ellos la presencia de ectasias en el circuito vertebrovasilar. La dosis promedio en pacientes con EH fué de 21,8 I.U. con un rango entre 12,5 I.U. y 50 u, mientras que la dosis promedio en pacientes con BE fué de 33,1 con un rango entre 25 y 75 u. El número total de infiltraciones fué de 1910 con un promedio de 9,8 para los EH y 5,5 para los BE. La duración promedio del efecto beneficioso de cada dosis fué de 3 meses para ambos grupos. Todos los pacientes se beneficiaron marcadamente (77 por ciento) y moderadamente (27 por ciento) con las infoltraciones aunque el 12,5 por ciento experimentó una ptosis transitoria como efecto colateral en algunas de las aplicaciones. Los pacientes con BE requirieron un incremento progresivo de un 21 por ciento de la dosis de toxina botulínica a lo largo de todo el tratamiento, mientras que en los EH se necesitó un 20 por ciento de aumento. Nuestra experiencia ilustra la utilidad de la toxina botulínica en el manejo del EH y el BE constituyendo un arma terapéutica sencilla y eficaz que debe ser considerada como el tratamiento de elección en este tipo de patología

Toxina botulínica no blefaroespasmo, no espasmo hemifacial e na distonia cervical: resultados em 33 pacientes / Botulinum toxin in blepharospasm, in hemifacial spasm and in cervical dystonia: results in 33 patients

Avaliamos os resultados terapêuticos obtidos com o emprego de toxina botulínica do tipo A em 33 pacientes com distonia (12 com blefaroespamo; 10 com espasmo hemifacial e 11 com torcicolo espasmódico). Utilizamos uma escala de pontuaçäo de gravidade antes de cada aplicaçäo, sendo reavaliados duas semanas após, seguindo a mesma escala. Entre os com blefaroespasmo, oito eram mulheres e quatro homens; a média de idade foi 57,7 anos; a média do tempo de doença de quatro anos; três tinham história similar na família; nove eram essenciais e três fizeram uso de neurolépticos (distonia tardia0. A dose média empregada ficou em 51,3 U, com a duraçäo média do efeito benéfico de 2,8 meses. Do total de 22 aplicaçöes (injeçöes e reinjeçöes), 14 (63,7 por cento) tiveram resultado ótimo, 5 (22,7 por cento) bom e três (13,6 por cento) nulo. Naqueles com espasmo hemifacial, oito eram mulheres e dois homens; a média de idade foi 52,6 anos; a média do tempo de doença 7,4 anos; oito eram essenciais e dois pós-paralíticos. A dose média do efeito benéfico de 3,4 meses. Nos pacientes com distonia cervical, oito eram homens e três mulheres; a média de idade foi 44,2 anos; a média do tempo de doença 12,2 anos; seis eram essenciais, três fizeram uso de neuroléptico e dois tinham história familiar. A dose média empregada ficou em 238,6 U, com a duraçäo média do efeito benéfico de 4,7 meses. Do total de 20 aplicaçöes, 18 (90 por cento) tiveram resultado bom, 1 (5 por cento) regular e 1 (5 por cento) nulo. Ptose palpebral, paresia facial e disfagia foram os efeitos colaterais mais encontrados. Concluímos que a toxina botulínica revelou-se eficas no tratamento destas condiçöes

Espasmo hemifacial: tratamento com toxina botulínica / Hemifacial spasm: treatment with botulinum toxin

Foram avaliados 21 casos de espasmos hemifacial tratados com toxina botulínica tipo A. A média de idade dos pacientes era de 53 anos e, a de duraçäo da doença, de 5 anos. Esses pacientes haviam recebido, previamente, tratamento convencional com anticonvulsivantes ou tratamento cirúrgico (técnicada Janneta) sem resposta satisfátoria. Todos os pacientes apresentaram melhora do quadro e o percentual médio de funçäo avaliado pela "Columbia Clinical Rating Scale" passou de 33 por cento para 90 por cento. O tempo de latência para início do efeito foi, em média de 4 dias (variaçäo de 1 a 8 dias); o pico de melhora doi atingido, em média, após 8 dias (variaçäo de 1 a 45 dias) e a duraçäo total da resposta foi em média de 110 dias (variaçäo de 60 a 330 dias). As complicaçöes observadas foram: lagoftalmo (13), paresia de território inferior da face(5), esquimose palpebral(4), ptose palpebral(1) e diplopia(1). Todas essas complicaçöes foram transitórias, regredindo em periodo que variou de alguns dias a 2 semanas. esses resultados säo semelhantes aos observados na literatura e enfatizam o papel da toxina botulínica como principal opçäo para o tratamento do espasmo hemifacial

Botulinum toxin A in blepharospasm and hemifacial spasm.

We report the first Indian experience of botulinum toxin A in the treatment of blepharospasm and hemifacial spasm. Sixteen patients, 7 with essential blepharospasm, 5 with Meige syndrome and 4 with hemifacial spasm received botulinum toxin A injection. One patient received 3 courses of injections, 2 received 2 courses and the rest received only one course. The effect was observed after a latent period of less than 48 hours in all patients and lasted for a mean of 16.65 weeks. More than 70% improvement occurred after 17/20 injections (85%). Poor response was more often seen when blepharospasm was associated with oromandibular dystonia (2/5 injections). Though the duration of response and subjective score of improvement was best in patients with hemifacial spasm, the numbers were very small for any statistical evaluation. The side effects were local, transient, mild and well tolerated. The commonest side effect was blepharoptosis.

Botulinum a toxin treatment of hemifacial spasm and blepharospasm

We studied the effects of botulinum A toxin in 101 patients with hemifacial spasm and 11 patients with blepharospasm in an open trial and double blind manner. All patients in the open trial and 6 patients in the double blind trial improved after the first injection of botulinum toxin. There was no improvement with placebo. The peak effect ranged from one to 6 days after injection and mean peak effect was 3.6 days in blepharospasm, and 4 days in hemifacial spasm. Of 144 treatments, 98.6% had excellent results, (below grade I). The duration of beneficial effect ranged 11 to 40 weeks (mean 16.5 weeks) in hemifacial spasm and 9 to 30 weeks (mean 14.2 weeks) in blepharospasm. Complications were encountered in 63.4% in hemifacial spasm and 72.7% in blepharospasm. The common side effects were dry eyes, mouth droop, ptosis and lid edema in order of frequency. These side effects were mild and resolved spontaneously in 1 to 3 weeks. Botulinum A toxin therapy is effective and convenient, and the treatment of choice for patients with hemifacial spasm and blepharospasm.

Spasmodic torticollis: medical and botulinum A toxin treatment

The exact pathophysiologic mechanisms of spasmodic torticollis and other idiopathic torsion dystonias remain largely unknown. Thus, a variety of drugs have been used alone or in combination on an empirical basis to treat these disorders, but to date none have efficacy that is proven and consistent. The drugs in use include anticholinergics, benzodiazepines, dopaminergics and dopamine antagonists with variable degrees of clinical improvement. Botulinum toxin A injection treatment for spasmodic torticollis is safe and efficacious with minimal adverse effect. However, it is expensive and beneficial effects are short-lasting. Only when a spasmodic torticollis patient's symptoms are refractory to combined treatment, using various drugs and Botulinum toxin injections, should the patient be considered a candidate for neurosurgical procedures.