Idiopathic dystonia clinical, profile of 76 brazilian patients

Andrade, Luiz Augusto Franco de; Ferraz, Henrique B.

doi:10.1590/s0004-282x1992000400003

Cited by 14 publications

(14 citation statements)

References 26 publications

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“…The pattern of CD spread in our group of patients was noteworthy and somewhat different from previous reports that showed a predilection for spread to the cranial segment or to the upper limb 6,7,12 . In fact, most of patients spreaded to cranial segment, but we found a high prevalence of generalized spreading (28% of patients).…”

Section: Discussioncontrasting

confidence: 99%

Retrocollis, anterocollis or head tremor may predict the spreading of dystonic movements in primary cervical dystonia

Godeiro-Júnior

Felı́cio

Aguiar

et al. 2009

Arq. Neuro-Psiquiatr.

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-Background and Purpose: Few studies have attempted to develop clinical predictors for cervical dystonia (CD) aiming at progression of the dystonic movement. Method: We retrospectively evaluated 73 patients with primary CD who underwent treatment with Botulinum toxin type-A (BTX-A). The patients were assembled in two groups according to the spread of dystonia during follow-up: spreading and non-spreading CD. We performed a binary logistic regression model using spreading of cervical dystonia as dependent variable aiming to find covariates which increase the risk of spreading. Results: Our logistic regression model found the following covariates and their respective risk ratios: time of disease >18.5 months=2.4, retrocollis=1.9, anterocollis=1.8, head tremor=1.6. Conclusion: Time of disease >18.5 months, retrocollis, anterocollis and head tremor may predict spreading of dystonic movement to other regions of the body in CD patients.Key WOrDs: cervical dystonia, progression, retrocollis, anterocollis, head tremor. Retrocolis, anterocolis ou tremor cefálico podem predizer a progressão dos movimentos distônicos em pacientes com distonia cervical primáriaResumo -Introdução: Poucos estudos avaliam preditores clínicos de progressão dos movimentos distônicos, para além da região cervical, em pacientes com distonia cervical (DC) primária. Método: Avaliamos, retrospectivamente, 73 pacientes com DC primária, que tinham sido submetidos ao tratamento com a toxina botulínica tipo A (BTX-A). estes pacientes foram divididos em dois grupos de acordo com a progressão ou não da DC para outras áreas do corpo. Aplicamos um modelo de regressão logística binária usando a progressão da distonia como variável dependente com o objetivo de identificar co-variáveis que aumentassem o risco de progressão. Resultados: O modelo de regressão logístico identificou as seguintes co-variáveis como preditoras de progressão e seus respectivos índices de risco: tempo de doença >18,5 meses=2,4, retrocolis=1,9, anterocolis=1,8, tremor cefálico=1,6. Conclusão: Tempo de doença >18,5 meses, retrocolis, anterocolis, e tremor cefálico podem predizer a progressão do movimento distônico para outras regiões do corpo em pacientes com DC primária.PAlAvrAs-ChAve: distonia cervical, progressão, retrocolis, anterocolis, tremor cefálico.

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Section: Discussioncontrasting

confidence: 99%

Retrocollis, anterocollis or head tremor may predict the spreading of dystonic movements in primary cervical dystonia

Godeiro-Júnior

Felı́cio

Aguiar

et al. 2009

Arq. Neuro-Psiquiatr.

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“…Prevalence data on secondary dystonia are limited. A Brazilian study of 122 patients with a dystonic syndrome, 46 (38%) were found to have a symptomatic form 2. Among these, the most frequent causes were tardive dystonia (35%) and perinatal cerebral injury (30%).…”

Section: Resultsmentioning

confidence: 99%

Secondary Dystonia-Clinical Clues and Syndromic Associations

Schneider¹,

Bhatia

2009

JMD

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Background:Dystonia is a hyperkinetic movement disorder defined by involuntary sustained muscle spasms and unusual postures. Etiologically, dystonic syndromes can be broadly divided into primary and secondary forms, dystonia-plus syndromes and heredodegenerative forms. In particular, diagnosis of secondary dystonic syndromes can be challenging in view of the variety of causes.Purpose:The purpose of this article is to highlight some clinical clues and syndromic associations as well as investigational findings which may be helpful in the approach to a patient with suspected secondary dystonia.Methods:We outline characteristic clinical and neuroimaging findings which may be directive in the diagnostic process of dystonia patients and facilitate making the correct diagnosis, thus allowing initiating the best treatment.Results:Secondary causes of dystonia include, among others, strategic brain lesions of various origins, metabolic disease, neurodegenerative conditions, and previous exposure to drugs or toxins. Presence of clinical signs including prominent oromandibular involvement, eye movement disorders, retinitis pigmentosa, deafness, peripheral neuropathy, parkinsonism or progressive dementia should alert the clinician to consider a secondary cause. Strategic lesions within the basal ganglia, but also within the brainstem, cerebellum or cortical areas may underlie dystonia and should thus be excluded.Conclusions:When thorough clinical examination reveals features atypical of primary dystonia, syndromic associations may help the clinician to narrow down the list of differential diagnosis. Directive investigations like neuroimaging may confirm the clinical suspicion.

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“…A ocorrência, praticamente igual, tanto no sexo masculino quanto no feminino, está de acordo com o encontrado na literatura 2,7,17 ; no entanto, em nosso meio, Andrade e Ferraz 2 , analisando 76 pacientes com distonia idiopática, encontraram maior incidência no feminino. Quanto à distribuição das distonias, também como mostra a literatura 717 , quase a metade (46,9%) era focal (Fig 1).…”

Section: Discussionunclassified

Distonias: aspectos clínicos e terapêuticos em 64 pacientes

Mattos

Rosso

Novis

1996

Arq. Neuro-Psiquiatr.

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RESUMO -Os Autores apresentam a experiência em 64 pacientes, com as várias formas clínicas de apresentação de distonias, acompanhados no Setor de Doenças Extrapiramidais do Serviço de Neurologia do Hospital Universitário Clementino Fraga Filho da UFRJ, assim como, revisam a literatura, cotejando os resultados. O acompanhamento desses pacientes durante 5 anos e 6 meses resultou nas seguintes observações: 33 do sexo masculino e 31 do feminino; 48 brancos, 10 pardos e 6 negros; a média do tempo de doença foi 9 anos e 8 meses. Quanto à distribuição do movimento anormal, 30 (46,9%) eram focais; 17 (26,6%), segmentares; 13 (20,3%), generalizadas; 3 (4,7%), hemidistonias; 1 (1,5%), multifocal. Quanto à idade de início, em 11 (17,2%) se apresentou antes dos 12 anos; em 6 (9,4%), entre 13 e 20 anos; e em 47 (73,4%), após os 20 anos. Correspondiam à origem idiopática esporádica, 39 (60,9%); idiopática familiar, 6 (9,4%); sintomática, 19 (29,7%). No que se refere à abordagem terapêutica destes pacientes, destacamos o emprego de anticolinérgicos, de agonistas e antagonistas dopaminérgicos e do baclofen isolado ou associado aos anticolinérgicos para as formas generalizadas. Para as distonias focais, os Autores concluem ser a toxina botulínica do tipo A o agente terapêutico mais eficaz aconselhado atualmente. PALAVRAS-CHAVE: distonia, distonia musculorum deformans, toxina botulínica Dystonias: clinical and therapeutic features in 64 patientsABSTRACT -The experience with 64 patients with dystonia seen at the Extrapyramidal Diseases Sector of the Neurology Department of the Hospital Universitário Clementino Fraga Filho of the UFRJ is presented as well as the pertinent review of the literature. The five-and-a-half-year of follow-up showed that 33 were male and 31 female; 48 were white, 10 mulatto and 6 negro; the mean time of disease was 9 years and 8 months. According to the distribution of the movement disorder, 30 (46.9%) were focal, 17 (26.6%) segmental, 13 (20.3%) generalized, 3 (4.7%) hemidystonia and 1 (1.5%) multifocal. In 11 (17.2%) the age of onset was before 12 years old, in 6 (9.4%) between 13 and 20 years old, and in 47 (73.4%) after 20 years old. According to the etiology, 39 (60.9%) were idiopathic sporadic, 6 (9.4%) were idiopathic familial and 19 (29.7%) were symptomatic. The therapeutical approach used in these patients were anticholinergic drugs, dopaminergic agonists or antagonists and baclofen, alone or associated with anticholinergic drugs for generalized dystonia. The authors conclude that botulinum toxin type A is the most valuable therapeutic agent in the treatment of focal dystonia.

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Idiopathic dystonia clinical, profile of 76 brazilian patients

Cited by 14 publications

References 26 publications

Retrocollis, anterocollis or head tremor may predict the spreading of dystonic movements in primary cervical dystonia

Retrocollis, anterocollis or head tremor may predict the spreading of dystonic movements in primary cervical dystonia

Secondary Dystonia-Clinical Clues and Syndromic Associations

Distonias: aspectos clínicos e terapêuticos em 64 pacientes

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