De 1986 a 1999, 2460 pacientes HIV-positivos internados foram avaliados em nosso Hospital. Alterações neurológicas foram encontradas em 1053 (42,8%). Neste grupo, 28 (2,7%) exibiam movimentos involuntários, 14 (50%) com parkinsonismo secundário, seis (21,4%) com hemicoréia/hemiballismo, quatro (14,2%) com mioclonias, dois (7,2%) com painful legs and moving toes, um (3,6%) com hemidistonia e um (3,6%) com tremor de Holmes. No grupo com parkinsonismo, 12 eram, provavelmente, secundários ao HIV; um à toxoplasmose mesencefálica e outro desencadeado pela metoclopramida. Todos com hemicoréia/hemiballismo eram homens e estavam relacionados com toxoplasmose nos gânglios da base. Nos quatro pacientes com mioclonia, em dois era generalizada, relacionada, em um, com toxoplasmose e, em outro, com a encefalopatia pelo HIV; nos outros dois era de origem medular. Nos dois pacientes com painful legs and moving toes, ficou demonstrada a neuropatia axonal pela biópsia do nervo periférico. Toxoplasmose da gânglia basal estava relacionada com hemidistonia contralateral. No paciente com tremor de Holmes havia lesões no mesencéfalo e no cerebelo, secundárias à co-infecção pelo bacilo da tuberculose e pelo Toxoplasma gondii. Concluímos que diversos distúrbios do movimento podem ocorrer em pacientes infectados pelo HIV. Costumam estar relacionados às infecções oportunísticas, ao uso de certos medicamentos, às lesões com efeito de massa e, possivelmente, à ação direta ou indireta do HIV.

From 1986 to 1999, 2460 HIV-positive inpatients were seen in our Hospital. Neurological abnormalities were detected in 1053 (42.8%) patients. In this group, 28 (2.7%) had involuntary movements, 14 (50%) with secondary parkinsonism, six (21.4%) with hemichorea/hemiballismus, four (14.2%) with myoclonus, two (7.2%) with painful legs and moving toes, one (3.6%) with hemidystonia and one (3.6%) with Holmes' tremor. The HIV itself (12 patients), toxoplasmosis of the midbrain (1) and metoclopramide-related symptoms (1) were the most probable causes for the parkinsonism. All patients with hemichorea/hemiballismus were men and in all of them toxoplasmosis of the basal ganglia, mostly on the right side, was the cause of the involuntary movements. Generalized myoclonus was seen in two patients and they were due to toxoplasmosis and HIV-encephalopathy respectively; two others presented with spinal myoclonus. The two patients with painful legs and moving toes had an axonal neuropathy. The patient with hemidystonia suffered from toxoplasmosis in the basal ganglia and the patient with Holmes' tremor had co-infection with tuberculosis and toxoplasmosis affecting the midbrain and cerebellum. We conclude that HIV-infected patients can present almost any movement disorder. They can be related to opportunistic infections, medications, mass lesions and possibly to a direct or indirect effect of the HIV itself.

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.

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.

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 pontuação de gravidade antes de cada aplicação, 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 tardia). A dose média empregada ficou em 51,3 U, com a duração média do efeito benéfico de 2,8 meses. Do total de 22 aplicações (injeções e reinjeções), 14 (63,7%) tiveram resultado ótimo, 5 (22,7%) bom e três (13,6%) 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-páralíticos. A dose média empregada ficou em 32 U. Do total de 15 aplicações, todos (100%) tiveram resultado ótimo, com a duração 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 duração média do efeito benéfico de 4,7 meses. Do total de 20 aplicações, 18 (90%) tiveram resultado bom, 1 (5%) regular e 1 (5%) nulo. Ptose palpebral, paresia facial e disfagia foram os efeitos colaterais mais encontrados. Concluímos que a toxina botulínica revelou-se eficaz no tratamento destas condições.

The effects of botulinum toxin type A were studied in 33 patients with dystonia (12 blepharospasms, 10 hemifacial spasms and 11 spasmodic torticollis). A rate scale was used to evaluate the severity of the dystonic movements, before and two weeks after each injection. Among blepharospasm patients, eight were female and four were male; the mean age was 57.7 years; the mean time of the disease duration was four years. Three had familial history for similar disease; nine were essential and three had used neuroleptic drugs (tardive dystonia). The mean dose used was 51.3 U, with a mean time of benefical effects of 2.8 months. For 22 injections and reinjections, 14 (63.7%) showed an excellent result, five (22.7%) good and three (13.6%) null. In the hemifacial spasm group, eight were female and two male; the mean age was 52.6 years; the mean time of the disease duration was 7.4 years; eight were essential and two post-paralytic. The mean dose used was 32 U. From the total of 15 injections and reinjections, all of them (100%) had an excellent result, with a mean time of benefical effect of 3.4 months. Among the cervical dystonic patients, eight were male and three female; the mean age was 44.2 years; the mean time of the disease duration was 12.2 years; six had essential dystonia, three had used neuroleptic drugs and two had familial history for similar disease. The mean dose used was 238.6 U, with the mean duration of effect of 3.5 months. From the total of 20 injections and reinjections, 18 (90%) had good result, one (5%) mild and one (5%) null. Ptosis, facial palsy and dysphagia were the most common side effects seen. We conclude that botulinum toxin is effective for the treatment of such disorders.

Os autores revisam a literatura a propósito dos tiques e da síndrome de Gilles de la Tourette, abordando os aspectos históricos, etiopatogênicos, neuropatológicos, semiológicos, clínicos e terapêuticos.

The concept of tic was developed at the end of the XIX century, emerging from the "chaos of choreas". Tic is defined as involuntary contractions of agonist and antagonist muscles in one or more parts of the body. It can be suppressed by voluntary efforts for seconds or hours, followed by exacerbations. Gilles de la Tourette's original article was published in 188S, in which he described nine patients with tics, and vocalisations.The pathogenesis of Gilles de la Tourette syndrome remained obscure. However, three factors have been considered: the neurochemical factor, related to the increased dopaminergic activity at the basal ganglia; the genetic factor and the non-genetic factors, for which environment more than genetic factors are involved. Pathologic examinations failed to reveal structural lesions, but PET studies showed metabolic hypofunction on the frontal, cingulate and possibly insular cortex, and on the inferior corpus striatum. The motor tics as well as the vocal tics can be simple or complex and are present in all patients. Other signs can be added to the previous tics: sensory tics, echophilia, coprophilia, obsessions, compulsions and impulsions. Diagnostic criteria of Gilles de la Tourette syndrome are based on: age of onset; presence of motor and vocal tics; voluntary suppression of the movements; variation in number, type, location and severity of tics; duration of more than one year. Haloperidol is the drug of choice for the treatment of Tourette's syndrome.