No ano de 2002, após analisarmos 28 pacientes HIV-positivos que apresentavam distúrbios do movimento, enfatizamos o declínio, não só do parkinsonismo, como também de outros movimentos involuntários em pacientes infectados pelo HIV nos últimos anos. O objetivo deste estudo é comparar os resultados clínicos entre pacientes HIV-positivos com parkinsonismo antes e depois da introdução do esquema HAART. Em 14 anos (1986-1999), 2.460 pacientes HIV-positivos foram avaliados em nosso Hospital dos quais 14 (0,6%) apresentaram parkinsonismo. Nos oito anos seguintes (2000-2007), 970 pacientes HIV-positivos foram avaliados e somente dois (0,2%) tinham parkinsonismo. Concluímos que após a introdução do esquema HAART houve evidente declínio do parkinsonismo secundário à AIDS.

In 2002, after analyzing 28 HIV-positive patients with movement disorders we emphasized the decreasing not only of Parkinsonism but also of other involuntary movements in HIV patients in the last few years. The objective of this study is to compare the clinical results between HIV-positive patients with Parkinsonism before and after HAART. In 14 years (1986-1999) 2,460 HIV-positive patients were seen in our Hospital 14 (0.6%) of which presented with Parkinsonism. Eight years after (2000-2007) 970 HIV positive patients were seen and only two (0.2%) had Parkinsonism. We conclude that after the introduction of HAART there was an evident decrease in AIDS-related Parkinsonism.

Estudamos as características clínico-evolutivas de pacientes com esclerose múltipla (EM) acompanhados no HUCFF-UFRJ desde 1978. Foram usados critérios de Poser et al. e MC Donald et al. para o diagnóstico de EM. De 188, 122 foram incluídos. Oitenta e cinco eram mulheres. A média de idade de início foi 32,2 anos (6,0-61,0±10,3), predominando caucasianos (n=82/67%). A forma recorrente-remitente (EMRR) foi mais freqüente (n=106/86,8%). Formas mono-sintomáticas no primeiro surto foram significativamente mais freqüentes em caucasianos do que em afro-brasileiros (p<0,05). Dezessete pacientes apresentavam a forma benigna (13,9%) e 43 a grave (35,2%). A forma benigna foi associada com a EMRR (p=0,01). A taxa de letalidade 2,12% (4 óbitos). Nossos resultados são semelhantes aos de outras séries brasileiras no que se refere ao sexo e idade, e falta de correlação entre EDSS e número de surtos; confirmamos gradiente sul-sudeste de distribuição afro-descendente, associação significativa entre primeiro surto mono-sintomático e caucasianos e menor freqüência de formas benignas.

We studied the clinical and evolution characteristics of multiple sclerosis (MS) patients followed since the onset of HUCFF/UFRJ in 1978. The diagnosis of MS was based on Poser's et al. and MC Donald's et al. criteria. From 188 patients, 122 were included. Eighty-five were females. The mean age onset was 32.2 years-old (range 6.0 to 61.0±10.3), mainly Caucasians (82/67%). The relapsing-remitting course (MSRR) was more frequent (106/86.8%). Monosymptomatic onset was significantly more frequent in Caucasians than in Afro-Brazilians (p<0.05). Seventeen patients had benign form of MS and these patients presented association with MSRR when compared with severe form (p=0.01). The mortality rate was 2.12% (4 patients died). This study was similar to other Brazilian series with regard to sex and age, and lack of correlation between EDSS and number of relapses; it confirmed south-southeast African-descendants gradient distribution and association between first mono-symptomatic relapses and Caucasian; we found lower frequency of benign forms.

A doença de Creutzfeldt-Jakob (DCJ) é a encefalopatia espongiforme subaguda transmissível mais frequente nos seres humanos. Aproximadamente 85% dos casos pertencem à forma esporádica da doença. Os outros 15% consistem na forma genética e iatrogênica. Relatamos o caso de uma paciente com a forma esporádica da doença de Creutzfeldt-Jakob, com comprometimento medular e apresentação clínica caracterizada por síndrome demencial e cerebelar, miofasciculação com arreflexia difusa e crises convulsivas do tipo tônico-clônico generalizada. É rara a associação das duas últimas manifestações clínicas. O caso foi considerado como provável DCJ até confirmação por autópsia e imunohistoquímica. Concluímos que se deve sempre pensar na DCJ em pacientes que apresentam demência rapidamente progressiva e, na ausência de sinais piramidais ou extrapiramidais, pensar em acometimento periférico e/ou medular.

Creutzfeldt-Jakob disease (CJD) is the most common subacute transmissible spongiform encephalopathy. Approximately 85% of the cases are sporadic. The remaining 15% consist of genetic and iatrogenic forms. We report a sporadic form of CJD with spinal cord involvement and a clinical manifestation characterized by dementia and cerebellar syndrome, myofasciculation with absent reflexes and seizures. The two last manifestations are rare. The clinical hypothesis was probable CJD which was confirmed with autopsy and immunohistochemistry. We conclude that CJD should always be suspected when rapidly progressive dementia occurs and the absence of pyramidal or extrapyramidal signs suggest a spinal cord and/or peripheral nerve involvement.

Tivemos como objetivo investigar por eletroneuromiografia (ENMG) e potenciais evocados somatossensitivos a possibilidade de acometimento do nervo periférico na mielopatia pelo HTLV-I (HAM), correlacionando os achados com os parâmetos clínicos e com a síntese intratecal de anticorpos anti HTLV-I. Os pacientes tinham sorologia negativa para HIV e apresentaram VDRL negativo. Outras causas para mielopatia ou neuropatia periférica foram excluídas. De 32 pacientes que realizaram ENMG, em 34,3% ela foi considerada sugestiva de neuropatia periférica. Esta foi sobretudo assimétrica (82%), sensitivo-motora (90%),com padrão axonal (54,5%) ou misto (45,4%). Em 63,6% dos casos de neuropatia periférica, havia sintomas correlatos. O potencial evocado auditivo foi anormal em apenas um caso. O potencial evocado visual foi anormal em 28,5% dos casos. Não havia sinais ou sintomas auditivos ou visuais. Em 85,5% dos casos obteve-se potencial evocado sensitivo alterado. Desses, 50% tinham manifestação clínica compatível. Em 28% dos pacientes com potencial evocado sensitivo anormal houve concomitância de ENMG sugestiva de neuropatia periférica.

The objective of the present study was to correlate electroneuromyography (ENMG) and evoked potentials findings with clinical aspects and intrathecal synthesis of HTLV-I antibodies production on HTLV-I myelopathy (HAM). Patients were seropositive for HTLV-I by different assays and seronegative for HIV and VDRL. They had no other causes of myelopathy and peripheral neuropathy. Peripheral neuropathy was stablished in 34.3% of the cases by ENMG. Peripheral neuropathy was mostly asymmetric (82%), sensory motor (90%), axonal (54,5%) or of a mixed type (45,4%). In 63.6% of these cases related symptoms were observed. ABR was abnormal in one patient and the PRVEP in 28.5% ,who were symptom-free. The SEP was abnormal in 85.7% of the cases, half of them presenting clinical complaints. In only 14% of the individuals with clinical manifestations, SEP was normal. In 28% of patients with abnormal SEP the ENMG disclosed a peripheral neuropathy.

Estudamos a influência de dados demográficos, fatores predisponentes, aspectos clínicos, variáveis operatórias sobre a evolução em 39 pacientes operados por endocardite infecciosa (EI). Utilizamos os testes t de Student, X²ou exato de Fisher em análises univariadas, regressão logística para determinação de fatores adversos independentes. O impacto do número destes fatores sobre a evolução foi estudado pelo teste exato de Fisher. Valvas mais afetadas: aórtica (20) e mitral (16); germes mais comuns: Staphylococcus aureus (12) e Streptococcus sp (10). Cirurgia de emergência e a presença de coma seis horas após a operação elevaram a letalidade (p=0,001 e p=0,0015), bem como infecção pelo S.aureus (p=0,023) e presença de complicações neurológicas (p=0,097). A concomitância de dois ou três destes fatores elevou particularmente a letalidade (>76,9%). Pacientes com EI devem receber cuidadosa avaliação quanto a indicação cirúrgica nas fases iniciais da doença, já que a concomitância de variáveis adversas e cirurgias em caráter de emergência elevam fortemente a letalidade por EI.

The factors leading to high postoperative mortality in active infectious endocarditis (IE) are poorly defined. We studied patients operated at an University Hospital between March 1978 and April 1992. We hipothesized that the summation of potential adverse factors would strongly increase mortality after surgery. We studied 39 patients (28 men), age range 13-70 years (mean±SD =32±16) operated during active IE (time from onset 52+48 days). Predisposing factor: rheumatic valvar disease in 14 cases, intravenous drug use in 5. Affected valves: aortic in 14, mitral in 10, tricuspid in 8, multiple structures in 7. In most cases, S aureus (12) or Streptoccocus sp (10) was isolated in blood cultures. Surgery was indicated in most patients because of heart failure (30), multiple embolic complications (17) or treatment failure (14). The possible adverse influence of specific demographic characteristics, clinical features and surgical variables was assessed by the Student t test or the %² test. Also, multple regression analysis was performed in order to identify independent adverse factors for increased mortality. Positive correlations were further investigated with the X² test to assess whether an increasing number of adverse factors could identify a special subset of patients with markedly elevated death risk. Fourteen patients (36%) died after surgery. Emergency surgery (p = 0.001), the presence of coma 6 hours after surgery (p = 0.0015) and S. aureus infection (p = 0.023) were all associated with increased mortality. The presence of neurological complications was correlated with a high mortality (54% vs. 27%). However this increase was of dubious statistical significance (p = 0.097). Multiple regression analysis confirmed S. aureus and emergency surgery as independent adverse factors for increased mortality. When put together, an increasing number of adverse factors was highly predictive of a fatal outcome, even after exclusion of that evaluated after surgery (level of consciousness). Patients with two or three adverse factors had a very high mortality rate (>76.9%). Mortality following surgery for active IE is increased in patients operated on an emergency basis especially if the infection is caused by S. aureus. The presence of neurological complications may also be associated with worse outcome. Early consideration of surgery should reduce the high mortality in patients with active IE.

A gênese da hiperglicemia surgida na fase aguda do AVE em pacientes não diabéticos correlaciona-se basicamente ao grau de injúria celular e, portanto, à gravidade da reação fisiológica de «stress». Experimentalmente, o efeito deletério da hiperglicemia após a lesão isquêmica parece depender, em primeiro lugar, do acúmulo intracelular de ácido lático, gerado pela via glicolítica anaeróbica. Este acúmulo anormal de ácido lático, e consequente acidose tissular, parece ocorrer especialmente na fase inicial de reperfusão, ou na presença de fluxo sanguíneo residual - isquemia parcial. Fatores adicionais invocados para explicar o agravamento do insulto isquêmico pela hiperglicemia são a redução do fluxo sanguíneo cerebral e, talvez, a produção de um estado hiperosmolar. Em pacientes diabéticos alterações hemorreológicas e microcirculatórias específicas, bem como maior susceptibilidade às complicações infecciosas, vêm somar-se, para tornar também comum uma evolução clínica desfavorável.

In non-diabetic patients, the appearance of hyperglycemia in the acute phase of stroke is related to the extension of cellular injury, and hence to the physiologic stress response. In animal models of ischemic insult, the deleterious effects of hyperglycemia depend heavily on the production of lactic acid «via» activation of the glycolitic anaerobic pathway. The abnormal production of lactic acid and consequent tissular acidosis appear mainly in the early post-reperfusion period, or in states of marked but partial reduction of blood flow. A direct reduction of cerebral blood flow and, perhaps, the production of a hyperosmolar state may contribute to worsening of the ischemic injury. In diabetic patients, previous hemoreologic and microcirculatory changes, and a greater susceptibility to infections may additionally reduce the chances of complete recovery after stroke.

Em uma série de 109 casos de infarto cerebral comprovado por tomografia computadorizada de crânio, diabetes mellitus esteve presente em 15,6%. Em 86 casos avaliados precocemente foi detectada hiper glicemia em 67,4%, e em 100% de 14 pacientes diabéticos. Houve aumento (não significativo) nas taxas de letalidade hospitalar precoce - até o 30º dia de internação - de pacientes diabéticos; suas chances de obter alta precocemente, porém, pareceram reduzidas. A evolução de pacientes com hiperglicemia também pareceu pior. Estes pacientes exibiram redução significativa das chances de obter alta hospitalar precoce. O risco de vida pareceu máximo em diabéticos hiperglicêmicos (42,9%). As aparentes diferenças de letalidade e necessidade de internação prolongada entre pacientes não diabéticos, normo ou hiperglicêmicos, não alcançaram significação estatística. Os achados do presente estudo parecem indicar uma somação de efeitos deletérios pela hiperglicemia e pelo diabetes no curso do infarto cerebral. Cabe agora a avaliação crítica da sugestão de que a normalização precoce de hiperglicemia poderia levar a redução do grau de lesão cerebral após AVE isquêmico.

We acessed the potential impact of diabetes mellitus and hyperglycemia on early prognosis in a study of 100 cases of CT proved brain infarction. Main end-points of this study were 30-days case-fatality rates and the probability of early discharge or prolonged hospitalization. Proportions of patients exhibiting diverse evolutions were compared by Fisher's test and, when the number of observations permitted, by chi-square test. Diabetes mellitus was diagnosed by history in 15.6% (17 patients). In 86 cases analysed within 72 hours of admission, hyperglycemia (glucose level higher than 110 mg/dL.) was detected in 67.4% (including all 14 diabetic patients evaluated). A nonsignificant increase in case fatality rates was detected in diabetic patients (35.3% vs 21.7%; p=0.18), but these patients probably were discharged early less frequently (23.5% vs 51.1%; p==0.06). Compared to the normoglycemic patients, hyperglycemic patients also fared worse, when considering altogether the early discharge and case fatality rates and the need for prolonged hospitalization (p=0.06). They also exhibited a nonsignificant increase in early case fatality rate (31% vs 15.4%; p=0.21) and a markedly reduced chance of obtaining early hospital discharge (37.9% vs 65.4%; p=0.03). Case fatality rates were maximal in diabetic hyperglycemic patients (42.9%) and seemed intermediate in non-diabetic hyperglycemic patients (27.3%). Considering the three end-points evaluated, the apparent differences between non-diabetic hyper and normoglycemic patients did not reach statistical significance (p=0.19). These results suggest that both diabetes mellitus and hyperglycemia «per se» have deleterious effects on the clinical evolution of patients with cerebral infarction. It is now time to consider a prospective study on the potential benefits of early correction of hyperglycemia in these patients.