[Creutzfeldt-Jakob disease: case report with spinal cord involvement]. / Doença de Creutzfeldt-Jakob: a propósito de um caso com comprometimento 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.

[Volumetric determination of cerebral infarction in the acute phase using skull computed tomography without contrast: comparative study of 3 methods]. / Determinação volumétrica do infarto cerebral na fase aguda usando tomografia computadorizada de crânio sem contraste: estudo comparando três métodos.

The determination of lesion volumes in acute cerebral Infarction (CI) has important prognostic and therapeutic implications. Three different methods -computerised technique, linear planimetry and the A.B.C/2 formula - were compared in 27 patients using non-contrast computerised tomography (CT) made at hospital admission (first 48 hours following symptom onset). Two independent observers performed the measurements. The Intra-class correlation coefficients (ICCs) were determined. The three methods exhibited high interrelation. There was a close correlation between the linear planimetry as well as the ABC/2 formula and the computerised method with ICCs of 0.94-0.95 and 0.835-0.90 respectively. Linear planimetry and the ABC/2 formula were also highly correlated, with ICCs between 0.97 and 0.99. In conclusion, the computerised method is probably the most accurate. Linear planimetry and the ABC/2 formula are also reliable, however. The A.B.C/2 formula should be applied routinely to CI volume determination based on its low cost and fast calculation.

[Multiple sclerosis, spinal cord ependymoma and intracranial meningioma: case report]. / Esclerose múltipla, ependimoma medular e meningioma intracraniano: relato de caso.

We report the association a multiple sclerosis (MS), spinal cord tumour and intracranial tumor in a 63 years-old female patient with a 10 years history of relapsing/remitting MS. Symptoms usually remitted in response to costicosteroid therapy. In 1997 the patient presented with paraparesis and paresis of right arm which did not respond to corticotherapy. A spinal RMI revealed in the cervical spinal an intra spinal cord tumour, further diagnosed as ependymoma, and a parietal region meningioma. We call attention to this rare association of central nervous system tumour and MS, enphasizing the need for investigation of new and uncommon symptoms during the evolution of MS.

[Intracranial tuberculomas developing during treatment of pulmonary tuberculosis: case report]. / Múltiplos tuberculomas intracerebrais na vigência de terapia específica para tuberculose pulmonar: a propósito de um caso.

The classical presentation of neurotuberculosis is meningitis. Intracranial tuberculomas are a rare manifestation of neurotuberculosis and are due to hematogenous dissemination of distant focuses of Mycobacterium tuberculosis infection. Around 1% of patients with central nervous system tuberculosis develop intracranial tuberculomas some weeks or months after the beginning of the specific treatment with tuberculostatic chemotherapy. The involution of the lesions is slow and does not mean drug resistance nor lack of adequate treatment. We describe the case, diagnosed and treated at the 25th Infirmary of Santa Casa da Misericórdia do Rio de Janeiro, of an immunocompetent male patient who developed meningitis and multiple intracranial tuberculomas during the specific treatment of miliary tuberculosis. The literature is revised and the diagnosis, treatment and possible immunological mechanisms are discussed.

An inverse correlation of HTLV-I viral load in CSF and intrathecal synthesis of HTLV-I antibodies in TSP/HAM.

The authors measured human T-cell lymphotrophic virus type I (HTLV-I) proviral load and intrathecal synthesis of antibodies to HTLV-I in CSF of 13 Brazilian patients with tropical spastic paraparesis/ HTLV-I-associated myelopathy (HAM). The authors also measured HTLV-I proviral load in peripheral blood mononuclear cells of five of these patients and found that it was 10- to 100-fold higher than that in CSF cells. The combination of HTLV-I proviral load and intrathecal synthesis of antibodies to HTLV-I appears to be a useful marker of disease progression. Patients with high viral load and no intrathecal synthesis of antibodies to HTLV-I had more rapidly progressing, serious clinical disease.

[Parinaud syndrome and Collier signal. Initial neurological manifestation in an HIV-1 positive patient]. / Síndrome de Parinaud e sinal de Collier. Manifestação neurológica inicial em paciente HIV-1 positivo.

We describe the third case in the literature of Parinaud's syndrome as the sole manifestation of brainstem toxoplasmosis, in a positive HIV-1 patient and review the literature concerning the possible etiologies of Parinaud's syndrome, enforcing that it is a rare manifestation of central nervous system toxoplasmosis. We refer to its occurrence without simultaneous obstructive hydrocephalus and increased intracranial pressure, pointing out the Collier signal which is not very well known, but has great semiotic value once it locates the lesion in the brainstem.

Emergency physician's diagnosis of stroke subtype. An accuracy study.

OBJECTIVE: To evaluate the accuracy of clinical unstructured and structured diagnosis of acute stroke subtypes--cerebral haemorrhage (CH), cerebral infarction (CI), subarachnoid haemorrhage (SAH). METHODS: Sixty consecutive patients with acute stroke admitted to the Emergency Ward of a Brazilian University Hospital were examined by emergency physicians and computerised tomography (CT). We also compared it (physician's unstructured diagnosis) to two published clinical scoring systems (structured diagnosis--Guy's Hospital and Siriraj Hospital) applied to three other populations--regarding the operational characteristics of the tests. RESULTS: In our personal data, among 9 variables that could discriminate CH and CI, three have statistically significant difference (p < 0.05): headache (p = 0.0002) and vomiting (p = 0.02) occurred more frequently in CH patients, but previous stroke in those with CI (p = 0.04). Unstructured diagnosis proved valid for SAH, with a +LHR = 39.7; and to a smaller degree for CI (-LHR = 0.1). However, it exhibited low sensitivity for the diagnosis of CH. Structured tests (Guy's Hospital and Siriraj Hospital) also failed to confidently diagnose stroke subtypes, especially CH. CONCLUSIONS: Both clinical diagnosis (made by emergency physicians) and the available diagnostic tests fail to confidently discriminate CH and CI.

Greater occipital nerve blockade in cervicogenic headache.

Cervicocogenic headache (CeH) is a relatively common disorder. Although on ideal treatment is available so far, blockades in different structures and nerves may be temporarily effective. We studied the effects of 1-2 mL 0.5% bupivacaine injection at the ipsilateral greater occipital nerve (GON) in 41 CeH patients. The pain is significantly reduced both immediately and as long as 7 days after the blockade. The improvement is less marked during the first two days, a phenomenon we called "tilde pattern". GON blockades may reduce the pool of exaggerated sensory input and antagonize a putative "wind-up-like effect" which may explain the headache improvement.

[Electroneuromyography and somatosensory evoked potentials in HTLV-I associated myelopathy]. / Eletroneuromiografia e potenciais evocados somatossensitivos na mielopatia pelo HTLV-I.

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 established 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.

Experience with t-PA treatment in a large South American city.

BACKGROUND AND PURPOSE: Early thrombolysis with intravenous recombinant tissue plasminogen activator (t-PA) reduces disability following ischemic stroke. However, only a small fraction of the potential candidates receive therapy. We studied the proportion of eligible patients who did not receive t-PA and the reasons therapy was withheld from patients admitted to a Brazilian university hospital. METHODS: From July 1996 to June 1997, each suspected case of hyperacute ischemic stroke led to the immediate evaluation by a research physician. Patients first seen in primary care centers near the research hospital were to be rapidly transferred. Reasons for patient exclusion were detailed. RESULTS: Fifty-six patients were evaluated; only 5 received t-PA therapy. The main reason for not receiving t-PA (34 cases) was late hospital arrival. This was caused by lack of recognition of the early stroke signs (n=13); delayed personal or family response (n=7); wrong medical orientation (n=4); and late patient transportation (n=10). A computed tomographic (CT) scand could not be done fast enough to allow therapy in 8 patients. Nine of 14 patients in whom neurological evaluation and CT scan were done within the 3-hour period were also excluded. The main reason was refusal of informed consent (n=5). Only a few patients were excluded because of medical contraindications (n=3) or the presence of early mass effect (n=1). CONCLUSIONS: The main factor limiting access of patients with ischemic stroke to t-PA therapy in Rio de Janeiro is a low public awareness of early stroke symptoms and of the need for urgent hospital evaluation. Public education, an efficient transport system for suspected stroke patients, and optimization of the use of available CT scanners could have a significant impact on the number of treated patients.

[Postoperative mortality in infective endocarditis: determinant factors]. / Fatores determinantes da letalidade após cirurgia para endocardite infecciosa.

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 hypothesized 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 Streptococcus 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 chi 2 test. Also, multiple regression analysis was performed in order to identify independent adverse factors for increased mortality. Positive correlations were further investigated with the chi 2 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.

[Knowledge and practices regarding stroke at a university hospital: Part 1. Education of the nursing staff: priorities for the treatment of cerebral infarction]. / Práticas e nível de conhecimento sobre doença cerebrovascular em um hospital universitário. Parte 1. Educação do corpo de enfermagem: prioridade para o tratamento do infarto cerebral.

BACKGROUND AND PURPOSE: This study was undertaken to evaluate professionals working at a university hospital as to their knowledge and attitudes towards stroke. METHODS: Individuals working in the hospital were divided in two groups, health care workers (HCW) and non-health care workers (NHCW), and further subdivided according to level of schooling, resulting in seven strata. A closed questionnaire addressing epidemiology, risk factors, pathophysiology, typical symptoms, treatment, clinical course and personal attitudes towards smoking and blood pressure control, was applied to a random sample of each stratum (total n = 309). Kruskal-Wallis test for multiple comparisons of non-parametric data was used. RESULTS: Significant differences between the seven groups were found. Knowledge was strongly associated with being a HCW and with level of formal education (p < 0.001), even after excluding physicians from the analysis (p < 0.001). In NHCW groups, knowledge was not associated with level of education (p = 0.421). In these groups, personal fear of suffering a stroke was the only variable predictive of knowledge. Smoking and poor monitoring of blood pressure levels were also more common in strata with the lowest levels of education and among NHCW. CONCLUSION: Poor knowledge and wrong attitudes towards stroke are frequent among individuals working in a Brazilian university hospital. Although these results are not necessarily applicable to the general population, they will certainly be useful for the development of educational programs on stroke.

[Knowledge and practices regarding stroke at a university hospital: Part 2. Medical education in the new era of treatment of cerebral infarction]. / Práticas e nível de conhecimento sobre doença cerebrovascular em um hospital universitário. Parte 2. Educação médica na nova era de tratamento do infarto cerebral.

BACKGROUND AND PURPOSE: This study was undertaken to evaluate professionals working at a University Hospital as to their knowledge and attitudes towards stroke. METHODS: Individuals working in the hospital were divided in two groups, Health care workers (HCW) and non-Health care workers (NHCW), and further subdivided according to level of schooling, resulting in seven strata. A closed questionnaire addressing epidemiology, risk factors, pathophysiology, typical symptoms, treatment, clinical course and personal attitudes towards smoking and blood pressure control, was applied to a random sample of each stratum (total n = 309). The physicians group included 48 individuals. Kruskal-Wallis test for multiple comparisons of non-parametric data was used. Special attention was given to the wrong answers. RESULTS: Physicians correctly answered 92.6% of the questions. Their performance was superior to that of all other groups in all subgroups of questions. However, a large number of errors was found in questions addressing mortality and hospital mortality following stroke and the intimate relation between coronary and cerebral atherosclerosis. Treatment options in cerebral infarction are also poorly recognised. CONCLUSIONS: Although physicians general knowledge about stroke is good, they frequently do not perceive it as a critical disease requiring urgent hospital evaluation and care. The importance of a thorough cardiac evaluation following stroke and of the intimate relation between cardiac and cerebral ischemic disease is also unclear to this group. More hours of stroke teaching and practical training in stroke could possibly fill these gaps.

Sumatriptan blocks spreading depression in isolated chick retina.

Spreading depression is a neurohumoral phenomenon that has been related to the pathophysiology of migraine. The recently introduced 5HT1D agonist anti-migraine compound sumatriptan blocks neurogenic extravasation and induces cerebral vasoconstriction, but the actual mechanism of action against migraine remains obscure. Retinal spreading depression (RSD) velocity has been measured in isolated chick retinas in the presence of 0.05-2.00 mM sumatriptan. This drug reversibly blocks RSD in a concentration-dependent manner. Since the preparation is blood-vessel free, this effect must be related to the nervous tissue.

[Neuropathogenesis of HIV infection]. / A neuropatogenia do vírus da imunodeficiência humana.

The spreading of human immunodeficiency virus (HIV) infection and its increasing scientific knowledge keep the medical staff involved with these patients in permanent need of updating themselves. The different neurologic manifestations caused by HIV are related to a variety of pathogenic mechanisms, as follows: immunodeficiency, autoimmunity, direct effects of the virus on the nervous system, and toxic and metabolic effects. The opportunistic infections are caused by the immunodeficiency due to the action of the virus on CD4+ T cells and on cells of the monocytic-macrophage lineage. Demyelinating polyradiculoneuropathy and polymyositis-like syndromes are related to autoimmune mechanisms involving, probably, the non-specific stimulation of T cells by viral proteins. The primary action of the virus on the nervous system brings out aseptic meningitis, cognitive dysfunction, dementia, vacuolar myelopathy and sensory polyneuropathy probably through liberation of neurotoxic products by the infected macrophages. Antiretroviral drugs and others used to treat patients with AIDS may also have neurotoxic effects. The better understanding of the neuropathogenesis of HIV infection will permit the use of new, and more specific, therapeutical options in the future as well as a more precocious control of its neurologic complications.

Electroneuromyography and neuromuscular findings in 16 primary hypothyroidism patients.

There has been few reports on electroneuromyography (ENMG) changes in hypothyroidism. The objectives of the present study were to investigate the frequency of ENMG abnormalities in hypothyroidism and correlate them with neurological signs and symptoms and muscle enzyme levels; and to compare latency, amplitude and nerve conduction velocity from selected nerves with controls. Sixteen patients suffering from primary hypothyroidism were submitted to ENMG before treatment. ENMG abnormalities were found in 87.5% of the patients; 46.6% had myopathy and 43.7% had carpal tunnel syndrome. There was no case of polyneuropathy. A clear-cut clinical, laboratorial and ENMG correlation was observed in patients with myopathy and carpal tunnel syndrome. The patients showed a significant tendency of nerve conduction slowness as compared with controls. The findings are in accordance with the well-known nerve and muscle damage in hypothyroidism.

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

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 beneficial 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 beneficial 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.(ABSTRACT TRUNCATED AT 250 WORDS)

Clinical factors adversely affecting early outcome after brain infarction.

PURPOSE AND METHODS: One-hundred-and-nine consecutive patients admitted during the acute phase of a CT-confirmed brain infarction (BI) were studied. Putative adverse influence of demographic and stroke risk factors, previous medical history, clinical presentation, initial and follow-up neurological examination, initial general evaluation, laboratory findings, chest X-ray and electrocardiographic findings, treatment, and topography and etiology of the ischemic insult was analysed. The end-point for assessment was early death (within 30 days). Statistical analysis was performed with univariate analysis and multiple regression. RESULTS: The main adverse factors related to an increased death risk during the first 30 days were, in decreasing order of importance: coma 48-72 hours after admission; stroke occurring in already hospitalized patients; Babinski sign on admission; minor degrees of impairment of consciousness 48-72 hours after admission; stroke related to large artery atherothrombosis and to embolism; a history of early impairment of consciousness; cardiac failure on admission. In 53 lucid patients on admission, only a history of congestive heart failure (CHF) was associated with a reduced survival rate. In 56 patients with impaired consciousness, the presence of a Babinski sign increased death risk, but the main factor predicting a high case-fatality rate was the persistence of consciousness disturbances after 48-72 hours. CONCLUSIONS: The presence of impairment of consciousness, especially coma, 2-3 days after disease onset, and a history of CHF greatly increase the early case fatality rate in patients with acute BI presenting with or without consciousness disturbances at admission, respectively. The use of a prognostic algorythm considering these few variables seems to predict the approximate 30-day fatality rates.