In Neurolinguistics, the use of diagnostic tests developed in other countries can create difficulties in the interpretation of results due to cultural, demographic and linguistic differences. In a country such as Brazil, with great social contrasts, schooling exerts a powerful influence on the abilities of normal individuals. The objective of the present study was to identify the influence of schooling on the performance of normal Brazilian individuals in the Boston Diagnostic Aphasia Examination (BDAE), in order to obtain reference values for the Brazilian population. We studied 107 normal subjects ranging in age from 15 to 84 years (mean ± SD = 47.2 ± 17.6 years), with educational level ranging from 1 to 24 years (9.9 ± 4.8 years). Subjects were compared for scores obtained in the 28 subtests of the BDAE after being divided into groups according to age (15 to 30, N = 24, 31 to 50, N = 33 and 51 years or more, N = 50) and education (1 to 4, N = 26, 5 to 8, N = 17 and 9 years or more, N = 61). Subjects with 4 years or less of education performed poorer in Word Discrimination, Visual Confrontation Naming, Reading of Sentences and Paragraphs, and Primer-Level Dictation (P < 0.05). When breakdown by schooling was 8 years or less, subjects performed poorer in all subtests (P < 0.05), except Responsive Naming, Word Recognition and Word-Picture Matching. The elderly performed poorer (P < 0.05) in Complex Ideational Material, Visual Confrontation Naming, Comprehension of Oral Spelling, Written Confrontation Naming, and Sentences to Dictation. We present the reference values for the cut-off scores according to educational level.

O vasoespasmo cerebral é responsável por aumento de morbidade e mortalidade em pacientes acometidos por hemorragia subaracnóide (HSA). Sua detecção de forma precoce e não-invasiva pelo método de Doppler transcraniano (DTC) pode contribuir para um melhor prognóstico destes doentes. OBJETIVO: Discutir o uso do método de DTC para diagnóstico do vasoespasmo cerebral secundário à HSA espontânea. MÉTODOS: Foram analisados os registros de velocidade de fluxo em artérias intracranianas por DTC em 31 pacientes com diagnóstico tomográfico de HSA, realizados seqüencialmente em conjunto com exame neurológico. Todos os pacientes (à exceção de um) eram portadores de aneurismas intracranianos. RESULTADOS: Vasoespasmo clínico foi constatado em 11 pacientes (36,6% do total), com correspondente vasoespasmo ultra-sonográfico presente em nove casos (82%). Em três casos (33,3%) as alterações de velocidade de fluxo precederam os sinais clínicos. Entre os 20 pacientes assintomáticos, 15 (75%) apresentaram medidas de velocidade normais, havendo evidência de vasoespasmo sonográfico sem repercussão clínica em cinco (25%). O índice de falso negativo na correlação entre exame clínico e DTC (sinais clínicos de vasoespasmo presentes, com velocidades de fluxo normais) foi de 18%. No entanto, houve correspondência entre os achados clínicos e ultra-sonográficos em 26 dos 31 pacientes (83,8%). Os valores de velocidades médias nas várias artérias e sua interpretação para cada subgrupo são discutidos com maiores detalhes. CONCLUSÃO: O método de DTC é um auxiliar valioso na detecção pré-sintomática do vasoespasmo secundário à HSA, permitindo uma intervenção terapêutica mais precoce. Os dados obtidos neste trabalho coadunam-se com os diversos estudos publicados na literatura.

Cerebral vasospasm accounts for an increased morbidity and mortality in patients with spontaneous subarachnoid hemorhage (SAH). Its early and non invasive detection by transcranial Doppler (TCD) may contribute to a better prognosis in such cases. PURPOSE: To discuss the use of transcranial Doppler (TCD) in the detection of cerebral vasospasm secondary cerebral to spontaneous subarachnoid hemorrhage (SAH). METHODS: We analyzed the flow velocities registered by TCD in intracranial arteries in 31 patients with SAH, performed sequentially, associated with neurological examinations. All patients (except one) had intracranial aneurysms. RESULTS: Clinical vasospasm was found in 11 patients (36,6% from the total), with correspondent sonographic vasospasm present in 9 cases (82%). In 3 cases (33.3%) the flow velocity alterations preceded the clinical symptoms. Between the 20 asymptomatic patients, 15 (75%) had normal flow velocity, with evidence of sonographic vasospasm without clinical repercussion in 5 (25%). The index of false negatives in the correlation between clinical examination and TCD (symptomatic vasospasm with normal flow velocity records) was 18%. However, there was correspondence between clinical and ultrasonographic findings in 26 of the 31 patients (83.8%). The average flow velocity values in the various arteries and its interpretation for each subgroup are discussed with further details. CONCLUSION: TCD is a valuable auxiliary method in the detection of cerebral vasospasm (even pre-symptomatic) secondary to SAH, allowing a more precocious therapeutic intervention. Our results are similar to those described in the literature.

A coreatetose paroxistica (CP) é entidade rara. Até mesmo profissionais que estudam desordens do movimento não costumam vê-la com freqüência. A ocorrência paroxística de movimentos distônicos, coréicos e atetósicos é a apresentação típica da síndrome. O início costuma ser abrupto e os ataques podem durar de alguns segundos até horas. Casos esporádicos e, mais frequentemente, casos familiares têm sido relatados. A abordagem terapêutica com anticonvulsivantes, como a carbamazepina, nem sempre tem sucesso. Com esta droga, porém, geralmente há boa resposta na variante cinesiogênica da CP. É relatado o caso de um paciente jovem do sexo masculino com essa variante da CP. O início da doença se deu na puberdade. O exame neurológico era normal entre os ataques. A investigação laboratorial, EEG, TC de crânio e RNM de segmento cefálico foram normais. Carbamazepina em doses baixas (100 mg/dia) foi eficaz no manejo dos ataques.

Paroxysmal choreoathetosis (PC) is a rare entity, and professionals who study movement disordes do not find it easily. Paroxysmal occurence of dystonic, choreic, athetosic movements is the typical feature of this syndrome. The onset is always subtle and the attacks can last from few seconds to hours. Sporadic cases and more common familial cases have been reported. The therapeutic approach with anticonvulsivant drugs like carbamazepine is not always successful. However, there is a good response to this drug in the kinesigenic form of PC. The case of a 21 years old male patient is reported here. The onset occurred during puberty, at 14 years old. Neurologic examination was normal between attacks. Subtle and brief choreic, athetosic and dystonic limb and legs movements precipitated by sudden quick and unexpected passive movements, as by startle, were observed during the attacks. There was no familial history. Routine laboratorial investigation, EEG, CT scan and MRI did not show significant changes. Carbamazepine in low dosages (100 mg/day) brought a complete control of the attacks.

Coréia pode ocorrer como manifestação neurológica do lupus eritematoso sistêmico, estando frequentemente associada à presença de anticorpos antifosfolípides. Evidência de coréia como sinal de atividade lúpica não foi descrita. Descrevemos o caso de uma paciente, com lupus eritematoso sistêmico e anticorpos antifosfolípides, que desenvolveu coréia, sendo esta manifestação considerada sinal de atividade dia doença.

Chorea may occur as a neurological manifestation of systemic lupus erythematosus and is often associated with detection of antiphospholipid antibodies. No evidence of chorea as a sign of lupus activity has been established. We describe a patient with systemic lupus erythematosus associated with antiphospholipid antibodies who developed chorea, which has been considered a sign of lupus activity.

Foram avaliados prospectivamente 118 pacientes (79 homens e 39 mulheres, com média de idade de 50,7 anos) submetidos a cirurgia cardiovascular com circulação extracorpórea. O objetivo foi identificar as complicações neurológicas mais freqüentes e relacioná-las a fatores de risco. As cirurgias tiveram a seguinte distribuição: 71 pacientes foram submetidos a revascularização do miocárdio, 18 a troca valvar. 6 a retroca valvar, 11 a comissurotomias. 5 a correção de aneurisma de aorta torácica e 7 a outros tipos. Apresentaram alterações neurológicas no primeiro período pós operatório 14 (11,9%) pacientes: delirium em 7 casos, acidente vascular cerebral isquêmico em 6 e crise epiléptica em 3. Os pacientes com hipertensão arterial sistêmica e os mais idosos tiveram risco maior de complicação neurológica, com significância estatística (p<0,05). Observou-se baixo índice de morbidade e mortalidade em relação aos dados de literatura

118 patients (79 mean and 39 women, mean age of 50.7 years) who underwent cardiovascular surgery and extracorporeal oxygenation were prospectively evaluated for neurologic complication and its correlation with risk factors. 71 were submitted to coronary artery graft by-pass (RM), 18 to valve replacement (TV), 6 to prosthetic valve replacement (RV), 11 to commissurotomy (Co), 5 to thoracic aortic aneurysm correction (An Ao T) and 7 to other surgeries (OT). All of them received extracorporeal oxygenation. No deaths were registered; 14 (11.9%) patients had neurologic abnormalities: delirium in 7 cases, ischemic stroke in 6, epileptic seizure in 3. Patients with systemic arterial hypertension and older patients exhibited a statistically significant (p<0.05) higher risk of complication. Compared to data of the lite, rature, we had a lower index of morbidity and mortality.

São descritos três casos d© degeneração hepatolenticular (DHL.) em que o efeito nefrotóxico da D-penicilina (DP) levou à substituição dessa droga por zinco, com resultados satisfatórios. O tempo médio de tratamento com zinco nos casos descritos foi de 2 anos e 7 meses. Com base nos dados de literatura e na experiência com esses pacientes, os autores consideram que os sais de zinco podem ser uma alternativa para o tratamento da DHL quando a DP determina o aparecimento de efeitos colaterais graves que levam à sua retirada definitiva.

Three patients with symptoms and signs of hepatolenticular degeneration (HLD) who developed serious renal side effects of D-penicillamine (DP) had their therapeutic schedule changed to zinc. Patient 1, a 55 year-old man had been well until 12 years old, when skeletal changes (osteomalacia) due to tubular renal disturbance began. His diagnosis of HLD had first been established at age of 32 when he presented with «wing-beat» tremor. He was then begun on DP and his neurological symptons resolved within one year of initiating therapy but skeletal abnormalities remained unchanged as a sequel. During the next 22 years the patient was continued on DP therapy but with poor compliance. Then the reapperance of his neurological manifestations occurred several times. By the age of 53, after one year without therapy, his neurological status has worse. DP was reinstituted but some weeks later his renal laboratorial parameters became serevely affected. DP was discontinued and zinc sulfate (220mg three times daily) was introduced. On this therapeutic regimen his renal laboratorial parameters returned to previous level after one month. Within one year on this therapeutic regimen neurological manifestations were resolved. After 31 months on zinc treatment he remains neurologically asymptomatic and his renal function is satisfactory. Patient 2, a 41 years old woman had been her diagnosis of HLD at age of 20, when following the diagnosis of the disease in her old brother, she was found to have the laboratorial features of HLD and bilateral Kayser-Fleischer rings. DP treatment was recommended at that time but she quitted the follow-up. When she. was 23, an esophageal variceal bleeding occurred. At the age of 30 she started DP treatment. During 9 years the patient, under regular use of the drug remained assymptomatic. At the age of 39 she developed acute renal failure due to penicillamine-induced immune complex nephropathy. DP was then stopped and prednisone introduced. After 4 months, as renal function returned to normal, prednisone was discontinued and zinc sulphate (220mg three times daily) introduced. She remained asymptomatic for 38 months. By the age of 41 a relapse of renal manifestations occurred, and she died months later. Patient 3, a 20 year-old man developed hemolytic anemia as the single manifestation of HLD at 16. DP was introduced with excellent results. Neverthless, 4 months later, with the appearance of severe renal side--effects, this drug was replaced by zinc sulphate (220mg three times daily). After 11 months under this therapeutic schedule he was hematologically asymptomatic but an epigastric distress appeared. Zinc sulphate was discontinued and zinc acetate (170mg three times daily) was introduced. Since that time, after 30 months of treatment with zinc, he remains asymptomatic and Kayser-Fleisher rings disappeared. A review of zinc salts therapy of HLD is presented and discussed.

Isotipos de imunoglobulinas (IgG e IgM) de proteína básica de mielina (PBM), cerebrosídeos (CER), gangliosídeos (GANG) e cardiolipina (CARD) foram investigados em amostras de liqüido cefalorraquidiano (LCR) de 33 pacientes com esclerose múltipla (EM), 18 com síndrome de Guillain-Barré (SGB), e 30 com lúpus eritematoso sistêmico (LES). Pacientes com EM revelaram concentrações positivas e significantes para IgG-PBM em 51,5% (p<0,05), IgM-PBM em 18,2%, IgG-CARD em 46,2%; CER e GANG foram detectados em cerca de 20% dos pacientes. A avaliação dos isotipos de imunoglobulinas no soro de pacientes com EM foi positiva em 20,6% para IgG-PBM e. 53,0% para IgM-PBM. No LCR dos pacientes com SGB a análise mostrou-se positiva em 56,3% para IgG-PBM (p<0,05), 53% para IgM-PBM, 38,5% para IgG-CER e 23% para IgM-CER, 50% para IgG-CARD e 31% para IgM-GANG. Os isotipos avaliados no soro de 14 pacientes com SGB foram positivos em 18,8% para IgG-PBM e 56,3% para IgM-PBM (p<0,05). No LCR, 50% dos pacientes com LES revelaram positividade para IgG-CARD e 241% para IgG-PBM. Os autores acreditam que a presença de anticorpos antifosfolipides seja conseqüente a epifenômeno imune, embora sua presença possa manter e perpetuar o evento imune intrínseco a essas doenças.

Immuneglobulins isotypes (IgG and IgM) for myelin basic protein (MBP), cerebrosides (CER), gangliosides (GANG) and cardiolipin (CARD) were detected in the cerebrospinal fluid (CSF) from 33 patients with multiple sclerosis (MS), 18 with Guillain-Barré syndrome (GBS) and 30 with systemic lupus erythematosus (SLE). In MS patients occurred positive and significant levels of IgG-MBP in 51,5% (p<0.05) and IgM-MBP in only 18.2%, IgG-CARD in 46.2%, as long as CER and GANG were detected in almost 20%. From serum samples of MS patients 20.6% presented IgG-MBP, while 53% showed positive levels foi IgM-MBP. The CSF analysis of patients with GBS showed that 56.3% revealed IgG-MBP (p<0.05), 53% for IgM-MBP. 3&.5% for IgG-CER and 23% for IgM-CER, while 50% of patients had IgG-CARD, as long -as 31% also had IgG-GANG. The serum evaluation from 14 patients showed that 18.8% had positive concentrations of IgG-MBP and 56.3% presented IgM-MBP (p<0.05) Except for 50% of patients with SLE who presented positive CSF levels of IgG-CARD. only 24.1% had positive levels of IgG-MBP. We believe that the presence of antiphosphohoid antibodies in CSF of the above mentioned diseases occurred as immune epiphenomena, but their appearance would permit the maintenance of and perpetuate the immune event.

Distúrbios do movimento, geralmente de tipo distónico, têm sido relatados em heterozigotos para doença de Wilson (DW). O presente relato assinala a presença em uma mesma família de heterozigoto para DW com quadro distónico e sua sobrinha com quadro clássico de DW. Discutem-se as peculiaridades dessa família, comparando-se aos dados da literatura, bem como os possíveis mecanismos etiopatrogênicos envolvidos.

Movement disorders, mostly dystonia, sometimes occur in heterozygotes for Wilson's disease (WD). A patient with metabolic abnormalities suggestive of heterozigose for WD and dystonia is reported. His niece showed the typical neurological and metabolic abnormalities of WD. This rare coincidence and the etiopathogenic mechanisms involved are discussed .

É relatado o caso de um paciente de 53 anos de idade com diagnóstico de síndrome de Shy-Drager. Testes laboratoriais e exame físico evidenciaram claramente o distúrbio do sistema nervoso autônomo. Foi também obtido traçado poligráfico do sono durante uma noite completa, que mostrou diminuição do sono tipo MOR e apnéias do tipo central. Os possíveis mecanismos destes achados polissonográficos são discutidos.

The authors report a case of Shy-Drager syndrome in a 53 year-old male patient. Autonomic failure was made evident by physical examiration as well as laboratory tests. A sleep recording showed decreased percentage of REM sleep and apneas of the central type. The possible mechanisms for this sleep disorder are discussed.

Neste relato preliminar os autores registram a constatação de alta prevalência de soropositividade para anticorpos dirigidos ao HTLV-I detectados pelo método de Western-blot (37,5%) em amostra de 16 pacientes brasileiros com mielopatias crônicas de causa não determinada.

In this preliminary report the authors present the finding of a high prevalence (37.5%) of seropositivity of antibodies to HTLV-I tested by Western blotting in a sample of 16 Brazilian patients with chronic myelopathies of undetermined origin.

É relatado o caso de um paciente de 11 anos de idade com oftalmoparesia extrínseca, ptose palpebral bilateral e tetraparesia desde os 7 anos de idade. A concentração de anticorpos contra receptor de acetilcolina por radioimunoensaio foi 0,6 nM/1; a pesquisa de anticorpos contra músculo estriado foi negativa. Exame eletromiográfico revelou decremento de 26,1%. Foi tratado com brometo de piridostigmina na dose de 60 mg/d. Submetido a biópsia de tecido muscular estriado (biceps braquial esquerdo) com avaliações por métodos histoquímicos e microscopia eletrônica, que revelaram: acúmulos de mitocôndrias na região subsarcolemal na coloração pela SDH; aumento da concentração de mitocôndrias e corpúsculos eletrodensos à microscopia eletrônica; esses achados são sugestivos de miopatia mitocondrial. Em conseqüência da interrupção das drogas anticolinesterásicas ocorreu piora das manifestações deficitárias, disfagia e dispnéia. Reintroduzidos os anticolinesterásicos, associados a imunossupressão com corticosteróides, houve melhora e retomada pelo paciente de suas atividades habituais. Destarte, é discutido o caráter inespecífico das alterações morfogenéticas e disfunções de mitocôndrias em outras patologias neuromusculares, incluindo a miastenia grave, como neste, caso em particular.

The case of an 11-year-old boy with external ophtalmoparesia, tetraparesia and bilateral eyelid ptosis is reported. He was 7-years-old when first symptoms appeared. Anticholinesterasic drugs were used. He was submitted to muscle biopsy. The results of histochemistry analysis showed storage of granulous material at the subsarcolemmal region of muscle fibers by SDH. Increase in the number of mitochondria with electron dense bodies was found at electron microscopy. Anticholinesterasic drugs administration was interrupted and consequently he got worse, and bouts of dyspnea occurred. Due to this worsening anticholinesterasic agents were reintroduced together with prednisone, and he improved. Due to clinical and histological expressions we think it is possible that morphological mitochondrial alterations may occur also in myasthenia gravis.

Registro de caso de paciente do sexo masculino com 40 anos de idade em que a tomografia computadorizada revelou a presença de processo expansivo no hemisfério cerebral direito. Os exames do líqüido cefalorraqueano e biópsia cerebral evidenciaram as características de neurossífilis. Após penicilinoterapia foi constatada atrofia focal no hemisfério cerebral direito. Este caso sugere que a forma de Lissauer da paralisia geral progressiva pode manifestar-se como processo expansivo cerebral.

A 40-year-old male patient with progressive dementia presented adversive seizures, and CT scan showed an enlarging focal mass lesion in the right cerebral hemisphere. Cerebrospinal fluid examination and brain biopsy confirmed the diagnosis of neurosyphilis. After a course of penicillin therapy there was disappearance of the cerebral mass lesion and the CT scan showed focal atrophy in the right cerebral hemisphere. This case suggests that Lissauer form of paretic neurosyphilis may present as a focal mass lesion.

São relatados dois casos de meningomielite brucelótica e um terceiro caso de hemorragia parenquimatosa e leptomeningite crônica com descrição anátomopatológica. Os autores destacam as formas clínicas e a importância das reações imunológicas no diagnóstico, assim como a necessidade do diagnóstico precose para o tratamento adequado.

Three cases of probable neurobrucellosis are reported. The diagnosis was made on the basis of imunological tests. Two patients with a clinical picture of meningomyelitis showed a definitive clinical improvement under tetraciclin and streptomòcin therapy. The imunological reactions found in the record case were even more positive in the spinal fluid than in the blood. In the case 3 with a clinical presentation of cerebral hemorrage the hitopathological studies demonstrated non specific chronic leptomeningitis and local hemorrages in the caudate nucleus bilaterally. The diagnose and treatment of neurobrucellosis are discussed, stressing the importance of an early therapy.

Foi realizado estudo citogenético e dermatoglífico em 10 pacientes com distrofia miotônica. Não foram encontradas anomalias nestes pacientes, exceto por uma ocasional translocação cromossômica.

Cytogenetic and dermatoglyphic studies were performed in 10 patients affected by Steinert's myotonic dystrophy. No anomalies were found in karyotype and dermatoglyphs in these patients, except for an occasional chromosomal translocation.

Registro de caso meningo-encefalo-mielite determinada por ameba de vida livre. Esta foi isolada a partir do LCR do paciente e identificada como Naegleria sp. A mesma ameba foi isolada em amostra de água da lagoa na qual o paciente costumava nadar. A terapêutica por Anfotericina-B foi satisfatória, tendo havido remissão do processo infeccioso. Como seqüela restaram alterações sensitivo-motoras decorrentes do processo mielítico.

A case of primary amebic meningoencephalomyelitis due to Naegleria sp observed in a 14 years old boy is reported. Symptoms due to myelitis at the dorsal level assumed ascending character during the first days of disease. Manifestations due to encephalic involvemente were discrete. Cerebrospinal fluid changes were marked by pleocytosis, and the eosinophil cells participation in the cytomorptiological profile was persistently high. Amphotericin-B was used intravenously (25 mgm/day) until 1,500 mgm of total dosis. Remission of encephalitic manifestations was prompt, as well as of meningeal signs. Cerebrospinal fluid changes disappeared progressively. Sensitive-motor changes due to spinal cord involvement persisted as permanent sequelae. Naegleria sp., was isolated from the cerebrospinal fluid in the acute stage of the disease, as well as, from the water of a lagoon where the patient used to swimm.