[Prophylaxis of migraine: open study with venlafaxine in 42 patients]. / Profilaxia da migrânea. Estudo aberto com a venlafaxina em 42 pacientes.

The present open study intended to show the prophylactic value of venlafaxine (serotonin and noradrenalin reuptake inhibitor) in 42 patients with migraine with or without aura during four months, from May to September 1997. The average age was 32.5 years and average crisis was 3.8 per month. Consistent side-effects included loss of weight, nausea and vomit. The efficacy of drug occurred in 37 patients (88.07%) with doses of 18.75 to 37.5 mg/day. We conclude that venlafaxine is an effective drug in prophylaxis of migraine patients.

Machado-Joseph disease of Azorean ancestry in Brazil: the Catarina kindred. Neurological, neuroimaging, psychiatric and neuropsychological findings in the largest known family, the "Catarina" kindred.

At the moment 9 seemingly independent families with the clinical diagnosis of MJD are known in Brazil. The largest family tree of Azorean ancestry contains 622 individuals in 9 generations. 236 were examined, 39 found to be affected by two examiners. Phenotypes I, II and III were expressed by 12, 23 and 4 patients with age of onset by phenotypes being 10-48, 14-54 and 30-55 respectively. Although clinically more severe, juvenile onset type I disease did not show as severe a ponto-mesencephalic atrophy on MRI as the father with type II disease of similar symptomatic duration. None of the 8 patients examined with MRI showed olivary atrophy or pallidal abnormalities. 12 affected and 23 at risk were evaluated with neuropsychological tests. Attention was normal in both groups. Verbal memory scores were below normal in the affected and there was greater decay with time than in the risk group. Both scored below normal in identifying silluettes and constructional praxis. Visual memory scores were well below normal for both, with many rotations but no omissions or confabulations. A peculiar pattern of multiplying internal details called "the fly-eye effect" was observed in 6 affected and 8 at risk. Defective color distinction when multiple colors presented close to each other, in face of proper naming of individual colors ("color simulatanagnosia"), was looked for in 29 people. 4/10 affected and 4/19 at risk showed this phenomenon. Cognitive dysfunctions in this MJD family are prominent in the sphere of vision. Whether they constitute an early manifestation in those at risk and thus serve as a clinical identifier of the illness is yet to be established. Depression was looked for in the history of the family with DSM III-R criteria and an attempt at quantification with the Montgomery-Asberg Rating Scale. There was no significant quantitative difference between affected and at risk. Once undeniably symptomatic however, the patients had no, or less depression than themselves before or at the early stages of the illness. Covert depression was appropriately excluded. Fully established MJD in this family seemed to exert a protective effect from depression.

Machado-Joseph disease of Azorean ancestry in Brazil: the Catarina kindred; neurological, neuroimaging, psychiatric and neuropsychological findings in the largest known family, the Catarina kindred

Há, até o momento, notícia de 9 famílias näo aparentadas com o diagnóstico clínico de doença de Machado-Joseph (MJD) no Brasil. Esta é a maior família do mundo com doença. É de origem açoriana e contem 622 indivíduos na árvore fenealógica. Destes, 236 foram examinados. Dois examinadores julgaron 39 como afetados. Respectivamente 12, 23 e 4 pacientes tinham os fenôtipos I, II e III da doença, com idades no início variando entre 10-48, 14-54 e 30-55. Doença tipo I de início juvenil näo mostrou atrofia täo severa nas imagens por ressonância magnética (RM) quanto doença tipo II de duraçäo igual, demonstrando que severidade clínica e grau de atrofia näo caminham paralelamente. Nenhum dos 8 pacientes examinados por RM tinha atrofia olivar ou anormalidades no globo pálido. Doze pacientes e 23 sob risco foram submetidos a avaliaçäo neuropsicológica. A atençäo foi normal em todos. Memória verbal estava pior nos doentes com maior decaimento com o tempo que nos sob risco. Ambos os grupos tiveram pontuaçäo abaixo do normal na identificaçäo de silhuetas e praxia construtiva. Memória visual estava bem abaixo do normal para ambos, com muitas rotaçöes, porém sem omissöes ou confabulaçäo. Padräo peculiar de multiplicaçäo dos detalhes internos, que denominamos o "efeito olho de mosca" foi visto em 6 doentes e 8 sob risco. Discriminaçäo defeituosa de cores, quando múltiplas cores eram apresentadas lado a lado, na ausência de anomia ou cegueira a cores, caracterizável como "simultagnosia a cores", surgiu como achado e foi pesquisada em 29 sujeitos 4/10 doentes e 6/19 sob risco mostram esta dificuldade. Conclui-se que disfunçöes cognitivas na esfera visual säo proeminentes nesta família. Se seriam próprias da doença e manifestaçäo precoce daqueles sob risco, está ainda para ser estabelecido. Depressäo foi avaliada com critérios do DSM III-R e com o Montgomery-Asberg Depression Rating Scale. Näo houve diferença entre doentes e sob risco. Entretanto, os pacientes tiveram menos depressäo do que tinham tido antes ou nas fases precoces da doença. A MJD plenamente instalada parecia exercer efeito protetor da depressäo