Motor neuron diseases in the University Hospital of Fortaleza (Northeastern Brazil): a clinico-demographic analysis of 87 cases

In this retrospective (1980-1998) study, we have analyzed clinico-demographically, from the records of the University Hospital of Fortaleza (Brazil), a group of 87 patients showing signs and symptoms of motor neuron diseases (MNDs). Their diagnosis was determined clinically and laboratorially. The WFN criteria were used for amyotrophic lateral sclerosis (ALS) diagnosis. The clinico-demographic analysis of the 87 cases of MNDs showed that 4 were diagnosed as spinal muscular atrophy (SMA), 5 cases as ALS subsets: 2 as progressive bulbar paralysis (PBP), 2 as progressive muscular atrophy (PMA) and 1 as monomelic amyotrophy (MA), and 78 cases of ALS. The latter comprised 51 males and 27 females, with a mean age of 42.02 years. They were sub-divided into 4 groups according to age: from 15 to 29 years (n= 17), 30 to 39 years (n= 18), 40 to 69 years (n= 39) and 70 to 78 years (n= 4). From the 78 ALS patients, 76 were of the classic sporadic form whilst only 2 were of the familial form. The analysis of the 87 patients with MNDs from the University Hospital of Fortaleza showed a predominance of ALS patients, with a high number of cases of juvenile and early onset adult sporadic ALS

Epilepsia partialis continua (Koshevnikov): a preliminary case report

We report on the preliminary clinical and electrophysiological aspects of an in-patient possibly presenting epilepsia partialis continua (Koshevnikov). We discuss the different etiologies and emphasize on the possible idiopathic form in this case

Tropical spastic paraparesis in Northeastern Brazil

Os autores apresentam 10 casos de provável paraparesia espástica tropical (PET) oriundos do Nordeste do Brasil (Hospital das Clínicas - Universidade Federal do Ceará). Os sintomas consistem de fraqueza dos membros inferiores, marcha espástica hiperreflexia, disfunçäo esfincteriana e sinais variados de distúrbio proprioceptivo. Outras etiologias de mielopatias foram excluídas pelos exames complementares. A idade dos pacientes ia de 21 a 59 anos, todos eram da raça negra e havia discreto predomínio de mulheres. Recentemente, tem-se atribuído ao retrovírus HTLV-I a etiologia da PET. Por falta de condiçöes técncias ainda näo pudemos determiná-la em nossos pacientes

A propósito de um caso de associaçäo de polimiosite com miastenia gravis tardia

Os autores relatam um caso clínico de associaçäo de polimiosite com miastenia gravis, dissertando sobre as características específicas de cada patologia assim como a possibilidade e significado etiopatológico da associaçäo das duas. Enfatizam as medidas terapêuticas tomadas e seus resultados, e a forma rara de polimiosite seguida posteriormente de miastenia