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

• Published in: Arquivos de neuro-psiquiatria Vol. 58; no. 4; pp. 986 - 989
• Main Authors: CASTRO-COSTA, CARLOS M. DE, ORIÁ, REINALDO B., VALE, OTONI C. DO, ARRUDA, JOSÉ ARNALDO M. DE, HORTA, WAGNER G., D'ALMEIDA, JOSÉ ARTUR C., SANTOS, TEREZINHA J.T., RAMOS, RODRIGO S.N., GIFONI, MARCUS A.C.
• Format: Journal Article
• Language: English
• Published: Brazil Academia Brasileira de Neurologia - ABNEURO 01.12.2000; Thieme Revinter Publicações
• Subjects: Adolescent; Adult; Age Distribution; Aged; amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - diagnosis; Brazil; Bulbar Palsy, Progressive - diagnosis; Diagnosis, Differential; Female; Fortaleza (Brazil); Hospitals, University; Humans; Male; Middle Aged; Motor Neuron Disease - diagnosis; motor neuron diseases; Muscular Atrophy, Spinal - diagnosis; NEUROSCIENCES; PSYCHIATRY; Retrospective Studies; Risk Factors; Brazil; Fortaleza (Brazil); motor neuron diseases; amyotrophic lateral sclerosis; Fortaleza (Brasil); doenças do neurônio motor; esclerose lateral amiotrófica
• ISSN: 0004-282X; 1678-4227; 0004-282X; 1678-4227
• DOI: 10.1590/S0004-282X2000000600002

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. Neste estudo retrospectivo (1980-1998), analisamos clínico-demograficamente, a partir dos prontuários do Hospital Universitário de Fortaleza (Brasil), um grupo de 87 pacientes que apresentavam sinais e sintomas de doenças do neurônio motor (DNMs). Eles foram diagnosticados clinicamente, e através de exames complementares. Para o diagnóstico da esclerose lateral amiotrófica (ELA), usamos os critérios da Federação Mundial de Neurologia. A análise clínico-demográfica dos 87 casos de DNMs evidenciou a existência de 4 casos de atrofia muscular espinhal (AME), 5 casos de variantes da ELA: 2 de paralisia bulbar progressiva (PBP), 2 de atrofia muscular progressiva (AMP) e 1 de amiotrofia monomélica (AM), e 78 casos de ELA. Esses últimos eram constituídos de 51 homens e 27 mulheres, com uma idade média de 42,02 anos. Eles foram subdivididos em 4 grupos etários: de 15 a 29 anos (n= 17), de 30 a 39 anos (n= 18), de 40 a 69 anos (n= 39) e de 70 a 78 anos (n= 4). Dos 78 casos de ELA, 76 eram da forma esporádica clássica, enquanto que apenas 2 da forma familiar. A análise dos 87 pacientes com DNMs do Hospital Universitário de Fortaleza mostrou predomínio de pacientes com ELA, com um número elevado de casos de ELA juvenil e da forma adulta de início precoce.