Esclerose lateral amiotrófica variante distal dos membros inferiores apresentação de três casos

Godoy, J. M.; Skacel, M.; Balassiano, S. L.; Neves, Joana; Filho, G. S. Goffredo; Castro, Rodrigo; Nicaretta, Denise Hack; Souza, R. G. S.; Mendes, Marcelo Heitor Ferreira

doi:10.1590/s0004-282x1991000300015

Cited by 2 publications

(2 citation statements)

References 6 publications

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“…According to the literature, the average survival of ALS patients s approximately of 3 years, with a duration ranging from 25 to 52 months 16,17 . Our small series of only 6 patients revealed concordant figures, with a mean duration of 2.5 years, and a median duration of 1 year, with a range of 12 to 72 months.…”

Section: Discussionmentioning

confidence: 99%

Amyotrophic lateral sclerosis: clinical analysis of 78 cases from Fortaleza (Northeastern Brazil)

Castro-Costa

Oriá

Machado-Filho

et al. 1999

Arq. Neuro-Psiquiatr.

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-We report on the clinical characteristics of amyotrophic lateral sclerosis (ALS) in Fortaleza (Northeastern Brazil). For this, we analyzed retrospectively (from 1980 to 1999) 78 cases of ALS from the Service of Neurology of the University Hospital of Fortaleza diagnosed clinically and laboratorially (EMG, muscle biopsy, myelography, blood biochemistry, muscle enzymes and cranio-cervical X-ray). The results showed that they were mostly sporadic ALS (76/78), and they were divided into definite (n= 36), probable (n= 20), possible (n= 15) and suspected (n= 7), according to the level of diagnostic certainty. They were also subdivided into juvenile (n= 17), early-onset adult (n= 18), age-specific (n= 39) and late-onset (n= 4) groups. Clinically, they presented as initials symptoms, principally, asymmetrical (30/78) and symmetrical (24/78) weakness of extremities, besides bulbar signs, fasciculations, and atrophy. Curiously, pain as first symptom occurred in an expressive fashion (17/78). The predominant initial anatomic site, in this series, was the spinal cord, and mainly affecting the arms. As to the symptom accrual from region to region, this occurs more quickly in contiguous areas, and fasciculations are predominant when bulbar region was associated.KEY WORDS: ALS, sporadic, early-onset, pain, anatomy, symptom accrual, fasciculations. Esclerose lateral amiotrófica: análise clínica de 78 casos de Fortaleza (Nordeste Brasileiro)RESUMO -Descrevemos as características clínicas da esclerose lateral amiotrófica (ELA) em Fortaleza (Nordeste Brasileiro). Para isso, fizemos uma análise retrospectiva (de 1980 a 1999) de 78 casos de ELA do Serviço de Neurologia do Hospital Universitário de Fortaleza, diagnosticados do ponto de vista clínico e laboratorial (EMG, biópsia de músculo, mielografia, análises hematológica e bioquímica, e raio X da junção crânio-cervical). Os resultados mostraram que esses casos eram principalmente da forma esporádica (76/78), e estes foram divididos, de acordo com o nível de certeza diagnóstica, em definidos (n= 36), prováveis (n= 20), possíveis (n= 15) e suspeitos (n= 7). Eles foram também subdivididos nos grupos juvenil (n= 17), adulto jovem (n= 18), idadeespecífico (n= 39) e tardio (n= 4). Clinicamente, os principais sintomas iniciais foram fraqueza assimétrica (38/ 78) e simétrica (24/78) das extremidades, além de sinais bulbares, fasciculações e atrofia. Curiosamente, dor como sintoma inicial ocorria de maneira expressiva (17/78). O local anatômico inicial predominante foi, nessa série, a medula espinhal, afetando principalmente os braços. O avanço regional de sintomas ocorreu mais rapidamente em áreas contíguas, e as fasciculações eram predominantes quando a região bulbar estava associada. PALAVRAS-CHAVE: ELA, esporádica, início precoce, dor, anatomia, avanço dos sintomas, fasciculações.Amyotrophic lateral sclerosis (ALS) is a condition of undetermined cause, which affects the anterior horn cells and the corticospinal tract.The etiology is manifold so that different causes have been...

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Section: Discussionmentioning

confidence: 99%

Amyotrophic lateral sclerosis: clinical analysis of 78 cases from Fortaleza (Northeastern Brazil)

Castro-Costa

Oriá

Machado-Filho

et al. 1999

Arq. Neuro-Psiquiatr.

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“…The authors classified the neurological disease as ALS, more specifically as a pseudopolyneuritic form of ALS or Patrikios' form, which starts in the lower limbs causing a reduction or abolition of deep tendon reflexes. 17 They did not rule out that the action of toxins or anemia related to the tuberculosis may have contributed to the muscle atrophy, but the association with intestinal tuberculosis seemed fortuitous to them.…”

Section: Tretiakoff and Amorim's Hypothesismentioning

confidence: 99%

Frontotemporal dementia and amyotrophic lateral sclerosis: Revisiting one of the first case reports with neuropathological examination

Nitrini

2014

Dement. neuropsychol.

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The occurrence of dementia in amyotrophic lateral sclerosis (ALS) was only widely recognized in the late 20th century. Hitherto, it was believed that dementia was a rare event due to the fortuitous association with other diseases. In 1924, Kostantin Nikolaevich Tretiakoff and Moacyr de Freitas Amorim reported a case of dementia with features of frontotemporal dementia (FTD) that preceded the motor signs of ALS. Neuropathological examination confirmed ALS and found no signs of other dementia-causing diseases. The authors hypothesized that dementia was part of ALS and recommended the search for signs of involvement of motor neurons in cases of dementia with an ill-defined clinical picture, a practice currently accepted in the investigation of cases of FTD. This was one of the first descriptions of dementia preceding the motor impairments of ALS and was published in Portuguese and French in Memórias do Hospício de Juquery.

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Esclerose lateral amiotrófica variante distal dos membros inferiores apresentação de três casos

Abstract: Os autores apresentam três casos de pacientes com esclerose lateral amiotrófica, variante de Patrikios, chamando a atenção para a importância do diagnóstico diferencial desta com as neuropatias periféricas.

Cited by 2 publications

References 6 publications

Amyotrophic lateral sclerosis: clinical analysis of 78 cases from Fortaleza (Northeastern Brazil)

Amyotrophic lateral sclerosis: clinical analysis of 78 cases from Fortaleza (Northeastern Brazil)

Frontotemporal dementia and amyotrophic lateral sclerosis: Revisiting one of the first case reports with neuropathological examination

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