Motor neurone disease: can we do better? A study of 42 patients.

Newrick, P. G.; Langton-Hewer, R

doi:10.1136/bmj.289.6444.539

Cited by 61 publications

(22 citation statements)

References 5 publications

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“…However, during recent years a change of attitude is developing towards a more active management of MND [8,9], resulting in an increased need for care and health service of these disabled patients and their families. For planning of management and care, knowledge of the epidemiology of MND is essential.…”

Section: Introductionmentioning

confidence: 99%

Incidence and Prevalence of Motor Neuron Disease in Two Danish Counties

Højer-Pedersen

Christensen²,

Jensen³

1989

Neuroepidemiology

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A total of 186 cases of motor neuron disease (MND) was identified in two Danish counties during the period 1974–1986. The average annual incidence rate was 1.4/100,000 population, and the male:female ratio of incidence rates was 1.5. Mean age at diagnosis was 64.3 ± 10.0 years. The incidence rates increased significantly with advancing age and reached a maximum at age 60–79 years, followed by a nonsignificant decrease. The average point prevalence was 3.1/100,000 population. Bulbar symptoms were part of the initial symptoms in 65 % of cases, and patients with bulbar onset were older than patients with spinal onset. Age- and sex-specific incidence rates indicated a marked male preponderance amongst the youngest patients, in contrast to a female preponderance in patients above 60 years with bulbar onset of MND. Familial MND accounted for 2.7% of cases.

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Section: Introductionmentioning

confidence: 99%

Incidence and Prevalence of Motor Neuron Disease in Two Danish Counties

Højer-Pedersen

Christensen²,

Jensen³

1989

Neuroepidemiology

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“…The scale does not lend itself readily to communication between physi cians and does not provide ordinal ratings on which to base treatment planning. Other scales are limited due to length [2], a need for special equipment [3], or lack of an ordinal basis [4][5][6][7],…”

Section: Introductionmentioning

confidence: 99%

Amyotrophic Lateral Sclerosis Severity Scale

et al. 1989

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The amyotrophic lateral sclerosis (ALS) severity scale has been developed to provide an ordinal staging system and a means of rapid functional assessment for patients with ALS. The scale allows an examiner to evaluate the symptoms of ALS numerically in four categories that describe speech, swallowing, lower extremity, and upper extremity abilities. These scores, combined with a vital capacity measured on a hand-held respirometer, provide a rapid, accurate assessment of a patient''s disease status and can be used for treatment planning. The ALS severity scale has been shown to have an average estimated reliability coefficient of 0.95 between examiners. Speech ratings were correlated >0.80 for objective speech measures. Rates of progression of the total score in a small group of patients ranged from –3.4 to –24.0 points/year with a mean of –11.3 points/year.

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“…Most frequently, pain in ALS is associated with reduced joint mobility, cramps and hypertonia 4 . However, pain in these patients is described as diffuse aching, burning and electric, or shocking-like in nature 20 , what points to its possible neuropathic origin.…”

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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Motor neurone disease: can we do better? A study of 42 patients.

Cited by 61 publications

References 5 publications

Incidence and Prevalence of Motor Neuron Disease in Two Danish Counties

Incidence and Prevalence of Motor Neuron Disease in Two Danish Counties

Amyotrophic Lateral Sclerosis Severity Scale

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

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