Misdiagnosis in Patients With Amyotrophic Lateral Sclerosis

Belsh, Jerry M.; Schiffman, Philip L.

doi:10.1001/archinte.1990.00390220055011

Cited by 62 publications

(29 citation statements)

References 31 publications

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“…Our study sug- gests that overall the death rate for whites was higher than that for blacks or other races. While better access to health care resulting in more accurate and frequent diagnosis of ALS among whites is one possibility for rate differences [29] , a race-specifi c exposure factor cannot be excluded. States in higher latitudes ('northern' states) appear more likely to have ALS-associated age-adjusted death rates that are higher than those for most southern states and a rate in the upper quartile of the state-specifi c rates.…”

Section: Discussionmentioning

confidence: 99%

Amyotrophic Lateral Sclerosis Mortality in the United States, 1979–2001

Sejvar

Holman²,

Bresee³

et al. 2005

Neuroepidemiology

101

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The etiology of nonfamilial amyotrophic lateral sclerosis (ALS) remains unknown. Earlier studies have suggested an increase in the incidence of ALS over time. We performed a retrospective analysis of ALS-associated death rates and trends in the United States for 1979–2001 using death records from the national multiple cause-of-death database. The US average annual age-adjusted ALS death rate was 1.84 per 100,000 persons for 1979 through 1998. Most deaths were among adults ≧65 years of age and the median age at death was 67 years. A small overall increase in the death rate was observed primarily between 1979 and 1983, with a subsequent plateau. This slight change in the overall rate reflected apparent increases in the rates among those persons ≧65 years of age, particularly women, and persons in the 20- to 49-year-old age group. The ALS-associated death rate appeared to differ by geographic area, with a higher occurrence among most northern states. Our findings suggest that the epidemiology of ALS-associated deaths in the United States demonstrated small increases in the overall age-adjusted death rate and in the death rates among elderly women and adults 20–49 years of age. Subpopulations at higher risk for ALS were males, whites, persons ≧65 years of age, and residents of northern states. This study provides information for further studies to examine the epidemiology and risk factors associated with ALS.

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Section: Discussionmentioning

confidence: 99%

Amyotrophic Lateral Sclerosis Mortality in the United States, 1979–2001

Sejvar

Holman²,

Bresee³

et al. 2005

Neuroepidemiology

101

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“…Differential diagnoses are less common than those of ALS itself, particularly in the bulbar form. Patients suffering from ALS are mostly diagnosed and followed in referral centers, and rates of misdiagnosis are reported to be less than 10% [19,20] even among those not followed in that way. In a study of the Irish register, 7% of patients had been diagnosed by a neurologist as having ALS with neuropathological investigations suggestive of ALS, but were finally considered to have another disease (ALS mimic syndromes) [21] .…”

mentioning

confidence: 99%

Can Mortality Data Be Used to Estimate Amyotrophic Lateral Sclerosis Incidence?

et al. 2010

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Background: Because studies of the incidence of amyotrophic lateral sclerosis (ALS) have uncertain feasibility and high costs, mortality rates are often used to provide an estimate. We performed a systematic review of the literature concerning mortality related to ALS. We aimed to use well-known criteria of good epidemiological practice to assess the methodological quality of the studies. Methods: A Medline and ScienceDirect literature search was performed to identify studies on ALS mortality published from 1971 to 2009. The literature was examined following 6 criteria. Results: Of the 29 studies examined, almost all presented a clear definition of the population at risk, but 55% of the papers did not report on the accuracy of death certificates, and the use of both ‘underlying’ and ‘contributory’ causes of death was identified in only 41% of cases. When comparing ALS mortality data between calendar dates, the codes from the International Classification of Diseases were consistent overall, except in 3 studies. A majority of articles that compared mortality patterns between geographical regions or ethnic groups discussed the key issues of comparability of health care and equality of access. Overall, among the 29 ALS mortality studies, only 3 complied with all the criteria. In 2 of them, the mortality rates were highly consistent with available incidence data. Conclusion: Only few studies on mortality data followed a high-quality methodology. When studies complied with the criteria, they showed good accuracy with regard to incidence rates. The criteria used in this study could also be used to guide future studies based on mortality data.

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“…2 According to guidelines proposed by the subcommittee on motor neuron diseases of the World Federation of Neurology Research Group on Amyotrophic Lateral Sclerosis, a diagnosis of ALS requires the presence of upper and lower motor neuron degeneration in the absence of neuroimaging evidence of any other process. 3 Our patient never met the established criteria for ALS; however, signal abnormalities in the basal ganglia were consistent with neurodegeneration, with brain iron accumulation excluding definite ALS-type motor neuron disease by strict criteria.…”

Section: Discussionmentioning

confidence: 99%

Adult Hallervorden–Spatz syndrome simulating amyotrophic lateral sclerosis

et al. 2003

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Hallervorden-Spatz syndrome (HSS) is a neurodegenerative disorder characterized by progressive dementia, dystonia, ataxia, and rigidity. An atypical form of adult-onset HSS was observed in a 36-year-old man presenting with progressive dysarthria. Markedly dysarthric speech and a weak atrophic tongue associated with a neurogenic pattern of motor unit recruitment in bulbar-supplied muscles on electromyography led to an initial impression of bulbar amyotrophic lateral sclerosis (ALS). Lack of expected progression of symptoms, however, prompted reinvestigation. Repeat brain magnetic resonance imaging demonstrated an "eye-of-the-tiger" pattern in the basal ganglia, characteristic of HSS, thus requiring genetic studies. DNA analyses of the pantothenate kinase gene (PANK2) was conducted and revealed two novel, disease-causing exon 3 missense mutations (Cys231Ser and Tyr251Cys). This case broadens the genotypic and phenotypic spectrum of HSS to include a late-onset syndrome resembling bulbar-onset ALS.

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Misdiagnosis in Patients With Amyotrophic Lateral Sclerosis

Cited by 62 publications

References 31 publications

Amyotrophic Lateral Sclerosis Mortality in the United States, 1979–2001

Amyotrophic Lateral Sclerosis Mortality in the United States, 1979–2001

Can Mortality Data Be Used to Estimate Amyotrophic Lateral Sclerosis Incidence?

Adult Hallervorden–Spatz syndrome simulating amyotrophic lateral sclerosis

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