Incidence Of Motor Neuron Disease Research Articles

Increased incidence of motor neuron disease in Sweden: a population-based study during 2002–2021

BackgroundMotor neuron diseases (MND), with amyotrophic lateral sclerosis constituting most cases, are rare conditions of unknown etiology. There have been reports of an increase in incidence during the latter half of the twentieth century in various Western countries, including Sweden. This study provides updated data on the incidence of MND in Sweden during the last 20 years.MethodsData was obtained from the Swedish National Patient Register on individuals diagnosed with MND from 2002 to 2021 and analysed in relation to group level data for the entire Swedish population. Incidence rates were calculated and presented in relation to year, age, sex, and region.ResultsIn the early 2000s, there was a crude incidence rate of 3.5–3.7 per 100,000 person-years, which then increased to 4.0–4.6 from 2008 onward. Age standardization to the starting year (2002) partially mitigated this increase. The incidence rate was greater among men compared to women and was highest within the age range of 70 to 84 years. There were indications of a higher incidence rate in the northernmost parts of the country, although the difference was not statistically significant.ConclusionsThe incidence rate of MND in Sweden now seems to have surpassed 4 cases per 100,000 person-years. This is higher when compared to both other European countries and previous Swedish studies. It remains to be determined if this increase reflects an actual increasing incidence of MND in Sweden or is due to other factors such as better registry coverage.

Incidence of Motor Neuron Disease (MND) in Patients Diagnosed with Cervical Spondylotic Myelopathy (CSM)

Objective: Motor neuron disease (MND) can occur in patients with cervical spondylotic myelopathy. The purpose of this study was to determine the prevalence of motor neuron disease in individuals suffering from cervical spondylotic myelopathy (CSM). Materials & Methods: A prospective study was conducted at the Lady Reading Hospital. The patients(n = 70) included were between ages 55 to 75 with established clinical and radiological diagnoses of CSM. Motor neuron disease was diagnosed clinically by Awaji criteria. The patients were evaluated with NCS/EMG (Nerve Conduction Studies/Electromyography) and followed for 6 months. The motor neuron disease was confirmed by modified El Escorial criteria. Results: 40 patients had bilateral upper limb weakness (57%), however, the rest of the 30 patients had unilateral upper extremity weakness (42.8%). Hoffman’s sign was positive in 60% of patients. Lhermitte's sign was also positive in 31% of patients. The majority of patients (55.7%) were found with grade 4 for deltoid followed by 51.4% patients for biceps brachii. MRI showed anterior cord compression at the level of C5 and C6 in 27.14% of patients and C6 C7 in 32.8% of patients. 15.7% of patients were suspected of having both CSM and MND. Conclusion: CSM and MND can occur in the same patients. The incidence of MND is 5.7% in patients with cervical spondylotic myelopathy, hence in CSM patients, workup must be done for MND as it affects the outcome of surgery in these patients.

A Review on Effect of Physical Activity as an Exogenous Factor and Cognitive Change in Motor Neuron Disease

Motor neuron disease (MND) is a terrible neurodegenerative illness with a poor prognosis and significant impairment. Despite recent advances in symptomatic care, there are few medicines that can affect survival. However, a better understanding of the underlying etiology would substantially aid the task of finding effective treatments. In the etiology of MND, many potential external risk factors have been hypothesized as part of a gene-environment interaction. Following reports of a greater than predicted incidence of MND in professional athletes, there has been an increasing interest in the role of intensive physical exercise in the development of the disease. Current hypotheses about the cellular and genetic causes of MND also support this conclusion. Epidemiological evidence, on the other hand, is contradictory and inconclusive. FTD/motor neuron disease is the name given to a motor neuronopathy that complicates frontotemporal dementia (FTD) (MND). FTD is marked by severe personality changes, abnormal social behavior, and executive difficulties caused by frontal and temporal neocortical atrophy. Bulbar palsy and limb amyotrophy are symptoms of motor neuron disease. Micro vacuolation of the cerebral cortex is the most common histological alteration, along with atrophy of the bulbar neurons and anterior horn cells of the spinal cord. Large pyramidal cortical neurons, surviving cranial nerve nuclei, and anterior horn cells all have ubiquitinated inclusion bodies. Evidence is accumulating that some patients with classical MND/amyotrophic lateral sclerosis (ALS) who are not regarded to be demented show frontal executive function abnormalities. Moreover, frontal lobe abnormalities have been demonstrated by neuroimaging. Keywords: MND, FTD, ALS, motor neuron, behavior, physical activity, cognitive change.

Analysis of incidence of motor neuron disease in England 1998–2019: use of three linked datasets

Objective: This study uses three linked datasets to provide an estimate of incidence of motor neuron disease (MND) in England from 1998 to 2019. Comparison is made to previous British studies. It examines age at diagnosis and ethnicity of those affected. Methods: The literature was searched for studies of MND incidence in Great Britain from 1995 to date. The QResearch and linked Hospital Episode Statistics and Death register databases were searched from 1998 to 2019 for cases of MND, and incidence calculated from 16.8 million adults and 112 million adult years of data. Results: We found 6437 adults with a diagnosis of MND giving an incidence of MND of 5.69/100,000 person years (95% CI 5.51–5.88); 6.57 (6.41–6.99) in men and 4.72 (4.49–4.97) in women when age-standardized to the 2011 UK population. The median age of diagnosis was 72 years. Peak incidence occurred in the 80–84 year age group in men and 75–79 in women. Age-standardized incidence was as high in Bangladeshi, Black Caribbean, Indian, other Asian and Pakistani people as in White people. Black African and Chinese people had a lower incidence. Conclusion: The use of three linked national datasets captured 33% more people than a primary care dataset alone. Patients were older than in previous studies and rates were high in all ethnic groups studied except Black African and Chinese people. We present the highest incidence of MND reported globally in the past 50 years. Methodological differences may in part explain differences with previous reports. The use of national datasets may have captured additional MND patients with serious comorbidities who have not seen a neurologist before death. A limitation of this approach is that unlike population registers, which minimize false positive diagnosis by neurologist review of each patient, we cannot review diagnosis for individuals as data are anonymized.

Motor neuron disease mortality rates in New Zealand 1992–2013

Background: We determined the mortality rates of motor neuron disease (MND) in New Zealand over 22 years from 1992 to 2013. Previous studies have found an unusually high and/or increasing incidence of MND in certain regions of New Zealand; however, no studies have examined MND rates nationwide to corroborate this. Methods: Death certificate data coded G12.2 by International Classification of Diseases (ICD)-10 coding, or 335.2 by ICD-9 coding were obtained. These codes specify amyotrophic lateral sclerosis, progressive bulbar palsy, or other motor neuron diseases as the underlying cause of death. Mortality rates for MND deaths in New Zealand were age-standardized to the European Standard Population and compared with rates from international studies that also examined death certificate data and were age-standardized to the same standard population. Results and Conclusion: The age-standardized mortality from MND in New Zealand was 2.3 per 100,000 per year from 1992–2007 and 2.8 per 100,000 per year from 2008–2013. These rates were 3.3 and 4.0 per 100,000 per year, respectively, for the population 20 years and older. The increase in rate between these two time periods was likely due to changes in MND death coding from 2008. Contrary to a previous regional study of MND incidence, nationwide mortality rates did not increase steadily over this time period once aging was accounted for. However, New Zealand MND mortality rate was higher than comparable studies we examined internationally (mean 1.67 per 100,000 per year), suggesting that further analysis of MND burden in New Zealand is warranted.

Farming and incidence of motor neuron disease: French nationwide study

The association of farming with motor neuron disease (MND) is unclear, with conflicting studies. We performed a French nationwide study of the association of farming with MND incidence, and compared findings with those for Parkinson's disease (PD), which has been shown to be more frequent in farmers. We used the French national health insurance and hospital discharge databases to identify MND/PD incident cases. The Mutualité Sociale Agricole (MSA) guarantees health insurance for farmers and agricultural workers. We compared the incidence of MND (2010-2014) and PD (2011-2012) in MSA farmers, MSA workers and non-MSA affiliates, and estimated relative risks (RRs) and 95% confidence intervals (CIs). Probabilistic sensitivity analyses were used for external smoking adjustment. Analyses relied on 8931 MND (MSA, 9%) and 45409 PD (MSA,11%) cases. There was a trend towards higher MND incidence in MSA farmers compared with non-MSA affiliates (RR,1.08; 95% CI,0.99-1.18) and MSA workers (RR, 1.13; 95% CI, 0.97-1.31) that strengthened after smoking adjustment (if associated with MND). PD incidence was higher in MSA farmers than non-MSA affiliates (RR, 1.13; 95% CI, 1.08-1.17) and MSA workers (RR, 1.10; 95% CI, 1.02-1.18); this association remained after smoking adjustment (RR, 1.09; 95% CI, 1.05-1.14). This French nationwide study suggested an association between farming and MND, and confirmed higher PD incidence in farmers in France, a country with high pesticide use.

Nationwide incidence of motor neuron disease using the French health insurance information system database

Objective: There are no estimates of the nationwide incidence of motor neuron disease (MND) in France. We used the French health insurance information system to identify incident MND cases (2012–2014), and compared incidence figures to those from three external sources. Methods: We identified incident MND cases (2012–2014) based on three data sources (riluzole claims, hospitalisation records, long-term chronic disease benefits), and computed MND incidence by age, gender, and geographic region. We used French mortality statistics, Limousin ALS registry data, and previous European studies based on administrative databases to perform external comparisons. Results: We identified 6553 MND incident cases. After standardisation to the United States 2010 population, the age/gender-standardised incidence was 2.72/100,000 person-years (males, 3.37; females, 2.17; male:female ratio = 1.53, 95% CI1.46–1.61). There was no major spatial difference in MND distribution. Our data were in agreement with the French death database (standardised mortality ratio = 1.01, 95% CI = 0.96–1.06) and Limousin ALS registry (standardised incidence ratio = 0.92, 95% CI = 0.72–1.15). Incidence estimates were in the same range as those from previous studies. Conclusions: We report French nationwide incidence estimates of MND. Administrative databases including hospital discharge data and riluzole claims offer an interesting approach to identify large population-based samples of patients with MND for epidemiologic studies and surveillance.

An Elderly Woman With Difficulty Reading and Abnormal Eye Movements

An Elderly Woman With Difficulty Reading and Abnormal Eye Movements

Incidence and lifetime risk of motor neuron disease in the United Kingdom: a population‐based study

To estimate the incidence and lifetime risk of motor neuron disease (MND) in a population-based sample in the United Kingdom. We identified new cases of MND during the period 1990-2005 in the General Practice Research Database, which includes clinical information from more than 3 million Britons enrolled with selected general practitioners. Individuals with a first medical diagnosis of MND recorded in the database were considered incident cases of the disease. The positive predictive value of the computer-based diagnosis was estimated through review of a sample of medical records from potential MND cases. In the period 1990-2005, 830 new cases of MND were identified. Age-standardized incidence of MND was 2.6 per 100 000 persons per year in women (95% CI: 2.3, 2.8) and 3.9 in men (95% CI: 3.6, 4.3). Incidence for both sexes peaked between 75 and 79 years. The rate of MND in men was 54% higher than in women (95% CI: 33%, 77%). The lifetime risk of MND, adjusting for competing causes of death, was 1 in 472 (2.1 per 1000) in women and 1 in 350 (2.9 per 1000) in men. No increase in MND incidence over time was apparent. In this population-based database, we found that MND incidence is higher in men than women, peaking in both sexes between 75 and 79 years.

An epidemiological study of motor neuron disease in Hong Kong

Worldwide, the incidence of motor neuron disease (MND) has been increasing steadily over recent decades. We reported a follow-up epidemiology study of MND in this locality. We identified the subjects from the computer database of the government hospital system between 1 January 1997 and 31 January 2002 by searching the ICD code starting from 335.xx. Every retrieved case or their records were reviewed and validated by neurologist(s) of the responsible regional hospitals which the patients attended. One hundred and twenty cases from seven regional hospitals (serving 48.05% of the HKSAR population) were identified, validated and confirmed to be MND or related diseases. Ninety-eight new cases were diagnosed during the study period. Average age of onset was 58.76 years; SD 14.12 (28-89) years. Male to female ratio was 1.72:1. Peak age of onset was 60-64 years without sex difference. The adjusted incidence rate was 0.60/100,000/year. The adjusted point prevalence at the prevalence date (31 January 2001) was 3.04/100,000. Despite the incidence and prevalence of MND among Hong Kong Chinese, it remained low compared to worldwide figures, and our data suggested a significant rise of MND or related disease in the last decade. A territory-wide prospective epidemiological study is indicated.

Geographical epidemiology of residence of patients with motor neuron disease in Lancashire and south Cumbria

OBJECTIVESTo seek objective evidence for geographical clustering of places of residence of patients with motor neuron disease (MND).METHODSA complete residential history from birth to onset of disease was obtained from...

Motor neuron disease on the Kii Peninsula of Japan: excess manganese intake from food coupled with low magnesium in drinking water as a risk factor

To identify important risk factors of motor neuron disease (MND) in a focus (Hohara) on the Kii peninsula of Japan, 24-h dietary duplicates, rice, drinking water, and soils were analysed for metals in Hohara and control areas. The manganese content in 24-h food in Hohara was significantly ( P < 0.01) higher than that in the remote control areas. The incidence of MND in Hohara was well explained by the two parameters, manganese content in food and the magnesium concentration in drinking water ( r 2 = 0.99), suggesting that MND in this focus can be understood as a result of excess intake of manganese from food coupled with low intake of magnesium from drinking water. The reference to epidemiology and occupational medicine supports this conclusion. The crude annual MND incidence per 10 5 population in other foci may be predicted as MND = e α ln[ β(Mn in food) γ /(Mg in water)], where α is 5.389–5.748, β is 0.170–0.166, and γ is 1.239–1.226.

Motor neuron disease in a defined English population: estimates of incidence and mortality.

Linked statistics from hospital records and death certificates were used to study the incidence of and mortality from motor neuron disease in a defined English population. The incidence of motor neuron disease, measured as first-admission rates for the disease, was studied from 1963 to 1985 and death certificates for the patients admitted to hospital were obtained to the end of 1990. The average annual first-admission rate for motor neuron disease was 2.1/100,000 men (95% confidence interval (CI) 1.9 to 2.4) and 1.7/100,000 women (95% CI 1.5 to 1.9). First-admission rates increased with age and peaked in the age range 65-84 years. Motor neuron disease was recorded on the death certificate for 86% of patients who died while they had the disease and there was no appreciable change over time in the recording of motor neuron disease as the underlying cause of death. The admission and mortality data derive from different sources, hospital statistical abstracts and death certificates respectively, but trends over time in the two data sets were similar. There was an increase in mortality during the period covered by the study, as there has been in mortality from motor neuron disease nationally, and the increase in mortality in the Oxford region was accompanied by an increase in first-admission rates. It is concluded that the increase in mortality from motor neuron disease probably occurred as a result of an increase in the diagnosed incidence of the disease rather than changes in death certification practice.

Motor Neuron Disease on the Kii Peninsula of Japan: Cycad Exposure

In the Hohara area of Nansei-cho, one of the foci of motor neuron disease (MND) on the Kii Peninsula, an inquiry was conducted to investigate whether cycad exposure is an etiological factor of MND in the western Pacific. Although cycads were commonly used as medicine around World War II all over Japan, the medicinal use of cycads was exceptional in the Hohara area. A questionnaire study in the Hohara and the neighboring four areas in Nansei-cho showed that cycad used as food, medicine, toys, and garden trees had no relation to the incidence of MND in these areas. The result in the Hohara area does not support the hypothesis that cycad is an etiological factor of MND common to the western Pacific foci.

Motor neuron disease in Cantabria.

Sixty-two patients with motor neuron disease (MND), encompassing amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP) and progressive muscular atrophy (PMA), were selected from within a defined area (Cantabria) in northern Spain, from 1974 to 1985. The annual incidence of MND was 1.01 per 100,000 inhabitants and the prevalence rate was 3.52 per 100,000. The male to female ratio was 1.78:1. Age-specific incidence rates increased with advanced age, with a maximum between 60 and 69 years for males and over 70 years for females. The median age at onset was 60.5 years. The average interval between the onset symptoms and diagnosis was 11 months. Fifty-three per cent of the patients had conventional or pseudopolyneuritic ALS, 36% had PBP and 11% had PMA. There were three familial cases. Two PMA patients had had acute poliomyelitis. The mean duration of the disease was 26.6 months and was significantly longer in males aged under 60 years. The survival rates in 50 patients with adequate follow-up were 18% after 5 years from onset and 6% after 10 years.

Descriptive epidemiology of motor neuron disease in Benghazi, Libya.

A total of 23 patients with motor neuron disease (MND), encompassing 17 cases of amyotrophic lateral sclerosis, 4 of progressive muscular atrophy and 2 of progressive bulbar palsy, was diagnosed in Benghazi, north-eastern Libya, between 1980 and 1985. The male to female ratio was 2.3:1. The average incidence of MND was 0.89/100,000 population/year (0.87 when age and sex-adjusted to the Libyan population). Eighteen patients were alive on the prevalence day, September 15, 1985, which provided a prevalence rate of 3.47/100,000 population (3.42 if adjusted). Age-specific incidence rates were highest in the 50- to 59-year-old age group, 8.14/100,000/year for men and 6.10/100,000/year for women. The median age at the time of diagnosis was 51 years, and the median duration for the 5 dead MND patients after the onset of the disease was 30 months. The median survival time for all MND cases combined was 42 months.

Study of the effects of environmental factors on the local incidence of amyotrophic lateral sclerosis

The etiology of amyotrophic lateral sclerosis has remained unknown since the disease was first described by Charcot and Joffroy in 1869. Although extensive epidemiological and genetic studies were carried out in the past several years in the Kii Peninsula of Japan as well as on Guam in the Mariana Islands, these studies did not allow a determination of what environmental factors induced the disease, and conclusive genetic factors have not emerged. In a search for possible etiological factors in the environment, the role of heavy metals is considered because a possible concentration of amyotrophic lateral sclerosis and Parkinsonism-dementia among former manganese miners on the Island of Guam was suggested in 1964. Previous chemical analyses of trace metals in human nervous system tissue and in environmental samples of river and drinking water, soil, plants, and fish provided no remarkable findings, because of the very small amounts of heavy metal ions in the human nervous system and because of the complicated chemical behavior of the metal ions in the environment. Then a further highly sensitive analysis by the neutron activation method was undertaken on materials from the Kii Peninsula in Japan and from Guam, both areas having a high incidence of motor neuron disease. The results show that, on Guam, the samples of river and drinking water have quite a high content of manganese; however, in Japan, factors of manganese encephalopathogeny are not observed and, rather, the low content of calcium and magnesium ions in water may play an important role in the cause of the disease.

Motor neuron disease in Lehigh County, Pennsylvania: An epidemiologic study

Thirty-one cases of motor neuron disease (MND) were diagnosed in residents of Lehigh County, Pennsylvania, in 1968–1975. Independently reviewed by two neurologists, these cases satisfy explicit diagnostic criteria for one of the three variants of MND—amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA), and progressive bulbar palsy (PBP). The annual incidence of MND adjusted for sex and age to the 1950 United States population was 1.32 cases per 100,000 population per year; this rate was almost identical to the only other reported rate for an area of the continental United States. The point prevalence for Lehigh county on 15 November, 1975 was 6.4 cases per 100,000 population.