Motor neurone disease in South Estonia Diagnosis and incidence rate

The current study evaluated the diagnostic standards of MND and epidemiological markers of MND in Estonia. A total of 108 patients were referred to the University Hospital from 1986 to 1995 with the first suggested diagnosis or final diagnosis of amyotrophic syndrome, amyotrophic lateral sclerosis (ALS), progressive bulbar paralysis (PBP) or progressive muscular atrophy (PMA). In addition neurologists of the region and the National Society of Neuromuscular disorders were contacted. Some 94 patients satisfied the diagnostic criteria. The annual incidence rate in South Estonia and in the city of Tartu ranged from 0.5 to 2.8 per 100,000. The mean annual incidence rate in Tartu is 1.98 and in South Estonia in general 1.3. The highest incidence rate was 8.3 for men in the age group 60 to 64 years and 7.49 in the age group 70–74; among female patients the highest incidence rate -4.6 was in the age group from 65 to 69.     

A descriptive study of epilepsy in the district of El Salvador, Chile, 1984–1988

To describe the epidemiological characteristics of epilepsy in a northern area of Chile, an investigation was conducted in four localities in the province of Copiapó (population of 17,694). Based on 314 cases of active epilepsy, the prevalence per 1000 at June 30, 1988 was 17.7. The average annual incidence for the period 1984-1988 was 113 per 100,000. Partial seizures were the most frequent type of seizure diagnosed (54.1%). Antecedents considered as possible etiological factors were found in 29.9% of cases. According to age of onset, 64.6% had their first attack before 15 years. Middle and low socioeconomic classes had higher prevalence rates of epilepsy. We compare our results with previous Latin-American studies.     

Motor neuron disease on Guam: geographic and familial Occurrence, 1956–85

We investigated the geographic and familial occurrence of motor neuron disease (MND) on Guam, and then considered etiologic hypotheses related to cycad use and metal intoxication. The research was based on 303 Chamorros from Guam and 3 Chamorros from other Mariana Islands, all with MND onset on Guam during 1956–85. Inarajan and Umatac, two southern districts, each had, for both sexes combined, an average incidence rate significantly higher than the corresponding overall rate for Guam. Also, for each sex, geographic patterns of incidence were significantly related to 1)socioeconomic level (men only), 2)cycasin concentrations in cycad flour samples (men and women), 3)iron concentrations in water samples (men and women), 4)silicon concentrations in water samples (men only), and 5)cobalt and nickel concentrations in soil samples (men and women). The MND risk in susceptible sibships was about 7–28 times greater than that in the general population. The cycad hypothesis conforms somewhat better than the metal intoxication hypothesis with the data presented.     

An epidemiological study of neuromyelitis optica in Cuba

Introduction   Two population-based studies of neuromyelitis optica (NMO) in non-white populations provided prevalence rates of 0.32 and 3.1 per 100,000 population. Objective   To estimate NMO prevalence in the multiethnic Cuban population by nation-wide case ascertainment. Methods   The study was conducted from October 1, 2003 to November 30, 2004 . Ninety percent of general practitioners and all neurologists responded positively to the request for information on cases suspected of optic neuritis (ON), transverse myelitis (TM), multiple sclerosis, or NMO. Among the population of 11,177,743 there were 798 suspected cases, including 89 with possible NMO, relapsing ON (RON) and TM. Of the 89, 87 were examined by two of us (Cabrera JA, Lara R) who selected the NMO cases according to the 1999 Mayo Clinic criteria as well as those with relapsing TM and RON. Results   58 cases provided a prevalence rate of 0.52 per 100,000 (95 % CI 0.39–0.67). The 7 males and 51 females gave rates of 0.13 (CI 0.05–0.26) and 0.91 (CI 0.68–1.20). The estimated average annual incidence rate was 0.053 per 100,000 (CI 0.040–0.068). Prevalence rates did not differ significantly among the three ethnic groups. Black NMO cases were significantly older, with more relapses and motor deficit, as well as more abnormalities in brainstem evoked potentials and in brain MRI (not meeting MS criteria). The predominant clinical form was relapsing over monophasic. Conclusions:   This Cuban multiethnic population had a prevalence of NMO of 0.52 per 100,000 and an estimated average annual incidence rate of 0.053 per 100,000 with no differences by ethnicity. Black patients were older, with more relapses and motor impairment. Statistical analyses were performed by Alina González-Quevedo, with contributionsby John F. Kurtzke.     

An epidemiological study of multiple sclerosis in central Sardinia, Italy

Objectives – To verify morbidity estimates in central Sardinia, Italy. Methods – A prevalence study was performed in the province of Nuoro, Central Sardinia, which has a population of 273,768 inhabitants (135,383 men and 138,385 women). A complete enumeration approach was adopted by using all possible case-collection sources. Results – On prevalence day, December 31, 1993, 394 subjects (124 men and 270 women) living in the study area were known to suffer from definite and probable MS, giving a crude prevalence rate of 143.9 cases per 100,000 people, 91.6 for males and 195.11 for females. The crude prevalence estimated on December 31, 1985, based on 282 MS cases alive in the study area, was 102.94 per 100,000. Conclusion – This study reinforced central Sardinia's position as a high and rising prevalence area for MS.     

Motor neuron disease on Guam: temporal xcurrence, 1941–85

Using a case registry, we investigated the temporal occurrence of motor neuron disease (MND) on Guam. MND with onset during 1941–85 was documented in 434 Chamorros and 9 non-Chamorro migrants who had lived on Guam before onset. Increased median age at onset and decreased age-adjusted incidence rates (since the early 1960s) were observed for Chamorros of both sexes. Our evidence about MND on Guam is consistent with: 1) The latent period duration has varied from years to decades; 2) With time, the exposure period or latent period, or both, have lengthened; 3) The high risk of acquiring the condition has been reduced since, at least, the early 1950s, and the most recent years of meaningful risk were the early to middle 1960s; 4) The critical age for acquiring the condition is in adolescence and adulthood; 5) Change of environment from Guam to overseas during childhood resulted in decreased risk of acquiring the condition.     

Stroke burden in Egypt: data from five epidemiological studies

Purpose: Accurate data on the epidemiology of stroke in Egypt is scarce. The aim of this review is to address this issue based on available community-based studies and compare the resulting findings to those of other regional and international studies.

Method: A systematic literature search was conducted to identify population-based epidemiological studies of stroke in Egyptians. Original articles published in English between 1990 and 2016 were included. Five studies from five different governorates in southern Egypt fulfilled the study criteria (Qena, Sohag, Assiut, New Valley and Red Sea).

Results: The mean and median crude prevalence rates (CPRs) across the five studies, which were conducted in southern Egypt were 721.6/100,000 and 655/100,000, respectively. The mean and median crude incidence rates (CIRs) were 187/100,000 and 180.5/100,000, respectively. The average CPR weighted by sample population size was 613/100,000 and the average CIR weighted by sample population size was 202/100,000.

Conclusion: The incidence and prevalence of stroke in Egypt are high. More population-based studies are urgently needed in northern Egypt and in Cairo – the capital of Egypt.     

Amyotrophic lateral sclerosis in Palermo, Italy: an epidemiological study

The incidence, prevalence and natural course of ALS were determined in the population of the province of Palermo, Italy. The average annual incidence calculated for the years 1973 through 1984, was 044./100.000 inhabitants. The prevalence rate on prevalence day December 31, 1984, was 1.67/100.000 population. The male/female ratio was 1.38. The mean age at onset was 54.3±11.02. The most common clinical form was the conventional one (61.4%); the bulbar form was more frequent among females than males. The mean duration of the disease was 33.7±35.8 months. The longest duration belongs to the pseudopolyneuritic form. The median survival was 36 months: 16 months for the bulbar, 36 months for the conventional and 51 months for the pseudopolyneuritic form.

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Sommario Questa indagine si prefigge di valutare l'incidenza, la prevalenza ed il decorso naturale della Sclerosi Laterale Amiotrofica nella provincia di Palermo nel periodo compreso tra il 1973 ed il 1984. L'incidenza annuale media è di 0,44/100.000 abitanti; il tasso di prevalenza al 31 Dicembre 1984 è di 1,67/100.000 abitanti. Il rapporto M/F risulta di 1,38; l'età media di esordio è di 54,3±11,01. La forma tipica è quella più comune (61,4%), mentre la forma bulbare è la più frequente tra i soggetti di sesso femminile. La durata media di malattia è di 33,7±35,8 mesi, sopravvivendo maggiormente i pazienti affetti dalla forma pseudopolineuritica. La sopravvivenza media è di 36 mesi. Una analisi differenziata per le singole forme evidenzia una sopravvivenza media di 16 mesi nella forma bulbare, di 36 mesi nella forma tipica e di 51 mesi nella forma pseudopolineuritica.

     

Motor neuron disease in the Province of Turin, Italy, 1971-1980

The incidence and prevalence of motor neuron disease (MND) in the Province of Turin, North-West Italy, were investigated for the period 1971-1980. The crude incidence rate of MND was 0.67/100,000/year. The annual incidence rate, age and sex adjusted to the Italian population in 1971 was 0.69 cases per 100,000 inhabitants, 0.94 for men and 0.45 for women, with a male to female incidence ratio of 2.09:1. The prevalence of MND was 2.62/100,000, 3.57 for males and 1.71 for females. The mean age at the time of diagnosis was 55.6 years. Annual incidence rates increased with advancing age. Amyotrophic lateral sclerosis was found to be 4 times more frequent than progressive muscular atrophy (0.53/100,000/year v. 0.14/100,000/year). The distribution of MND was uneven in the Province suggesting a proportional relationship to the distribution of population density. Possible explanations of this finding are discussed.     

Moroccan national study on prevalence of mental disorders: a community‐based epidemiological study

Objective: The main objective of this study was to assess the prevalence of common mental disorders in the Moroccan general population. Method: On a systematic representative randomized sample, the Moroccan Arabic version of the Mini International Neuropsychiatric Interview (MINI) was used to assess the prevalence of mood, anxiety, substance, and alcohol abuse disorders. Results: Among 5498 subjects interviewed, 40.1% had at least one current mental disorder. Current major depressive disorder was the most common (26.5%), and at least one anxiety disorder was found in 37% of the sample. Mental disorders were more frequent among female, urban, divorced, and unemployed subjects. Conclusion: Mental disorders are common in the Moroccan general population, particularly mood and anxiety disorders.     

Epidemiological and clinical characteristics of myasthenia gravis in Belgrade, Yugoslavia (1983–1992)

This is the first epidemiological study of myasthenia gravis (MG) in the area of Belgrade. During the survey period (1983 1992), 124 incidental cases of MG were observed, producing an average annual incidence rate of 7.1 per million population (women, 8.3; men, 5.8). Age and sex specific incidence rates for females demonstrated a bimodal pattern, with the first peak in the age group between 20 and 40, and the second peak in the age group 70-80. The age-specific rates for males showed unimodal pattern, reaching a maximum in the age group between 60 and 80. There was a tendency of more frequent disease appearance in the urban as opposed to the suburban districts. On the prevalence day, December 31, 1992, the point prevalence rate was 121.5 per million (women, 142.5; men, 98.8). Only for incidental cases, the point prevalence rate was 77.1 (women, 83.2; men, 70.4). The average annual mortality rate was 0.47 per million (females, 0.52; males, 0.42), while cumulative lethality was 5.6 (women, 5.6; men, 5.7). Most frequently initial symptoms were ocular, occurring in 58% patients. Through the period of investigation ocular symptoms were generalized in 68%, most frequently in the first 2 years (62.5%). Thymoma was confirmed in 11.3% of patients. In this group there was equal presence of both sexes, older median age at onset, and more severe clinical course of MG. Associated autoimmune disease was found in 17 out of 124 incidental cases (13.7%). The most common were thyroid diseases (7.3%). Family history of MG was recorded in 2 cases belonging to 1 family (1.6%).     

The incidence and prevalence of Huntington's disease: A systematic review and meta‐analysis

Huntington's disease (HD) is a rare, neurodegenerative disorder characterized by chorea, behavioral manifestations, and dementia. The aim of this study was to estimate the incidence and prevalence of HD through a systematic review of the literature. Medline and Embase databases were searched using terms specific to HD as well as studies of incidence, prevalence, and epidemiology. All studies reporting the incidence and/or prevalence of HD were included. Twenty original research articles were included. Eight studies examined incidence, and 17 studies examined prevalence. Meta‐analysis of data from four incidence studies revealed an incidence of 0.38 per 100,000 per year (95% confidence interval [CI]: 0.16, 0.94). Lower incidence was reported in the Asian studies (n = 2), compared to the studies performed in Europe, North America, and Australia (n = 6). The worldwide service‐based prevalence of HD, based on a meta‐analysis (n = 13 studies), was 2.71 per 100,000 (95% CI: 1.55–4.72). Eleven studies were conducted in Europe, North American, and Australia, with an overall prevalence of 5.70 per 100,000 (95% CI: 4.42–7.35). Three studies were conducted in Asia, with an overall prevalence of 0.40 per 100,000 (95% CI: 0.26–0.61). Metaregression revealed a significantly lower prevalence of HD in Asia, compared to European, North American, and Australian populations. HD is a devastating neurodegenerative disorder with a higher prevalence in Europe, North America, and Australia than in Asia. The difference in prevalence of this genetic disorder can be largely explained by huntingtin gene haplotypes. © 2012 Movement Disorder Society     

Cholesterol as a risk factor for Alzheimer's disease – epidemiological evidence

Although dementia is usually a late-life syndrome, it is now well known that pathological changes begin quite early in adulthood, outside the classical age borders of geriatric specialties. In order to design effective preventive strategies, adequate information can only be gathered by taking a life-long view of Alzheimer's disease (AD). Dementia risk is the result of exposure to both harmful and protective factors along the life course, and these factors, as well as their impact on the individual's health status, change over time. This review aims at presenting current epidemiological data on serum cholesterol levels and dietary fat intake as risk factors for dementia/AD, and at discussing the reasons and significance of contradictions between various studies. Reducing dementia risk may be possible by influencing the serum lipid profile. A more detailed characterization of the mechanisms behind the association of cholesterol (in both serum and brain) with dementia/AD, mechanisms about which little is currently known, would allow a better translation of research findings into clinical practice.     

Epidemiology of multiple sclerosis: incidence and prevalence rates in Denmark 1948–64 based on the Danish Multiple Sclerosis Registry

The Danish Multiple Sclerosis Registry (DMSR) is a national register based upon the ethnically homogeneous Danish population of about 5 millions. The DMSR was founded in 1956 following a nationwide Danish prevalence survey of MS in 1949 and a continuous registration of incident cases of MS since January 1, 1948. Included in DMSR are all Danish cases of MS (or suspicion of MS) diagnosed by a neurologist or a department of neurology. The sources of notification are the 22 neurological departments in Denmark, the National Patient Registry, the neuropathological departments, the Registry of Causes of Death, and, up to 1975, the Disablement Insurance Court. Notified cases which do not comply with the standardized diagnostic criteria of the DMSR are excluded. An estimate of the completeness of the DMSR is 90-95% and the validity is around 94%. Age- and sex-specific incidence rates of MS for the interval 1948-64 are presented. The crude annual incidence rate of MS in Denmark was in the year 1948-64 4.42 per 100,000 population, 22% higher in females than males. There was a significant geographical variation of incidence rates and a significant downward trend in incidence rates during the interval, whereas the prevalence rates showed a slight increase.     

Mortality risk in adults with newly diagnosed and chronic epilepsy: a population‐based study

Background: We analyzed mortality in adult patients with newly diagnosed and chronic epilepsy over a 13‐year period. Methods: Eighty‐one patients aged ≥20 years with newly diagnosed epilepsy and 309 adult patients with chronic epilepsy were originally identified from population‐based incidence and prevalence studies conducted in Tartu between 1994 and 1996. Patients with epilepsy were followed until the date of death or until the end of 2007. The standardized mortality ratio (SMR) was analyzed for both cohorts. The influences of age at diagnosis, sex, epilepsy syndrome, seizure type, risk factors and treatment compliance on the SMR were also investigated. Results: The SMR was significantly increased in both cohorts, but was higher in patients with chronic epilepsy (SMR 3.1; 95% confidence interval [CI] 2.5–3.8) relative to patients with newly diagnosed epilepsy (SMR 2.6; 95% CI 1.8–3.5). In the newly diagnosed epilepsy cohort, the increased mortality risk was more pronounced in patients with complex partial seizures (SMR 5.6; 95% CI 2.4–11.0). In the chronic epilepsy cohort, the mortality risk was higher in patients with secondary generalized tonic‐clonic seizures (SMR 3.4; 95% CI 2.5–4.5). Non‐compliant patients had twice the mortality risk (SMR 4.2; CI 95% 2.7–6.2) compared to those who were on anticonvulsant treatment. Conclusions: Mortality rates are higher in people with newly diagnosed and chronic epilepsy. Mortality risks should be discussed with patients with epilepsy, especially if anticonvulsant treatment is refused despite recurrent seizures.     

Population‐based epidemiologic study of Wilson’s disease in Taiwan

Background: The purpose of the study is to investigate the epidemiology and medical expenses of Wilson’s disease in Taiwan. Methods: Cases of Wilson’s disease were identified from the National Health Insurance Research Database with corresponding International Classification of Diseases, Ninth Revision (ICD‐9) code 275.1 from January 2000 to December 2005. Age and sex‐specific incidences were estimated by dividing the incidence number by population data obtained from the Department of Statistics, Ministry of the Interior. Results: During the study period, 495 cases of Wilson’s disease were identified. The male to female ratio was 1.28. The average annual incidence rate was 0.27 per 100 000, and the incidence rate peaked in the age group of 15–19 years. Most cases were juvenile and young adults. The prevalence increased steadily during the study period. Cirrhosis of the liver was the most common co‐existing condition. Conclusions: This is the first population‐based epidemiologic study of Wilson’s disease in Taiwan. Because of the effective and affordable treatment, the prevalence of Wilson’s disease in Taiwan is expected to increase continuously.     

Amyotrophic lateral sclerosis in Middle-Finland: an epidemiological study

ABSTRACT- Amyotrophic lateral sclerosis (ALS) was diagnosed in 36 patients in Middle-Finland Central Hospital District during 1976–1981. The annual incidence of ALS was 2.4 per 100,000 population and the prevalence rate was 6.4 per 100,000 population. The age-specific incidences of ALS were similar for men and women with a maximum of 14/100,000/year in the age group 60–69 years. The initial symptoms originated in 37% of the patients from bulbar and in 63% from spinal levels. Bulbar onset was more common in patients aged 60 years or more compared with younger patients. Patients with bulbar onset had a significantly poorer prognosis than those with spinal onset, which explained the poorer prognosis of older patients.
4 matched controls were chosen for each ALS patient from the files of the Central Hospital. There was no difference between the patients and the controls with respect to previous injuries, surgical operations, malignant neoplasms, or exposure to domestic animals. An earlier observation that evacuees from Karelia ceded to USSR after World War II should have a prevalence twice that of the remaining population was not substantiated.     

The epidemiology of epilepsy in Europe – a systematic review

Population-based epidemiological studies on epilepsy are available mainly from the UK and the Nordic, Baltic and western Mediterranean countries. No studies were identified from large areas of Europe, especially from the former eastern Europe (except the Baltic countries) and the eastern Mediterranean countries. Based on the prevalence of epilepsy in different studies and accounting for incomplete case identification the estimated number of children and adolescents in Europe with active epilepsy is 0.9 million (prevalence 4.5–5.0 per 1000), 1.9 million in ages 20–64 years (prevalence six per 1000) and 0.6 million in ages 65 years and older (prevalence seven per 1000). Approximately 20–30% of the epilepsy population have more than one seizure per month. Based on the age-specific incidence rates in European studies, the estimated number of new cases per year amongst European children and adolescents is 130 000 (incidence rate 70 per 100 000), 96 000 in adults 20–64 years (incidence rate 30 per 100 000) and 85 000 in the elderly 65 years and older (incidence 100 per 100 000). The proportion of both new and established cases with epilepsy in the young, adults and elderly in individual countries may differ substantially from total European distribution because of differences in age structure.