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Motor neuron disease in Cantabria.

J M López-Vega1, J Calleja, O Combarros

  • 1Department of Medicine (Section of Neurology), National Hospital Marqués de Valdecilla, Faculty of Medicine, Santander, Spain.

Acta Neurologica Scandinavica
|January 1, 1988
PubMed
Summary

Motor neuron disease (MND), including amyotrophic lateral sclerosis (ALS), affects 1.01 per 100,000 annually. Incidence rises with age, with a median onset of 60.5 years and a 1.78:1 male-to-female ratio.

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Area of Science:

  • Neurology
  • Epidemiology

Background:

  • Motor neuron disease (MND) encompasses amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), and progressive muscular atrophy (PMA).
  • Understanding the incidence and prevalence of MND is crucial for public health planning and resource allocation.

Purpose of the Study:

  • To determine the incidence and prevalence of MND in a defined region of northern Spain.
  • To characterize the demographic, clinical, and survival patterns of MND patients.

Main Methods:

  • A retrospective study of 62 patients diagnosed with MND between 1974 and 1985 in Cantabria, Spain.
  • Calculation of annual incidence and prevalence rates per 100,000 inhabitants.
  • Analysis of age-specific incidence, male-to-female ratio, age at onset, diagnostic interval, disease subtypes, duration, and survival rates.

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Main Results:

  • The annual incidence of MND was 1.01 per 100,000, with a prevalence of 3.52 per 100,000.
  • The male-to-female ratio was 1.78:1, with peak incidence in males aged 60-69 and females over 70.
  • Median age at onset was 60.5 years; ALS constituted 53% of cases. Mean disease duration was 26.6 months, with 5-year and 10-year survival rates of 18% and 6%, respectively.

Conclusions:

  • MND exhibits a notable incidence and prevalence in the studied population, with distinct age and sex patterns.
  • Survival rates remain low, highlighting the aggressive nature of the disease and the need for further research and improved management strategies.