Motor neuron disease in the province of Turin, Italy, 1966-1980. Survival analysis in an unselected population

P. Mortara, A. Chiò, M. G. Rosso, M. Leone, D. Schiffer

Research output: Contribution to journalArticlepeer-review

Abstract

We performed a population based survival analysis of all incident cases (220) of motor neuron disease (MND) in the province of Turin, Italy, during the period 1966-1980. 175 cases were diagnosed as amyotrophic lateral sclerosis (ALS), 43 as progressive muscular atrophy (PMA) and 2 as progressive bulbar palsy (PBP). The life-tables of MND, adjusted as to the "expected" mortality, showed a survival rate of 27.8% and 22.0% at 5 and 10 years, respectively. The course of PMA and ALS cases was different, with a 5-year survival rate of 66.8% and 17.7%, respectively. Nevertheless both life-tables showed a similar pattern with a rapidly fatal outcome in the first 3 years after diagnosis and a slower death rate in the following years. In each curve, the slopes suggested the presence of 2 subgroups with different prognosis. It is to be stressed that a percentage of PMA patients (25.7%) showed a rapidly fatal outcome and that a subgroup of ALS patients (26.6%) showed a relatively benign course. This might suggest a different individual susceptibility to the disease.

Original languageEnglish
Pages (from-to)165-173
Number of pages9
JournalJournal of the Neurological Sciences
Volume66
Issue number2-3
DOIs
Publication statusPublished - 1984

Keywords

  • Amyotrophic lateral sclerosis
  • Motor neuron disease
  • Prognosis
  • Progressive muscular atrophy

ASJC Scopus subject areas

  • Ageing
  • Clinical Neurology
  • Surgery
  • Neuroscience(all)
  • Developmental Neuroscience
  • Neurology

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