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Letter
Time to generalisation as a predictor of prognosis in amyotrophic lateral sclerosis
  1. Rosanna Tortelli1,2,
  2. Massimiliano Copetti3,
  3. Francesco Panza1,2,4,
  4. Rosa Cortese2,
  5. Rosa Capozzo1,
  6. Eustachio D'Errico2,
  7. Andrea Fontana3,
  8. Isabella Laura Simone2,
  9. Giancarlo Logroscino1,2
  1. 1 Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari Aldo Moro, at Pia Fondazione “Card. G. Panico”, Tricase, Lecce, Italy
  2. 2 Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari Aldo Moro, Bari, Italy
  3. 3 Unit of Biostatistics, IRCCS “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, Foggia, Italy
  4. 4 Geriatric Unit & Laboratory of Gerontology and Geriatrics, Department of Medical Sciences, IRCCS “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, Foggia, Italy
  1. Correspondence to Dr Giancarlo Logroscino, Department of Basic Medical Sciences, Neurosciences, and Sense Organs, University of Bari Aldo Moro, Bari, Italy; and Department of Clinical Research in Neurology, University of Bari Aldo Moro, “Pia Fondazione Cardinale G Panico”, Tricase, Lecce, Italy, 73039; giancarlo.logroscino{at}uniba.it

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Introduction

In amyotrophic lateral sclerosis (ALS), prognosis is usually based on death or tracheostomy as outcomes. Nevertheless, the early course is known to be a reliable indicator of the whole course of the disease.1 The identification of outcomes in early stages would determine better planning of interventions and more appropriate stratification of newly diagnosed patients for randomised clinical trials (RCTs). The time of disease spread is probably linked to the rate of progression and the intensity of neuro-axonal degeneration,2 and it has already been related to survival in ALS.3 The aim of the present study was to evaluate if time to generalisation (TTG) may predict survival in ALS.

Methods

All patients with a diagnosis of possible, probable or definite ALS, according to El Escorial criteria,4 and an onset of the disease between January 2004 and December 2007, referred to the Centre of Motor Neuron Disease, University of Bari, were enrolled in the current study. The study was terminated in April 2012. Death or tracheostomy was used as outcome measure to study the 4-year and 5-year mortality. Considered clinical variables were: (1) age at symptom onset (AAO); (2) site of symptom onset (bulbar region or spinal region); (3) time of diagnosis; (4) onset-diagnosis interval (ODI), defined as the time from the onset of the first symptom to diagnosis of ALS; (5) score of the revised ALS Functional Rating Scale (ALSFRS-r) at baseline;5 (6) TTG, considered as the time of spreading of the clinical signs from spinal (cervical, thoracic, or lumbosacral regions), or bulbar localisation or both. To detect generalisation, all the lower and upper motor neuron signs listed in the El Escorial criteria for the four body regions were investigated;4 TTG was censored at the time of the last follow-up visit; (7) overall survival (OS), defined as …

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