title: Relevance of the pyramidal syndrome in amyotrophic lateral sclerosis.
abstract: BACKGROUND
  Pyramidal signs (hyperreflexia, spasticity, Babinski sign) are essential for the diagnosis of amyotrophic lateral sclerosis (ALS). However, these signs are not always present at onset and may vary over time, besides which their role in disease evolution is controversial. Our goal was to describe which pyramidal signs were present and how they evolved in a cohort of patients with ALS, as well as their role in prognosis.
  
  
  METHODS
  Retrospective analysis of prospectively collected patients diagnosed with ALS in our centre from 1990 to 2015.
  
  
  RESULTS
  Of a total of 130 patients with ALS, 34 (26.1%) patients showed no pyramidal signs at the first visit while 15 (11.5%) had a complete pyramidal syndrome. Of those patients without initial pyramidal signs, mean time of appearance of the first signs was 4.5 months. Babinski sign was positive in 64 (49.2%) patients, hyperreflexia in 90 (69.2%) and 22 (16.9%) patients had spasticity. Pyramidal signs tended to remain unchanged over time, although they seem to appear at later stages or even disappear with time in some patients. We found no association between survival and the presence of changes to pyramidal signs, although decreased spasticity was associated with greater clinical deterioration (ALSFR scale) (P<.001).
  
  
  CONCLUSIONS
  A quarter of patients with ALS initially showed no pyramidal signs and in some cases they even disappear over time. These data support the need for tools that assess the pyramidal tract.
authors.affiliation: Sección de Neurofisiología Clínica, Unidad de Enfermedades Neuromusculares, Servicio de Neurología, Hospital del Mar, Barcelona, España.
authors.forename: N
authors.initials: N
authors.lastname: Álvarez
authors.affiliation: Unidad de Enfermedades Neuromusculares, Servicio de Neurología, Hospital del Mar, Barcelona, España.
authors.forename: L
authors.initials: L
authors.lastname: Díez
authors.affiliation: Servicio de Neurología, Hospital del Mar, Barcelona, España.
authors.forename: C
authors.initials: C
authors.lastname: Avellaneda
authors.affiliation: Servicio de Neurología, Hospital del Mar, Barcelona, España.
authors.forename: M
authors.initials: M
authors.lastname: Serra
authors.affiliation: Unidad de Enfermedades Neuromusculares, Servicio de Neurología, Hospital del Mar, Barcelona, España; Instituto Hospital del Mar de Investivaciónes Médicas (IMIM), Barcelona, España. Electronic address: MARubio@parcdesalutmar.cat.
authors.forename: M Á
authors.initials: MÁ
authors.lastname: Rubio
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis*; D005260:Female; D006801:Humans; D008297:Male; D009128:Muscle Spasticity / Q000209:etiology*; D011379:Prognosis; D012021:Reflex, Abnormal / Q000502:physiology*; D001405:Reflex, Babinski / Q000502:physiology*; D012189:Retrospective Studies; D013030:Spain
keywords: Amyotrophic lateral sclerosis; Babinski; Babinski sign; Esclerosis lateral amiotrófica; Espasticidad; Hiperreflexia; Hipertonía; Hyperreflexia; Hypertonia; Pyramidal signs; Signos piramidales; Spasticity