Clinical profile of motor neuron disease patients with lower urinary tract symptoms and neurogenic bladder

J Neurol Sci. 2017 Jul 15:378:130-136. doi: 10.1016/j.jns.2017.04.053. Epub 2017 May 2.

Abstract

Introduction: Lower urinary tract symptoms (LUTS) are frequent in motor neuron disease (MND) patients, but clinical factors related to them are unknown. We describe differences in LUTS among MND phenotypes and their relationship with other clinical characteristics, including prognosis.

Methods: For this study, we collected clinical data of a previously published cohort of patients diagnosed with classical amyotrophic lateral sclerosis (cALS), progressive muscular atrophy (PMA) or primary lateral sclerosis (PLS) with and without LUTS. Familial history was recorded and the C9ORF72 expansion was analysed in the entire cohort. Patients were followed-up for survival until August 2016.

Results: Fifty-five ALS patients (37 cALS, 10 PMA and 8 PLS) were recruited. Twenty-four reported LUTS and neurogenic bladder (NB) could be demonstrated in nine of them. LUTS were not influenced by age, phenotype, disability, cognitive or behavioural impairment, or disease progression, but female sex appeared to be a protective factor (OR=0.39, p=0.06). Neither family history nor the C9ORF72 expansion was linked to LUTS or NB. In the multivariate analysis, patients reporting LUTS early in the disease course tended to show poorer survival.

Conclusions: In this study, LUTS appear to be more frequent in male MND patients, but are not related to age, clinical or genetic characteristics. When reported early, LUTS could be a sign of rapid disease spread and poor prognosis. Further prospective longitudinal and neuroimaging studies are warranted to confirm this hypothesis.

Keywords: Amyotrophic lateral sclerosis; Lower urinary tract symptoms; Motor neuron disease; Neurogenic bladder; Primary lateral sclerosis; Progressive muscular atrophy; Urodynamics.

MeSH terms

  • Aged
  • Amyotrophic Lateral Sclerosis / complications
  • Amyotrophic Lateral Sclerosis / diagnosis
  • Amyotrophic Lateral Sclerosis / genetics
  • Amyotrophic Lateral Sclerosis / physiopathology*
  • C9orf72 Protein / genetics
  • Cross-Sectional Studies
  • Female
  • Follow-Up Studies
  • Humans
  • Lower Urinary Tract Symptoms / complications
  • Lower Urinary Tract Symptoms / diagnosis
  • Lower Urinary Tract Symptoms / genetics
  • Lower Urinary Tract Symptoms / physiopathology*
  • Male
  • Middle Aged
  • Motor Neuron Disease / complications
  • Motor Neuron Disease / diagnosis
  • Motor Neuron Disease / genetics
  • Motor Neuron Disease / physiopathology*
  • Multivariate Analysis
  • Muscular Atrophy, Spinal / complications
  • Muscular Atrophy, Spinal / diagnosis
  • Muscular Atrophy, Spinal / genetics
  • Muscular Atrophy, Spinal / physiopathology*
  • Phenotype
  • Prognosis
  • Sex Factors
  • Survival Analysis
  • Urinary Bladder, Neurogenic / complications
  • Urinary Bladder, Neurogenic / diagnosis
  • Urinary Bladder, Neurogenic / genetics
  • Urinary Bladder, Neurogenic / physiopathology*

Substances

  • C9orf72 Protein
  • C9orf72 protein, human