Highlights
- •Lower urinary tract symptoms and neurogenic bladder are frequent in ALS patients.
- •Clinical characteristics of patients with and without lower urinary tract symptoms are similar.
- •Early urinary symptoms and neurogenic bladder appears to be a sign of poor prognosis.
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
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of the Neurological SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Controversies and priorities in amyotrophic lateral sclerosis.Lancet Neurol. Mar 2013; 12 (Elsevier Ltd): 310-322
- Upper motor neuron and extra-motor neuron involvement in amyotrophic lateral sclerosis: a clinical and brain imaging review.Neuromuscul. Disord. 2009; 19 (Elsevier B.V.): 53-58
- Stages of pTDP-43 pathology in amyotrophic lateral sclerosis.Ann. Neurol. Jul 2013; 74: 20-38
- Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment.Lancet Neurol. Apr 2013; 12: 368-380
- Cognitive changes predict functional decline in ALS: a population-based longitudinal study.Neurology. Apr 23, 2013; 80: 1590-1597
- ALS-Plus syndrome: non-pyramidal features in a large ALS cohort.J. Neurol. Sci. 2014; 345 (Elsevier B.V.): 118-124
- Sympathetic disturbances increase risk of sudden cardiac arrest in sporadic ALS.J. Neurol. Sci. 2007; 254: 78-83
- Decreased heart rate variability predicts death in amyotrophic lateral sclerosis.Muscle Nerve. 2012; 46: 341-345
- Neural control of the bladder: recent advances and neurologic implications.Neurology. 2010; 75: 1839-1846
- Lower urinary tract dysfunction in the neurological patient: clinical assessment and management.Lancet Neurol. 2015; 14 (Elsevier Ltd): 720-732
- Autonomic impairment in amyotrophic lateral sclerosis.Curr. Opin. Neurol. 2005; 18: 487-493
- EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)—revised report of an EFNS task force.Eur. J. Neurol. Mar 2012; 19 (cited 2013 Nov 14): 360-375
- TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord.Acta Neuropathol. Jun 12, 2014; 128: 423-437
- Increased prevalence of bladder and intestinal dysfunction in amyotrophic lateral sclerosis.Amyotroph. Lateral Scler. Frontotemporal Degener. 2014; 15: 174-179
- Urinary disorders in amyotrophic lateral sclerosis subjects.Amyotroph. Lateral Scler. Sep 2011; 12 (cited 2015 Jun 30): 352-355
- Urodynamic findings in amyotrophic lateral sclerosis patients with lower urinary tract symptoms: results from a pilot study.Neurourol. Urodyn. Feb 19, 2016; https://doi.org/10.1002/nau. 22976
- El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis.Amyotroph. Lateral Scler. Other Motor Neuron Disord. Dec 2000; 1: 293-299
- The history of progressive muscular atrophy: syndrome or disease?.Neurology. Feb 26, 2008; 70: 723-727
- The natural history of primary lateral sclerosis.Neurology. 14 Mar 2006; 66: 647-653
- The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III).J. Neurol. Sci. Oct 31, 1999; 169: 13-21
- Intrarater reliability of manual muscle test (Medical Research Council scale) grades in Duchenne's muscular dystrophy.Phys. Ther. Feb 1992; 72 (115-22-6)
- Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): revised diagnostic criteria.Amyotroph. Lateral Scler. Front Degener. 2017; (in press)
- Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS.Neuron. 2011; 72: 245-256
- Prognostic factors in ALS: a critical review.Amyotroph. Lateral Scler. 2009; 10: 310-323
- Cognitive dysfunction in lower motor neuron disease: executive and memory deficits in progressive muscular atrophy.J. Neurol. Neurosurg. Psychiatry. Feb 1, 2011; 82 (BMJ Publishing Group Ltd): 170-175
- Deciphering amyotrophic lateral sclerosis: what phenotype, neuropathology and genetics are telling us about pathogenesis.Amyotroph. Lateral Scler. Frontotemporal Degener. May 16, 2013; (Informa Healthcare Stockholm): 5-18
- Lower urinary tract dysfunction and neuropathological findings of the neural circuits controlling micturition in familial amyotrophic lateral sclerosis with L106V mutation in the SOD1 gene.Rinsho shinkeigaku. Mar 8, 2016; 56: 69-76
- Amyotrophic lateral sclerosis: moving towards a new classification system.Lancet Neurol. 2016; 15: 1182-1194
- Cognitive changes predict functional decline in ALS: a population-based longitudinal study.Neurology. 2013; 80: 1590-1597
- Predictors of impaired communication in amyotrophic lateral sclerosis patients with tracheostomy-invasive ventilation.Amyotroph. Lateral Scler. Front Degener. 2015; 8421: 1-9
- Genetic epidemiology of amyotrophic lateral sclerosis: a systematic review and meta-analysis.J. Neurol. Neurosurg. Psychiatry. Jan 5, 2017; (in press)
Article info
Publication history
Published online: May 02, 2017
Accepted:
April 30,
2017
Received in revised form:
April 28,
2017
Received:
December 10,
2016
Identification
Copyright
© 2017 Elsevier B.V. All rights reserved.