Amyotrophic lateral sclerosis in Slovenia – analysis of patient population at the Ljubljana Institute of Clinical Neurophysiology
Abstract
Backgorund: Data on epidemiology and disease characteristics of amyotrophic lateral sclerosis (ALS) are geographically limited and no systematically collected data exist for slovenian patients. We performed a retrospective descriptive study on clinical attributes and disease course of patients with ALS treated at the Institute of clinical neurophysiology (ICN), University Medical Centre Ljubljana since the foundation of a specialised ALS group.
Methods: All 271 patients treated at ICN in the 10-year period between 2003 and 2012 were analysed. Data on basic demographic characteristics, phenotype of disease onset, diagnostic delay, survival, family history, use of percutaneous gastrostomy (PEG), of non-invasive ventilation and of riluzole were obtained.
Results: Mean age at symptoms onset was 62.7 ± 11.4 years, median diagnostic delay 11 (IQ range 7–19) months and mean survival from time of enrolment 16.4 ± 15.1 months. 179 (66.1%)patients had spinal onset disease and 71 (26.2%) bulbar onset disease. Factors associated with longer survival were lower age at enrolment, longer diagnostic delay and use of PEG. The proportion of patients using non-invasive ventilatory support was rising through the analysed years.
Conclusions: Disease characteristics and survival in our series are similar to data from other tertiary care centres. The need for non-invasive ventilatory support in ALS patients is increasing.
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References
Kiernan M, Vucic S, Cheah B, Turner MR, Eisen A, Hardiman O, et al. Amyotrophic lateral sclerosis. Lancet. 2011; 377: 942-55.
Murray B, Mitsumoto H. Disorders of upper and lower motor neurons. In: Daroff B, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley's Neurology in Clinical Practice. Philadelphia:Elsevier Inc.; 2012. pp. 1855-89.
Spataro R, Lo Re M, Piccoli T, Piccoli F, La Bella V. Causes and place of death in Italian patients with amyotrophic lateral sclerosis. Acta Neurol Scand. 2010; 122: 217-23.
Cellura E, Spataro R, Taiello AC, La Bella V. Factors affecting the diagnostic delay in amyotrophic lateral sclerosis. Clin Neurol Neurosurg. 2012; 114: 550-4.
Czaplinski A, Yen AA, Simpson EP, Appel SH. Slower disease progression and prolonged survival in contemporary patients with amyotrophic lateral sclerosis: is the natural history of amyotrophic lateral sclerosis changing? Arch Neurol. 2006; 63: 1139-43.
Chio A, Logroscino G, Traynor BJ, Collins J, Simeone JC, Goldstein LA, et al. Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiol. 2013; 41: 118-30.
Traxinger K, Kelly C, Johnson BA, Lyles RH, Glass JD. Prognosis and epidemiology of amyotrophic lateral sclerosis. Neurol Clin Pract. 2013; 3: 313-20.
Kraemer M, Buerger M, Berlit P. Diagnostic problems and delay of diagnosis in amyotrophic lateral sclerosis. Clin Neurol Neurosurg. 2010; 112: 103-5.
Al-Chalabi A, Hardiman O. The epidemiology of ALS: a conspiracy of genes, environment and time. Nat Rev Neurol. 2013; 9: 617-28.
Logroscino G, Traynor BJ, Hardiman O, Chio A, Couratier P, Mitchell JD, et al. Descriptive epidemiology of amyorophic lateral sclerosis: new evidence and unsolved issues. J Neurol Neurosurg Psychiatry. 2009; 79: 6-11.
Nakamura R, Atsuta N, Watanabe H, Hirakawa A, Watanabe H, Ito M, et al. Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients. J Neurol Neurosurg Psychiatry. 2013; 84: 1365-71.
Roche JC, Rojas-Garcia R, Scott KM, Scotton W, Ellis CE, Burman R, et al. A proposed staging system for amyorophic lateral sclerosis. Brain. 2012; 135: 847-52.
Magnus T, Beck M, Giess R, Puls I, Naumann M, Toyka KV. Disease progression in amyotrophic lateral sclerosis: Predictors of survival. Muscle Nerve. 2002; 25: 709-14.
Ringholz G, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Shulz PE. Prevalence and patterns of cognitive impairment in sporadic amyotrophic lateral sclerosis. Neurology. 2005; 65: 586-90.
Štukovnik V, Zidar J, Repovš G. Kognitivna oškodovanost pri amiotrofični lateralni sklerozi – nevropsihološka perspektiva. 2013; 82: 755-66.
Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, et al. Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009; 73: 1227-33.
Chio A, Canosa A, Calvo A. Prospective epidemiological registers: a valuable tool for uncovering ALS pathogenesis. J Neurol Neurosurg Psychiatry. 2011; 82: 1066.
Cui F, Liu M, Chen Y, Huang X, Cui L, Fan D, et al. Epidemiological characteristics of motor neuron disease in Chinese patients. Acta Neurol Scand. 2014; 130: 111-7.
Gundersen MD, Yaseen R, Midgard R. Incidence and clinical features of amyotrophic lateral sclerosis in More and Romsdal County, Norway. Neuroepidemiology. 2011; 37: 58-63.
Piemonte and Valle d'Aosta Register for Amyotrophic lateral sclerosis (PARALS). Incidence of ALS in Italy: evidence for a uniform frequency in Western countries. Neurology. 2001; 56: 239-44.
Forbes RB, Colville S, Swingler RJ. The epidemiology of amyotrophic lateral sclerosis (ALS/MND) in people aged 80 or over. Age Ageing. 2004; 33: 131-4.
Statistični urad Republike Slovenije. Prebivalstvena piramida Slovenija 1971-2061. Available 23. 8. 2014 from: http://www.stat.si/Piramida2.asp.
Murphy M, Quinn S, Young J, Parkin P, Taylor B. Increasing incidence of ALS in Canterbury, New Zealand. Neurology. 2008; 71: 1889-95.
Mandrioli J, Faglioni P, Nichelli P, Sola P. Amyotrophic lateral sclerosis: prognostic indicators of survival. Amyotroph Lateral Scler. 2006; 7: 217-20.
Chiò A, Calvo A, Moglia C, Gamna F, Mattei A, Mazzini L, et al. Non-invasive ventilation in amyotrophic lateral sclerosis: a 10 year population based study. J Neurol Neurosurg Psychiatry. 2012; 83: 377-81.
Chiò A, Calvo A, Ghiglione P, Mazzini L, Mutani R, Mora G, et al. Tracheostomy in amyotrophic lateral sclerosis: a 10-year population-based study in Italy. J Neurol Neurosurg Psychiatry. 2010; 81: 1141-3.
Bourke SC, Bullock RE, Williams TL, Shaw PL, Gibson GJ. Noninvasive ventilation in ALS: indications and effect on quality of life. Neurology. 2003; 61: 171-7.
Leonardis L, Dolenc Goršelj L, Vidmar G. Factors related to respiration influencing survival and respiratory function in patients with amyotrophic lateral sclerosis: a retrospective study. Eur J Neurol. 2012; 19: 1518-24.
Byrne S, Walsh C, Lynch C, Bede P, Elamin M, Kenna K, et al. Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry. 2011; 82: 623-7.
Vrabec K, Glavač D, Rogelj B, Ravnik-Glavač M. Genetic analysis of amyotrophic lateral sclerosis in the Slovenian population. Eur J Hum Genet. 2014; 22 suppl 1: s165-6.
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