CLINICAL CHARACTERISTICS OF PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS IN THE REPUBLIC OF SRPSKA
Abstract
Introduction: Amyotrophic lateral sclerosis (ALS) is a rare, fatal and progressive neurodege-
nerative disease which begins due to selective damage to motor neurons. The disease begins
as spinal (spinal cervical, spinal lumbosacral and spinal thoracic form) and bulbar form. The
diagnosis is set by using revised El Escorial criteria.
Aim: Aims of the paper were determining demographic characteristics of ALS patients, ca-
tegory of the disease and functional disability of patients using Revised Amyotrophic Lateral
Sclerosis Functional Rating Scale (ALSFRS-R).
Material and methods: In this cross-sectional study, medical histories of ALS patients,
which were diagnosed at the Clinic of Neurology of the University Clinical Center of the
Republic of Srpska, were examined. Demographic characteristics, initial form of the
disease, category of disease (using revised El Escorial criteria), functional disability (using
ALSFRS-R) and dia-gnostic delay were analyzed.
Results: This study included 30 patients, 20 (67%) were male, 10 (33%) were female. The
ALS began at 57 ± 12 years, and average diagnostic delay was 10 months. The disease
started as a spinal form in 26 (87%) patients, out of which 13 (43%) had spinal cervical, 12
(40%) spinal lumbosacral and 1 (3%) spinal thoracic form of disease. The initial bulbar
form was reported in 4 (13%) patients. The average ALSFRS-R score was 33,5. Patients with
a spinal lumbosacral form of the disease had statistically significant lower score of
ALSFRS-R (p = 0.048) which indicates bigger functional motoric disablement, compared
to other forms of disease. Low values of ALSFRS-R are correlated with more accurate
diagnosis, according to the revised El Escorial criteria.
Conclusion: Results of this study showed that ALS is more frequent and occurs earlier
in men. The disease most commonly started as spinal cervical form. Patients with spinal
lumbo-sacral form of the disease had significantly lower values of ALSFRS-R which
indicates a faster progression of the disease. As patients acquire lower ALSFRS-R scores and
greater disability, they progress to the definite ALS category.
References
Kostić V. Neurologija za studente medicine. Beograd: Medicinski fakultet. 2016.
Rowland LP, Shneider NA: Amyotrophic lateral sclerosis. N Engl J Med 2001, 344(22):1688-1700.
Collins D, Goodfellow J, Silva D, Dardis R, Nagaraja S. Neurology and Neurosurgery. UK: JP Medical Ltd. 2016.
Sorenson EJ, Stalker AP, Kurland LT, Windebank AJ. Amyotrophic lateral sclerosis in Olmsted County, Minnesota, 1925 to 1998. Neurology 2002;59(2):280282. doi: 10.1212/wnl.59.2.280
Brooks BR, Miller RG, Swash M, Munsat TL. El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders 2000;1(5):293-299. doi: 10.1080/146608200300079536
Cedarbaum J, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B et al. The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. Journal of the Neurological Sciences. 1999;169(1-2):13-21.
Norris F, Shepherd R, Denys E, U K, Mukai E, Elias L, Norris H. Onset, natural history and outcome in idiopathic adult motor neuron disease. Journal of the Neurological Sciences 1993;118(1):48-55. doi: 10.1016/0022-510x(93)90245-t
Miller R, Mitchell J, Lyon M, Moore D. Riluzole for amyotrophic lateral sclerosis. Cochrane Database Syst Rev. 2007;24
Traynor BJ, Alexander M, Corr B, Frost E, Hardiman O. Effect of a multidisciplinary amyotrophic lateral sclerosis (ALS) clinic on ALS survival: a population based study, 1996-2000. Journal of Neurology, Neurosurgery and Psychiatry 2003;74(9):1258-1261.doi: 10.1136/jnnp.74.9.1258
Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Woolley SC. 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. Neurology 2009;73(15):1227-1233. doi: 10.1212/wnl.0b013e3181bc01a4
Makki AA, Benatar M. The electromyographic diagnosis of amyotrophic lateral sclerosis: Does the evidence support the El Escorial criteria. Muscle and Nerve 2007;35(5):614-619. doi: 10.1002/mus.20748
Traynor BJ, Codd MB, Corr B, Forde C, Frost E, Hardiman OM. Clinical features of amyotrophic lateral sclerosis according to the El Escorial and Airlie House diagnostic criteria: a population based study. Archives of Neurology 2000;57(8):1171. doi: 10.1001/archneur.57.8.1171
Huisman MHB, de Jong SW, van Doormaal PTC, Weinreich SS, Schelhaas HJ, van der Kooi AJ, van den Berg LH. Population based epidemiology of amyotrophic lateral sclerosis using capture-recapture methodology. Journal of Neurology, Neurosurgery and Psychiatry 2011;82(10):1165-1170. doi: 10.1136/jnnp.2011.244939
Kurtzke J. Epidemiology of amyotrophic lateral sclerosis. Adv Neurol 1982 36:281-302.
Logroscino G, Traynor BJ, Hardiman O, Chio A, Mitchell D, Swingler RJ, Beghi E. Incidence of amyotrophic lateral sclerosis in Europe. Journal of Neurology, Neurosurgery and Psychiatry 2010;81(4):385-390. doi: 10.1136/jnnp.2009.183525
Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano P, Chinea A. A comprehensive review of amyotrophic lateral sclerosis. Surgical Neurology International 2015;6(1):171. doi: 10.4103/2152-7806.169561
Oskarsson B, Horton DK, Mitsumoto H. Potential Environmental Factors in Amyotrophic Lateral Sclerosis. Neurologic Clinics 2015;33(4):877-888. doi: 10.1016/j.ncl.2015.07.009
Paganoni S, Macklin EA, Lee A, Murphy A, Chang J, Zipf A, Atassi N. Diagnostic timelines and delays in diagnosing amyotrophic lateral sclerosis (ALS). Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 2014;15(5-6):453-456. doi: 10.3109/21678421.2014.903974
Stevic Z, Kostic-Dedic S, Peric S, Dedic V, Basta I, Rakocevic-Stojanovic V, Lavrnic D. Prognostic factors and survival of ALS patients from Belgrade, Serbia. Amyotroph Lateral Scler Frontotemporal Degener. 2016;17(7-8):508-514.