Research Article
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Year 2021, Volume: 4 Issue: 5, 576 - 581, 05.09.2021
https://doi.org/10.32322/jhsm.940104

Abstract

References

  • Charcot JMJ. A. Deux cas d’atrophie musculaire progressive avec lesions de la substance grise et des faisceaux antero-lateraux de la moelle epiniere [French]. Arch Physiol Neurol Pathol 1869; 2: 744.
  • Worms PM. The epidemiology of motor neuron diseases: a review of recent studies. J Neurol Sci 2001; 191: 3.
  • Byrne S, Walsh C, Lynch C, et al. Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry 2011; 82: 623-7.
  • Taylor JP, Brown RH Jr, Cleveland DW. Decoding ALS: from genes to mechanism. Nature 2016; 539: 197.
  • 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: official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases 2000; 1: 293-9.
  • Manjaly ZR, Scott KM, Abhinav K, et al. The sex ratio in amyotrophic lateral sclerosis: A population based study. Amyotroph Lateral Scler 2010; 11: 439-42.
  • Luna J, Diagana M, Ait Aissa L, et al. Clinical features and prognosis of amyotrophic lateral sclerosis in Africa: the TROPALS study. J Neurol Neurosurg Psychiatry 2019; 90: 20-9.
  • Turgut N, Varol SaraÇoglu G, Kat S, et al. An epidemiologic investigation of amyotrophic lateral sclerosis in Thrace, Turkey, 2006-2010. Amyotroph Lateral Scler Frontotemporal Degener 2019; 20: 100-6.
  • Uysal H, Taghiyeva P, Türkay M, Köse F, Aktekin M. Amyotrophic lateral sclerosis in Antalya, Turkey. A prospective study, 2016-2018 [published online ahead of print, 2020. Amyotroph Lateral Scler Frontotemporal Degener 2020; 1-7.
  • Brylev L, Ataulina A, Fominykh V, et al. The epidemiology of amyotrophic lateral sclerosis in Moscow (Russia). Amyotroph Lateral Scler Frontotemporal Degener 2020; 21: 410-5.
  • Argyriou AA, Polychronopoulos P, Papapetropoulos S, et al. Clinical and epidemiological features of motor neuron disease in south-western Greece. Acta Neurol Scand 2005; 111: 108-13.
  • Li TM, Alberman E, Swash M. Comparison of sporadic and familial disease amongst 580 cases of motor neuron disease. J Neurol Neurosurg Psychiatry 1988; 51: 778-84.
  • Camu W, Khoris J, Moulard B, et al. Genetics of familial ALS and consequences for diagnosis. French ALS Research Group. J Neurol Sci 1999; 165 Suppl 1: 21-6.
  • Chiò A, Logroscino G, Traynor BJ, et al. Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology 2013; 41: 118-30.
  • Longinetti E, Wallin AR, Samuelson K, et al. The Swedish motor neuron disease quality registry. Amyotroph Lateral Scler Frontotemporal Degener 2018; 19: 528-37.
  • Leighton DJ, Newton J, Stephenson LJ, et al. Changing epidemiology of motor neurone diseases in Scotland. J Neurol 2019; 266: 817-25.
  • Aktekin MR, Uysal H. Epidemiology of Amyotrophic Lateral Sclerosis. Turk J Neurol 2020; 26: 187-96
  • Haverkamp LJ, Appel V, Appel SH. Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain 1995; 118: 707-19.
  • 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.
  • Li TM, Alberman E, Swash M. Clinical features and associations of 560 cases of motor neuron disease. J Neurol Neurosurg Psychiatry 1990; 53: 1043-5.
  • Beghi E, Millul A, Micheli A, Vitelli E, Logroscino G; SLALOM Group. Incidence of ALS in Lombardy, Italy. Neurology 2007; 68: 141-5.
  • Ragonese P, Cellura E, Aridon P, et al. Incidence of amyotrophic lateral sclerosis in Sicily: A population based study. Amyotroph Lateral Scler 2012; 13: 284-7.
  • .Chen L, Zhang B, Chen R, et al. Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China. J Neurol Neurosurg Psychiatry 2015; 86: 1075-81.
  • Jun KY, Park J, Oh KW, et al. Epidemiology of ALS in Korea using nationwide big data. J Neurol Neurosurg Psychiatry 2019; 90: 395-403.
  • Lee CT, Chiu YW, Wang KC, et al. Riluzole and prognostic factors in amyotrophic lateral sclerosis long-term and short-term survival: a population-based study of 1149 cases in Taiwan. J Epidemiol 2013; 23: 35-40.
  • Dorst J, Chen L, Rosenbohm A, et al. Prognostic factors in ALS: a comparison between Germany and China. J Neurol 2019; 266: 1516-25.
  • Kacem I, Sghaier I, Bougatef S, et al. Epidemiological and clinical features of amyotrophic lateral sclerosis in a Tunisian cohort. Amyotroph Lateral Scler Frontotemporal Degener 2020; 21: 131-9.
  • Marin B, Logroscino G, Boumediene F, et al. Clinical and demographic factors and outcome of amyotrophic lateral sclerosis in relation to population ancestral origin. Eur J Epidemiol 2016; 31: 229-45.
  • Louwerse ES, Visser CE, Bossuyt PM, Weverling GJ. Amyotrophic lateral sclerosis: mortality risk during the course of the disease and prognostic factors. The Netherlands ALS Consortium. J Neurol Sci 1997; 152 Suppl 1:10-7.
  • Chiò A, Logroscino G, Hardiman O, et al. Prognostic factors in ALS: A critical review. Amyotroph Lateral Scler 2009; 10: 310-23.
  • Stevic Z, Kostic-Dedic S, Peric S, et al. Prognostic factors and survival of ALS patients from Belgrade, Serbia. Amyotroph Lateral Scler Frontotemporal Degener 2016; 17: 508-14.
  • Corcia P, Pradat PF, Salachas F, et al. Causes of death in a post-mortem series of ALS patients. Amyotroph Lateral Scler 2008; 9: 59-62.

Clinical and epidemiological features of amyotrophic lateral sclerosis in eastern Turkey

Year 2021, Volume: 4 Issue: 5, 576 - 581, 05.09.2021
https://doi.org/10.32322/jhsm.940104

Abstract

Aim: The number of studies on the epidemiological and clinical data of amyotrophic lateral sclerosis (ALS) patients in Turkey is quite low and the studies on this subject reflect the data of western regions of Turkey. In this study, we aimed to present the demographic, clinical and mortality features of ALS patients diagnosed in the last 10 years in a large reference hospital in the Eastern Anatolia region of Turkey.
Material and Method: 42 ALS patients diagnosed between January 2011 and January 2021 in ………., Faculty of Medicine, Department of Neurology were included in the study. The data of the patients were obtained by retrospectively scanning the patient electronic files registered in the database of our hospital. The age, gender, examination findings, clinical course, treatments they received and the cause of death of the patients were recorded.
Results:26 of ALS patients were men and 16 were women. The mean age of onset was 53.4 ± 12.3 and the mean diagnostic delay was 13.6 ± 6.9 months. 64.2% of the patients had onset in the spinal region, and 35.7% in the bulbar region. Weakness/atrophy of the upper extremity muscles and swallowing difficulty were the most common onset symptoms. All ALS patients were using riluzole. 11 ALS patients died. The mean time between diagnosis and death in patients who died was 27.2 ± 18.6 months. The most common causes of death in ALS patients were pneumonia and sepsis.
Conclusion: Age of onset of ALS was low in our patients. The number of patients in the clinically definite ALS group was the highest. Weakness/atrophy of the upper extremity muscles and swallowing difficulty were the most common onset symptoms. Approximately 2/3 of the patients had spinal, and 1/3 bulbar region onset. There was no significant difference between spinal and bulbar onset patients in terms of gender, age of disease onset, mortality, and life expectancy of patients with death. The rate of using riluzole was high. The most common causes of death in ALS patients were pneumonia and sepsis.

References

  • Charcot JMJ. A. Deux cas d’atrophie musculaire progressive avec lesions de la substance grise et des faisceaux antero-lateraux de la moelle epiniere [French]. Arch Physiol Neurol Pathol 1869; 2: 744.
  • Worms PM. The epidemiology of motor neuron diseases: a review of recent studies. J Neurol Sci 2001; 191: 3.
  • Byrne S, Walsh C, Lynch C, et al. Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry 2011; 82: 623-7.
  • Taylor JP, Brown RH Jr, Cleveland DW. Decoding ALS: from genes to mechanism. Nature 2016; 539: 197.
  • 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: official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases 2000; 1: 293-9.
  • Manjaly ZR, Scott KM, Abhinav K, et al. The sex ratio in amyotrophic lateral sclerosis: A population based study. Amyotroph Lateral Scler 2010; 11: 439-42.
  • Luna J, Diagana M, Ait Aissa L, et al. Clinical features and prognosis of amyotrophic lateral sclerosis in Africa: the TROPALS study. J Neurol Neurosurg Psychiatry 2019; 90: 20-9.
  • Turgut N, Varol SaraÇoglu G, Kat S, et al. An epidemiologic investigation of amyotrophic lateral sclerosis in Thrace, Turkey, 2006-2010. Amyotroph Lateral Scler Frontotemporal Degener 2019; 20: 100-6.
  • Uysal H, Taghiyeva P, Türkay M, Köse F, Aktekin M. Amyotrophic lateral sclerosis in Antalya, Turkey. A prospective study, 2016-2018 [published online ahead of print, 2020. Amyotroph Lateral Scler Frontotemporal Degener 2020; 1-7.
  • Brylev L, Ataulina A, Fominykh V, et al. The epidemiology of amyotrophic lateral sclerosis in Moscow (Russia). Amyotroph Lateral Scler Frontotemporal Degener 2020; 21: 410-5.
  • Argyriou AA, Polychronopoulos P, Papapetropoulos S, et al. Clinical and epidemiological features of motor neuron disease in south-western Greece. Acta Neurol Scand 2005; 111: 108-13.
  • Li TM, Alberman E, Swash M. Comparison of sporadic and familial disease amongst 580 cases of motor neuron disease. J Neurol Neurosurg Psychiatry 1988; 51: 778-84.
  • Camu W, Khoris J, Moulard B, et al. Genetics of familial ALS and consequences for diagnosis. French ALS Research Group. J Neurol Sci 1999; 165 Suppl 1: 21-6.
  • Chiò A, Logroscino G, Traynor BJ, et al. Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology 2013; 41: 118-30.
  • Longinetti E, Wallin AR, Samuelson K, et al. The Swedish motor neuron disease quality registry. Amyotroph Lateral Scler Frontotemporal Degener 2018; 19: 528-37.
  • Leighton DJ, Newton J, Stephenson LJ, et al. Changing epidemiology of motor neurone diseases in Scotland. J Neurol 2019; 266: 817-25.
  • Aktekin MR, Uysal H. Epidemiology of Amyotrophic Lateral Sclerosis. Turk J Neurol 2020; 26: 187-96
  • Haverkamp LJ, Appel V, Appel SH. Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain 1995; 118: 707-19.
  • 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.
  • Li TM, Alberman E, Swash M. Clinical features and associations of 560 cases of motor neuron disease. J Neurol Neurosurg Psychiatry 1990; 53: 1043-5.
  • Beghi E, Millul A, Micheli A, Vitelli E, Logroscino G; SLALOM Group. Incidence of ALS in Lombardy, Italy. Neurology 2007; 68: 141-5.
  • Ragonese P, Cellura E, Aridon P, et al. Incidence of amyotrophic lateral sclerosis in Sicily: A population based study. Amyotroph Lateral Scler 2012; 13: 284-7.
  • .Chen L, Zhang B, Chen R, et al. Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China. J Neurol Neurosurg Psychiatry 2015; 86: 1075-81.
  • Jun KY, Park J, Oh KW, et al. Epidemiology of ALS in Korea using nationwide big data. J Neurol Neurosurg Psychiatry 2019; 90: 395-403.
  • Lee CT, Chiu YW, Wang KC, et al. Riluzole and prognostic factors in amyotrophic lateral sclerosis long-term and short-term survival: a population-based study of 1149 cases in Taiwan. J Epidemiol 2013; 23: 35-40.
  • Dorst J, Chen L, Rosenbohm A, et al. Prognostic factors in ALS: a comparison between Germany and China. J Neurol 2019; 266: 1516-25.
  • Kacem I, Sghaier I, Bougatef S, et al. Epidemiological and clinical features of amyotrophic lateral sclerosis in a Tunisian cohort. Amyotroph Lateral Scler Frontotemporal Degener 2020; 21: 131-9.
  • Marin B, Logroscino G, Boumediene F, et al. Clinical and demographic factors and outcome of amyotrophic lateral sclerosis in relation to population ancestral origin. Eur J Epidemiol 2016; 31: 229-45.
  • Louwerse ES, Visser CE, Bossuyt PM, Weverling GJ. Amyotrophic lateral sclerosis: mortality risk during the course of the disease and prognostic factors. The Netherlands ALS Consortium. J Neurol Sci 1997; 152 Suppl 1:10-7.
  • Chiò A, Logroscino G, Hardiman O, et al. Prognostic factors in ALS: A critical review. Amyotroph Lateral Scler 2009; 10: 310-23.
  • Stevic Z, Kostic-Dedic S, Peric S, et al. Prognostic factors and survival of ALS patients from Belgrade, Serbia. Amyotroph Lateral Scler Frontotemporal Degener 2016; 17: 508-14.
  • Corcia P, Pradat PF, Salachas F, et al. Causes of death in a post-mortem series of ALS patients. Amyotroph Lateral Scler 2008; 9: 59-62.
There are 32 citations in total.

Details

Primary Language English
Subjects Health Care Administration
Journal Section Original Article
Authors

Recep Yevgi 0000-0002-6586-2635

Nuray Bilge 0000-0002-9328-1678

Publication Date September 5, 2021
Published in Issue Year 2021 Volume: 4 Issue: 5

Cite

AMA Yevgi R, Bilge N. Clinical and epidemiological features of amyotrophic lateral sclerosis in eastern Turkey. J Health Sci Med / JHSM. September 2021;4(5):576-581. doi:10.32322/jhsm.940104

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