Clinical features of motor neuron disease patients treated at Unifesp outpatient’s clinic
DOI:
https://doi.org/10.34024/rnc.2008.v16.8631Keywords:
Motor Neuron Disease, Amyotrophic Lateral Sclerosis, EpidemiologyAbstract
Introduction. Motor neuron diseases are a group of fatal, progressive neurodegenerative disorders comprising: primary lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy, and amyotrophic lateral sclerosis (ALS). The most frequent early symptoms are muscle weakness, cramps, muscle atrophy, fasciculation and dysphasia. The earlier a diagnosis is obtained, the more effective a treatment or the better a management can be provided. Therefore, studies to determine the clinical profile of MND along with the determination of the time elapsed between the early symptoms and the first visit to a doctor can contribute to raise the level of awareness of such diseases. Method. Twenty patients starting their treatment at the UNIFESP outpatient’s clinic for neuromuscular diseases were subject of the present study. The statistical analysis of the data collected used the test for difference between two proportions and Spearman’s correlation test. Results. Among the 20 patients aged 63.30 ± 4.55, 60% presented with ALS. The mean time elapsed from the first symptom and the first examination at the UNIFESP clinic was 20.85 months, most patients were white with men/women ratio of 1:1, and 45% of the patients worked at jobs with physical demands. Most patients reported muscle weakness as the first symptom (45%). Conclusion. The clinical profile agrees with those reported in previous studies. The mean time elapsed between the occurrence of the first symptom and the first visit to a doctor was 20.85 ± 6.77 months.
Metrics
References
Michell JD, Borasio GD. Amyotrophic Lateral Sclerosis. Lancet 2007;369:2031-41.
Winhammar JMC, Rowe DB, Henderson RD, Kiernan MC. Assesment of Disease Progression in Motor Neuron Disease. Lancet Neurol 2005; 4:229-38.
Ferraz MEMR, Zanotelli E, Oliveira ASB, Gabbai AA. Atrofia Muscular Progressiva. Arq Neuropsiquiatr 2004;62(1): 119-26.
Oliveira ASB, Zanoteli E, Gabbai AA. Atualização Terapêutica 2005: manual prático de diagnóstico e tratamento. São Paulo: Arte Médicas, 2005, 975-85.
Rowland LP, Shneider MD. Amyotrophic Lateral Sclerosis. N Engl J Med 2001;344:1688-700.
Castro-Costa CM, Oriá RM, Do Vale OC, Arruda JAM, Horta WG, D’Almeida JAC, et al. Amyotrophic Lateral Sclerosis: Clinical Analysis Of 78 Cases from Fortaleza (Northeastern Brazil). Arq Neuropsiquiatr 2000;58(3-A):607-15.
Anne D, Walling MD. Amyotrophic Lateral Sclerosis: Lou Gehrig’s Disease. Am Fam Physician 1999;59(6):1489-96.
Voltarelli JC. Perspectivas de terapia celular na Esclerose Lateral amiotrófica. Ver Bras Hematol Hemoter 2004;26 (3):155-6.
Mandrioli J, Faglioni P, Merelli E, Sola P. The epidemiology of ALS in Modena, Italy. Neurology 2003;60:683-9.
Govoni V, Graniere E, Fallica E, Casetta I. Amyotrophic Lateral Sclerosis, rural environment and agricultural work in the Local Health District of Ferrara, Italy, in the years 1964-1998. J Neurol 2005;252:1322-7.
Drory VE, Artmonov I. Earlier onset and shorter survival of amyotrophic lateral sclerosis in Jewish patients of North African origin A clue to modifying genetic factors? J Neurol Sci 2007;258:39-43.
Leigh PN, Abrahams S, Al-Chalabi A. The management of motor neurone disease. J Neurol Neurosurg Psiquiatr 2003;74(Suppl IV):32-47.
Brooks BR, Sanjak M, Belden D, Juhasz-Poscine K, Waclawik A. Natural history of amyotrophic lateral sclerosis - impairment,disability, handicap. In: Brown RH, Meininger V, Swash M (eds.). Amyotroph Lateral Scler. London: Martin Dunitz, 2000, 31-58.
Czaplinski A, Yen AA, Appel SH. Amyotrophic Lateral Sclerosis: early predictors of prolonged survival. J Neurol 2006;253:1428-36.
Logroscino G, Traynor BJ, Hardiman O, Chio A, Couratier P, Mitchell JD, et al. Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues. J Neurol Noeurosur Psychiatr 2008;79:6-11.
Iwasaki Y, Ikeda K, Ichikawa Y, Igarashi O, Kinoshita M. The diagnostic interval in amyotrophic lateral sclerosis. Clin Neurol Neurosurg 2002;104:87-9.
Argyriou AA, Polychronopoulos P, Papapetropoulos S, Ellul J, Andriopoulos I, Katsoulas G, et al. Clinical and epidemiological features of motor neuron disease in south-western Greece. Acta Neurol Scand 2005;111:108-13.
Corrado L, Alfonso SD, Bergamaschi L, Testa L, Leone M, Nasuelli N, et al. SOD 1 gene mutation in Italian patients with Sporadic Amyotrophic Lateral sclerosis (ALS). Neuromuscul Disord 2006;16:800-4.
Shoesmith CL, Strong MJ. Update for family physicians amyotrophic lateral sclerosis.Can Fam Physician 2006;52 (12):1563-9.
Forbes RB, Colville S, Parratt J, Swingler RJ. The incidence of motor neuron disease in Scotland J Neurol 2007;254 (7):866-9.
Sorenson EJ, Stalker AP, Kurland LP, Windebank AJ. Amyotrophic lateral sclerosis in Olmsted County, Minnesota, 1925 to 1998. Neurology 2002;59:280-2.
Okamoto K, Kobashi G, Washio M, Sasaki S, Yokoyama T, Miyake Y, et al. Descriptive Epidemiology of Amyotrophic Lateral Sclerosis in Japan, 1995-2001. J Epidemiol 2005;15(1):20-3.
Alcaz S, Jarebinski M, Pekmezovic T, Stevic-Morinkovic Z, Pavlovic S, Apostolski S. Epidemiological and clinical characteristics of ALS in Belgrade, Yugoslavia. Acta Neurol Scand 1996;94:264-8.
Tomik B, Nicotra A, Ellis CM, Murphy C, Rabe-Hesketh S, Parton M, et al. Phenotypic differences between African and white patients with motor neuron disease: a case-control study. J Neurol Neurosurg Psychiatr 2000;69:251-3.
Cronin S, Hardiman O, Traynor BJ. Ethnic variation in the incidence of ALS: a systematic review. Neurology 2007; 68:1002-7.
Zoccolella S, Beghi E, Palagano G, Fraddosio A, Samarelli V, Lamberti P, et al. Signs and symptoms at diagnosis of amyotrophic lateral sclerosis: a population-based study in southern Italy. Eur J Neurol 2006;13:789-92.
Weisskopft MG, McCullough ML, Morozova N, Calle EE, Thun MJ, Ascherio A. Prospective Study of Occupation and Amyotrophic Lateral Sclerosis Mortality. Am J Epidemiol 2005;162:1146-52.
Valenti M, Pontiere FE, Conti F, Altobelli E, Manzoni T, Frati L. Amyotrophic lateral sclerosis and sports: a case- control study. Eur J Neurol 2005;12(3):223-5.
Belli S, Vanacore N. Proportionate mortality of Italian soccer players: Is amyotrophic lateral sclerosis an occupational disease? Eur J Epidemiol 2005;20:237-42.
Chio A, Benzi G, Dossena M, Mutani R, Mora G. Severely increased risk of amyotrophic lateral sclerosis among Italian professional football players. Brain 2005;128:472-6.
