Epidemiology and Clinical Description of Amyotrophic Lateral Sclerosis in Low Income Setting: A Syndrome with Short Survival
American Journal of Psychiatry and Neuroscience
Volume 3, Issue 5-1, October 2015, Pages: 5-8
Received: May 27, 2015; Accepted: May 28, 2015; Published: Jun. 17, 2015
Views 4059      Downloads 100
Authors
Komi Assogba, Neurology service, Campus University Teaching Hospital, Lome, Togo
Souleymane Brah, Department of Internal medicine, Niamey University Teaching Hospital, Niamey, Niger
Damelan Kombate, Neurology service, Campus University Teaching Hospital, Lome, Togo
Kossivi M. Apetse, Neurology service, Campus University Teaching Hospital, Lome, Togo
Rabi Barry-Barque, Neurology service, Campus University Teaching Hospital, Lome, Togo
Mofou Belo, Neurological Clinic, Sylvanus Olympio University Hospital of Lome, Lome, Togo
Koffi A. A. Balogou, Neurology service, Campus University Teaching Hospital, Lome, Togo
Article Tools
Follow on us
Abstract
Background: Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease. It evolves to loss of autonomy and death. Objective: To describe the cases of ALS clinically definite observed in hospital field. Patients and methods: The retrospective study has covered a period of 10 years (2003-2012) and a total of 10,128 patient’s files were analyzed. The neurology department of our level 3 hospital has located the current study. Six patients with no particular medical history have been reported. The inclusion patients are cases where the diagnosis of ALS was clinically definite according to the modified El Escorial classification. About 978 of likely, possible and probable cases of ALS were not included. Results: The mean age was 49 years (24 and 67), all males, with a frequency of 0.59 ‰ and an incidence rate of 0.6 cases per year. The clinical signs were marked by the pyramidal syndrome and peripheral neuropathic motor syndrome. MRI or CT scan signs were marked by the bulbar light atrophy. EMG had shown spontaneous activities with reinnervation signs. The histology exam has found neurogenic fascicular atrophy. The average duration of progression of the disease from the diagnostic to death was 17.6 months (6 to 36). The median of survival all cases combined was 42 weeks. Symptomatic treatment was associated to rehabilitation. Riluzole has been established despite its high cost, but without success. Conclusion: This observation highlighted the major difficulties encountered in the management of ALS and its increasing frequency in south Saharan Africa.
Keywords
Amyotrophic Lateral Sclerosis, Degenerative Diseases, Motor Neuron
To cite this article
Komi Assogba, Souleymane Brah, Damelan Kombate, Kossivi M. Apetse, Rabi Barry-Barque, Mofou Belo, Koffi A. A. Balogou, Epidemiology and Clinical Description of Amyotrophic Lateral Sclerosis in Low Income Setting: A Syndrome with Short Survival, American Journal of Psychiatry and Neuroscience. Special Issue: Clinical Neurosciences in Tropical Practice . Vol. 3, No. 5-1, 2015, pp. 5-8. doi: 10.11648/j.ajpn.s.2015030501.12
References
[1]
Van den Berg JP, Kalmijn S, Lindeman E, Veldink JH, de Visser M, Van der Graaff MM, et al. Multidisciplinary ALS care improves quality of life in patients with ALS. Neurology 2005;65:1264-67.
[2]
Mitsumoto H, Rabkin JG. Palliative care for patients with amyotrophic lateral sclerosis: "prepare for the worst and hope for the best". JAMA 2007;298:207-16.
[3]
Bensimon G, Lacomblez L, Delumeau JC, Bejuit R, Truffinet P, Meininger V, et al. A study of riluzole in the treatment of advanced stage or elderly patients with amyotrophic lateral sclerosis. J Neurol 2002;249(5):609-15.
[4]
Practice advisory on the treatment of amyotrophic lateral sclerosis with riluzole: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 1997;49:657-62.
[5]
Rowland LP. Amyotrophic lateral sclerosis. Curr Opin Neurol Neurosurg1994;7:310-15.
[6]
Mitchell JD, Borasio GD. Amyotrophic lateral sclerosis. Lancet 2007;369:2031-41.
[7]
Brooks BR, Miller RG, Swash M, Munsat TL, et al. World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2000;1:293-99.
[8]
Wiedau-Pazos M, Goto JJ, Rabizadeh S, Gralla EB, Roe JA, Lee MK. Altered reactivity of SOD1 in FALS. Science 1996; 271:515-18.
[9]
Allen S, Heath PR, Kirby J, Wharton SB, Cookson MR, Menzies FM, et al. Analysis of the cytosolic proteome in a cell culture model of FALS reveals alterations to the proteasome, antioxidant defenses and nitric oxide synthetic pathways. J Biol Chem 2003;278:6371-83.
[10]
Hand CK, Khoris J, Salachas F, Gros-Louis F, Lopes AA, Mayeux-Portas V, et al. A novel locus for familial amyotrophic lateral sclerosis on chromosome 18q. Am J Hum Genet 2002;70:251-56.
[11]
Delmont E, Roth S, Heudier P, Cua E, Kaphan R, Campagni JP, et al. Primary hyperparathyroidism, a differential diagnosis of motor neuron diseases. Rev Med Interne 2001;22:1253-55.
[12]
Traynor BJ, Codd MB, Corr B, Forde C, Frost E, Hardiman OM. Amyotrophic lateral sclerosis mimic syndromes: a population-based study. Arch Neurol 2000;57:1171-76.
[13]
Eymard B. Polymyositis, dermatomyositis and inclusion body myositis, nosological aspects. Presse Med 2003;32:1656-67.
[14]
Attout H, Rahmeh F, Ziegler F. Gougerot-Sjögren syndrome simulating amyotrophic lateral sclerosis. Rev Med Interne 2000;21:708-12.
[15]
Verma A, Berger JR, Snodgrass S, Petito C. Motor neuron disease: a paraneoplastic process associated with anti-hu antibody and small-cell lung carcinoma. Ann Neurol 1996; 40:112-6.
[16]
Le Forestier N, Maisonobe T, Piquard A, Rivaud S, Crevier-Buchman L. Does primary lateral sclerosis exist? A study of 20 patients and a review of the literature. Brain 2001;124:1989-99.
[17]
Lomen-Hoerth C. Characterization of amyotrophic lateral sclerosis and frontotemporal dementia. Dement Geriatr Cogn Disord 2004;17:337-41.
[18]
Sorenso EJ, Windbank AJ, Mandrekar JN, Bamlet SH. Subcutaneous IGF-1 is not beneficial in 2-year ALS trial. Neurology 2008;71:1770-75.
[19]
Gordon PH, Moore DH, Miller RG, Florence JM, Verheijde JL, Doorish C, et al. Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial. Lancet Neurol 2007;6:1045-53.
[20]
Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol 2006;5:140-47.
[21]
Rabinstein AA, Wijdicks EF. Warning signs of imminent respiratory failure in neurological patients. Semin Neurol 2003;23:97-102.
[22]
Kasarskis EJ, Scarlata D, Hill R, Fuller C, Stambler N, Cedarbaum JM. A retrospective study of percutaneous endoscopic gastrostomy in ALS patients during BDNF and CNTF trials. J Neurol Sci 1999;169:118-25.
[23]
McDermott CJ, Shaw PJ. Diagnosis and management of motor neuron diseases. BMJ 2008;336:658-62.
[24]
Jackson CE, Gronseth G, Rosenfeld J, Barohn RJ, Dubinsky R, Simpson CB, et al. Randomized double-blind study of botulium toxin type B for sialorrhea in ALS patients. Muscle Nerve 2009;39:137-43.
[25]
Rosen HJ, Cummings J. A real reason for patients with pseudobulbar affect to smile. Ann Neurol 2007; 61:92-6.
ADDRESS
Science Publishing Group
1 Rockefeller Plaza,
10th and 11th Floors,
New York, NY 10020
U.S.A.
Tel: (001)347-983-5186