Subscribe to RSS
DOI: 10.1055/s-0042-1755282
Characterization of the nigroestriatal system in a sample of patients with amyotrophic lateral sclerosis
Caracterização do sistema nigroestriatal em uma amostra de pacientes com esclerose lateral amiotrófica Financia Support Carlos Roberto de Mello Rieder - receives support from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) – Brazil (scholarship of research productivity).
Abstract
Background The coexistence of amyotrophic lateral sclerosis (ALS) with clinical forms of Parkinson disease (PD), although uncommon, is found to a greater degree than one would expect by chance. The pathological mechanisms of ALS and PD are still not fully understood, and the coexistence of these two diseases suggests that they could share mechanisms in common.
Objective Here we present a sample of patients with clinically definitive or probable ALS who were evaluated with single-photon emission computed tomography SPECT/TRODAT and compared with non-ALS controls.
Methods Patients with clinically definite or probable ALS were assessed with the amyotrophic lateral sclerosis functional rating scale (ALSFRS) to define severity and had their demographic data collected. The TRODAT results of patients with ALS were compared with those of patients with a diagnosis of PD with less than 10 years of duration, and with patients with a diagnosis of others movement disorders not associated with neurodegenerative diseases.
Results A total of 75% of patients with ALS had TRODAT results below the levels considered normal; that was also true for 25% of the patients in the control group without neurodegenerative disease, and for 100% of the patients in the PD group. A statistically significant difference was found between patients with ALS and the control group without neurodegenerative disease in the TRODAT values < 0.05.
Conclusions Our study fits with the neuropathological and functional evidence that demonstrates the existence of nigrostriatal dysfunction in patients with ALS. Further research to better understand the role of these changes in the pathophysiological process of ALS needs to be performed.
Resumo
Antecedentes A coexistência da esclerose lateral amiotrófica (ELA) com formas clínicas da doença de Parkinson (DP), embora incomum, é encontrada em um grau maior do que seria esperado ao acaso. Os mecanismos patológicos da ELA e da DP ainda não são totalmente compreendidos e a coexistência dessas duas doenças sugere que elas podem compartilhar mecanismos em comum.
Objetivo Apresentamos uma amostra de pacientes com ELA clinicamente definida ou provável que foram avaliados com tomografia computadorizada por emissão de fóton único (SPECT)/TRODAT e comparados com controles sem ELA.
Métodos Pacientes com ELA clinicamente definida ou provável foram avaliados com a escala funcional de esclerose lateral amiotrófica (ALSFRS) para definir a gravidade e foram coletados os seus dados demográficos. Os resultados do TRODAT de pacientes com ELA foram comparados com aqueles de pacientes com diagnóstico de DP com menos de 10 anos de duração e com pacientes com diagnóstico de outros distúrbios do movimento não associados a doenças neurodegenerativas.
Resultados Um total de 75% dos pacientes com ELA apresentou resultados de TRODAT abaixo dos níveis considerados normais; 25% no grupo controle sem doença neurodegenerativa e 100% no grupo DP. Uma diferença estatisticamente significativa foi encontrada entre os pacientes com ELA e o grupo controle sem doença neurodegenerativa nos valores de TRODAT p < 0,05.
Conclusões Nosso estudo está de acordo com as evidências neuropatológicas e funcionais que demonstram a existência de disfunção nigroestriatal em pacientes com ELA. Mais pesquisas para entender melhor o papel dessas mudanças no processo fisiopatológico da ELA precisam ser realizadas.
Keywords
Amyotrophic Lateral Sclerosis - Parkinson Disease - Dopamine Plasma Membrane Transport Proteins - Striatonigral Degeneration - SPECTPalavras-chave
Esclerose Amiotrófica Lateral - Doença de Parkinson - Proteínas da Membrana Plasmática de Transporte de Dopamina - Degeneração Estriatonigral - SPECTAuthors' Contributions
CAJM, LHTF, NSJ, DTN, EGCN: acquisition of data, literature review; FTR, CRMR: critical revision of manuscript for intellectual content, study supervision.
Publication History
Received: 17 July 2021
Accepted: 12 October 2021
Article published online:
17 October 2022
© 2022. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)
Thieme Revinter Publicações Ltda.
Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil
-
References
- 1 Boillée S, Vande Velde C, Cleveland DW. ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron 2006; 52 (01) 39-59
- 2 McDermott CJ, Shaw PJ. Diagnosis and management of motor neurone disease. BMJ 2008; 336 (7645): 658-662
- 3 Tiryaki E, Horak HA. ALS and Other Motor Neuron Diseases. Continuum 2014; 20 (05) 1185-1207
- 4 Mathis S, Goizet C, Soulages A, Vallat JM, Masson GL. Genetics of amyotrophic lateral sclerosis: A review. J Neurol Sci 2019; 399: 217-226 DOI: 10.1016/j.jns.2019.02.030.
- 5 Özoğuz A, Uyan Ö, Birdal G. et al. The distinct genetic pattern of ALS in Turkey and novel mutations. Neurobiol Aging 2015; 36 (04) 1764.e9-1764 .e18
- 6 Boylan K. Familial Amyotrophic Lateral Sclerosis. Neurol Clin 2015; 33 (04) 807-830 DOI: 10.1016/j.ncl.2015.07.001.
- 7 Kato S, Oda M, Tanabe H. Diminution of dopaminergic neurons in the substantia nigra of sporadic amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol 1993; 19 (04) 300-304
- 8 Gilbert RMW, Fahn S, Mitsumoto H, Rowland LP. Parkinsonism and motor neuron diseases: twenty-seven patients with diverse overlap syndromes. Mov Disord 2010; 25 (12) 1868-1875 DOI: 10.1002/mds.23200.
- 9 Saberi S, Stauffer JE, Schulte DJ, Ravits J. Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants. Neurol Clin 2015; 33 (04) 855-876
- 10 Park HK, Lim YM, Kim JS. et al. Nigrostriatal dysfunction in patients with amyotrophic lateral sclerosis and parkinsonism. J Neurol Sci 2011; 301 (1-2): 12-13 DOI: 10.1016/j.jns.2010.11.017.
- 11 Vogels OJM, Veltman J, Oyen WJ, Horstink MW. Decreased striatal dopamine D2 receptor binding in amyotrophic lateral sclerosis (ALS) and multiple system atrophy (MSA): D2 receptor down-regulation versus striatal cell degeneration. J Neurol Sci 2000; 180 (1-2): 62-65
- 12 Sharma KR, Sheriff S, Maudsley A, Govind V. Diffusion tensor imaging of basal ganglia and thalamus in amyotrophic lateral sclerosis. J Neuroimaging 2013; 23 (03) 368-374
- 13 Hideyama T, Momose T, Shimizu J, Tsuji S, Kwak S. A positron emission tomography study on the role of nigral lesions in parkinsonism in patients with amyotrophic lateral sclerosis. Arch Neurol 2006; 63 (12) 1719-1722
- 14 Borasio GD, Linke R, Schwarz J. et al. Dopaminergic deficit in amyotrophic lateral sclerosis assessed with [I-123] IPT single photon emission computed tomography. J Neurol Neurosurg Psychiatry 1998; 65 (02) 263-265 DOI: 10.1136/jnnp.65.2.263.
- 15 Takahashi H, Snow BJ, Bhatt MH, Peppard R, Eisen A, Calne DB. Evidence for a dopaminergic deficit in sporadic amyotrophic lateral sclerosis on positron emission scanning. Lancet 1993; 342 (8878): 1016-1018
- 16 Deriu M, Murgia D, Paribello A, Marcia E, Melis M, Cossu G. Pisa syndrome as presenting symptom of amyotrophic lateral sclerosis. J Neurol 2011; 258 (11) 2087-2089 DOI: 10.1007/s00415-011-6057-2.
- 17 Drui G, Carnicella S, Carcenac C. et al. Loss of dopaminergic nigrostriatal neurons accounts for the motivational and affective deficits in Parkinson's disease. Mol Psychiatry 2014; 19 (03) 358-367 DOI: 10.1038/mp.2013.3.