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Role of the nigrosome 1 absence as a biomarker in amyotrophic lateral sclerosis.
Moreno-Gambín, María Isabel; Tembl, José I; Mazón, Miguel; Cañada-Martínez, Antonio José; Martí-Bonmatí, Luis; Sevilla, Teresa; Vázquez-Costa, Juan F.
Afiliación
  • Moreno-Gambín MI; Neurosonology Laboratory, Department of Neurology, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
  • Tembl JI; Neurosonology Laboratory, Department of Neurology, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
  • Mazón M; Radiology and Biomedical Imaging Research Group (GIBI230), Hospital Universitario y Politécnico La Fe, Valencia, Spain.
  • Cañada-Martínez AJ; Biostatistics Unit, Instituto de Investigación Sanitaria la Fe (IIS La Fe), Valencia, Spain.
  • Martí-Bonmatí L; Radiology and Biomedical Imaging Research Group (GIBI230), Hospital Universitario y Politécnico La Fe, Valencia, Spain.
  • Sevilla T; ALS Unit, Department of Neurology, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
  • Vázquez-Costa JF; Neuromuscular Research Unit, Instituto de Investigación Sanitaria La Fe (IIS La Fe), Valencia, Spain.
J Neurol ; 269(3): 1631-1640, 2022 Mar.
Article en En | MEDLINE | ID: mdl-34379200
INTRODUCTION: The absence of nigrosome 1 on brain MRI and the hyperechogenicity of substantia nigra (SNh) by transcranial sonography are two useful biomarkers in the diagnosis of parkinsonisms. We aimed to evaluate the absence of nigrosome 1 in amyotrophic lateral sclerosis (ALS) and to address its meaning. METHODS: 136 ALS patients were recruited, including 16 progressive muscular atrophy (PMA) and 22 primary lateral sclerosis (PLS) patients. The SNh area was measured planimetrically by standard protocols. The nigrosome 1 status was qualitatively assessed by two blind evaluators in susceptibility weight images of 3T MRI. Demographic and clinical data were collected and the C9ORF72 expansion was tested in all patients. RESULTS: Nigrosome 1 was absent in 30% of ALS patients (36% of PLS, 29% of classical ALS and 19% of PMA patients). There was no relationship between radiological and clinical laterality, nor between nigrosome 1 and SNh area. Male sex (OR = 3.63 [1.51, 9.38], p = 0.005) and a higher upper motor neuron (UMN) score (OR = 1.10 [1.02, 1.2], p = 0.022) were independently associated to nigrosome 1 absence, which also was an independent marker of poor survival (HR = 1.79 [1.3, 2.8], p = 0.013). CONCLUSION: In ALS patients, the absence of nigrosome 1 is associated with male sex, UMN impairment and shorter survival. This suggests that constitutional factors and the degree of pyramidal involvement are related to the substantia nigra involvement in ALS. Thus, nigrosome 1 could be a marker of a multisystem degeneration, which in turn associates to poor prognosis.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Atrofia Muscular Espinal / Enfermedad de la Neurona Motora / Esclerosis Amiotrófica Lateral Tipo de estudio: Guideline / Prognostic_studies Límite: Humans / Male Idioma: En Revista: J Neurol Año: 2022 Tipo del documento: Article País de afiliación: España Pais de publicación: Alemania
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Atrofia Muscular Espinal / Enfermedad de la Neurona Motora / Esclerosis Amiotrófica Lateral Tipo de estudio: Guideline / Prognostic_studies Límite: Humans / Male Idioma: En Revista: J Neurol Año: 2022 Tipo del documento: Article País de afiliación: España Pais de publicación: Alemania