J F Vázquez-Costa1, M Campins-Romeu2, J J Martínez-Payá3, J I Tembl4, M E Del Baño-Aledo5, J Ríos-Díaz6, V Fornés-Ferrer7, M J Chumillas8, T Sevilla9. 1. Neuromuscular Research Unit, Instituto de Investigación Sanitaria la Fe (IIS La Fe), Valencia, Spain; ALS Unit, Department of Neurology, Hospital Universitario y Politécnico La Fe, Valencia, Spain; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Valencia, Spain. Electronic address: vazquez_juacos@gva.es. 2. ALS Unit, Department of Neurology, Hospital Universitario y Politécnico La Fe, Valencia, Spain. 3. ECOFISTEM Research Group, Health Sciences Department, Facultad de Ciencias de la Salud, Universidad Católica de Murcia, Campus de los Jerónimos s/n, 30107 Guadalupe (Murcia), Spain. 4. Neurosonology Laboratory, Department of Neurology, Hospital Universitario y Politécnico La Fe, Valencia, Spain. 5. Physiotherapy Department, Facultad de Medicina, Campus de Espinardo 30100, Universidad de Murcia, Spain. 6. Centro de Ciencias de la Salud San Rafael, Universidad Antonio de Nebrija, Madrid, Paseo de la Habana 70 bis, 28036 Madrid, Spain; Fundación San Juan de Dios, Madrid, Spain. 7. Biostatistics Unit, Instituto de Investigación Sanitaria la Fe (IIS La Fe), Valencia, Spain. 8. Neuromuscular Research Unit, Instituto de Investigación Sanitaria la Fe (IIS La Fe), Valencia, Spain; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Valencia, Spain; ALS Unit, Department of Neurophisiology, Hospital Universitario y Politécnico La Fe, Valencia, Spain. 9. Neuromuscular Research Unit, Instituto de Investigación Sanitaria la Fe (IIS La Fe), Valencia, Spain; ALS Unit, Department of Neurology, Hospital Universitario y Politécnico La Fe, Valencia, Spain; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Valencia, Spain; Department of Medicine, University of Valencia, Valencia, Spain.
Abstract
OBJECTIVE: To describe the fasciculation pattern in ALS and to analyse its clinical and pathophysiological significance. METHODS: Ultrasound of 19 muscles was performed in 44 patients with a recent diagnosis (<90 days) of ALS. The number of fasciculations was recorded in each muscle and the muscle thickness and strength were additionally measured in limb muscles. A subgroup of patients were electromyographically assessed. RESULTS: US was performed in 835 muscles and EMG was available in 263 muscles. US detected fasciculations more frequently than EMG. Fasciculations were widespread, especially in upper limbs onset patients and in the cervical region. Fasciculations' number inversely associated with ALSFR-R and body mass index (BMI) and directly with BMI loss and upper motor neuron (UMN) impairment. Our statistical model suggest that fasciculations increase with the initial lower motor neuron (LMN) degeneration, reach their peak when the muscle became mildly to moderately weak, decreasing afterwards with increasing muscle weakness and atrophy. CONCLUSIONS: Our study suggests that both UMN and LMN degeneration trigger fasciculations causing BMI loss. The degree of LMN impairment could account for differences in fasciculations' rates within and between muscles. SIGNIFICANCE: In ALS, fasciculations could explain the link between hyperexcitability and BMI loss.
OBJECTIVE: To describe the fasciculation pattern in ALS and to analyse its clinical and pathophysiological significance. METHODS: Ultrasound of 19 muscles was performed in 44 patients with a recent diagnosis (<90 days) of ALS. The number of fasciculations was recorded in each muscle and the muscle thickness and strength were additionally measured in limb muscles. A subgroup of patients were electromyographically assessed. RESULTS: US was performed in 835 muscles and EMG was available in 263 muscles. US detected fasciculations more frequently than EMG. Fasciculations were widespread, especially in upper limbs onset patients and in the cervical region. Fasciculations' number inversely associated with ALSFR-R and body mass index (BMI) and directly with BMI loss and upper motor neuron (UMN) impairment. Our statistical model suggest that fasciculations increase with the initial lower motor neuron (LMN) degeneration, reach their peak when the muscle became mildly to moderately weak, decreasing afterwards with increasing muscle weakness and atrophy. CONCLUSIONS: Our study suggests that both UMN and LMN degeneration trigger fasciculations causing BMI loss. The degree of LMN impairment could account for differences in fasciculations' rates within and between muscles. SIGNIFICANCE: In ALS, fasciculations could explain the link between hyperexcitability and BMI loss.
Authors: José Ríos-Díaz; María Elena Del Baño-Aledo; José I Tembl-Ferrairó; Maria J Chumillas; Juan F Vázquez-Costa; Jacinto J Martínez-Payá Journal: Eur Radiol Date: 2019-01-21 Impact factor: 5.315
Authors: James A Bashford; Aidan Wickham; Raquel Iniesta; Emmanuel M Drakakis; Martyn G Boutelle; Kerry R Mills; Chris E Shaw Journal: Brain Commun Date: 2020-02-20