Parkinsonian traits in amyotrophic lateral sclerosis (ALS): a prospective population-based study

Calvo, Andrea; Chiò, Adriano; Pagani, Marco; Cammarosano, Stefania; Dematteis, Francesca; Moglia, Cristina; Solero, Luca; Manera, Umberto; Martone, Tiziana; Brunetti, Maura; Balma, Michele; Castellano, Giancarlo; Barberis, Marco; Cistaro, Angelina; Artusi, Carlo Alberto; Vasta, Rosario; Montanaro, Elisa; Romagnolo, Alberto; Iazzolino, Barbara; Canosa, Antonio; Carrara, Giovanna; Valentini, Consuelo; Li, Tie Qiang; Nobili, Flavio; Lopiano, Leonardo; Rizzone, Mario Giorgio

doi:10.1007/s00415-019-09305-0

Cited by 26 publications

(35 citation statements)

References 33 publications

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“…Approximately 13% of all ALS patients develop frontotemporal dementia [33] in their disease course, and a similar proportion of ALS patients develop ALS‐plus syndrome [34]. An association between PD and ALS is also recognized in Brait‐Fahn‐Schwartz disease [35], characterized by levodopa‐responsive parkinsonism followed by ALS, and more recent studies report that one‐third of ALS patients develop parkinsonism [18]. However, neuroimaging studies suggest that the parkinsonism in these patients is unrelated to functional impairment of nigrostriatal regions [18] that are commonly associated with PD.…”

Section: Discussionmentioning

confidence: 99%

“…An association between PD and ALS is also recognized in Brait‐Fahn‐Schwartz disease [35], characterized by levodopa‐responsive parkinsonism followed by ALS, and more recent studies report that one‐third of ALS patients develop parkinsonism [18]. However, neuroimaging studies suggest that the parkinsonism in these patients is unrelated to functional impairment of nigrostriatal regions [18] that are commonly associated with PD. Although reported ALS patients with parkinsonism were more likely to be male, with similar clinical, demographic and genetic features compared with ALS cases without parkinsonism [10], it is unlikely that the cases reported in the present study represent all ALS patients that develop clinical parkinsonism.…”

Section: Discussionmentioning

confidence: 99%

“…α‐synuclein deposits in neurons are usually associated with Lewy bodies in Parkinson's disease (PD) and dementia with Lewy bodies. A recent large prospectively based study identified parkinsonian features in up to 30% of ALS patients [18], and 18F‐fluoredeoxyglucose‐positron‐emission tomography suggests brain networks other than nigrostriatal regions that are commonly associated with PD were likely to be involved. Currently, systematic analyses have not been performed to determine the prevalence of Lewy body disease (LBD) associated with clinical parkinsonism in a prospectively collected ALS cohort.…”

Section: Introductionmentioning

confidence: 99%

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Coexisting Lewy body disease and clinical parkinsonism in amyotrophic lateral sclerosis

Forrest

Kim

Sousa

et al. 2021

Euro J of Neurology

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Background Amyotrophic lateral sclerosis (ALS) is associated with a range of clinical phenotypes and shows progressive degeneration of upper and/or lower motor neurons, and phosphorylated 43 kDa TAR DNA‐binding protein (pTDP‐43) inclusions in motor and non‐motor pathways. Parkinsonian features have been reported in up to 30% of ALS patients, and Lewy bodies, normally associated with Lewy body disease (LBD), have been reported in a small number of ALS cases, with unknown clinical relevance. This study investigates the prevalence of clinically relevant LBD in a prospectively studied ALS cohort to determine whether concomitant pathology contributes to the clinical heterogeneity. Methods All ALS cases held by the New South Wales Brain Bank (n = 97) were screened for coexisting LBD consistent with clinical disease (Braak ≥ stage IV). Relevant clinical and genetic associations were determined. Results Six cases had coexisting LBD Braak ≥ stage IV pathology. The age at symptom onset (69 ± 7 years) and disease duration (4 ± 3 years) in ALS cases with coexisting LBD did not differ from ALS cases. Three patients had lower limb onset and two patients had bulbar onset. Two patients developed the clinical features of Parkinson's disease, with one receiving a dual diagnosis. All cases had no known relevant family history or genetic abnormalities. Conclusion The prevalence of clinically relevant LBD pathology in ALS is higher than in the general population, and has implications for clinical and neuropathological diagnoses and the identification of biomarkers.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Coexisting Lewy body disease and clinical parkinsonism in amyotrophic lateral sclerosis

Forrest

Kim

Sousa

et al. 2021

Euro J of Neurology

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“…ALS-parkinsonism, PLS-parkinsonism, ALS-FTD-parkinsonism…) [34]. Moreover, parkinsonian features and a dopaminergic deficit have also been described in the striatum of a variable proportion of cALS patients [30,[35][36][37]. Intriguingly, no association between extrapyramidal features and dopaminergic striatal deficit in ALS patients has been found [30,35,36], suggesting that other circuits are responsible of these symptoms.…”

Section: Discussionmentioning

confidence: 99%

Role of the nigrosome 1 absence as a biomarker in amyotrophic lateral sclerosis

et al. 2021

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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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“…The larger the FA, the stronger the anisotropic dispersion of water molecules, indicating that the degree of myelination is high, the fiber consistency is high, the number of axons is large and the arrangement is close; the lower the FA, the stronger the isotropic dispersion of water molecules, reflecting axonal and / or myelin development disorders, axonal fibrosis and myelin decomposition. By measuring the DTI parameters above, we can evaluate the white matter fiber bundle quantitatively and judge the extent and scope of white matter fiber bundle damage caused by various diseases [53].…”

Section: ) Diffusion Tensor Imaging Parametersmentioning

confidence: 99%

Evaluation of Motor Neuron Injury in ALS by Different Parameters of Diffusion Tensor Imaging

Sang

2020

IEEE Access

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease involving upper and lower motoneurons selectively. The injury of lower motoneurons is closely related to the onset, progression and prognosis of ALS. Therefore, the assessment of the injury of lower motoneurons is of great significance to ALS. Diffusion tensor imaging (DTI) is a new imaging method improved and developed on the basis of diffusion weighted imaging. It uses the anisotropic principle of diffusion movement of water molecules to quantify it from multiple directions, so as to reflect the changes of fine structure and function of living tissues. It can be more objective to find the damage of the upper motor neurons, help the early diagnosis of ALS, and objectively monitor and evaluate the development and treatment of ALS. In this paper, the research and application of different parameters of diffusion tensor imaging to evaluate the motor neuron injury in ALS were studied. Patients with motor neuron injury in ALS were collected as case group and healthy volunteers as control group. The diagnostic value of different parameters of axial DTI in ALS was discussed. The FA and MD values of the pyramidal tract in ALS patients have obvious changes compared with those in healthy people. It can objectively and quantitatively evaluate the pathological changes of the pyramidal tract in ALS patients, and provide valuable information for the diagnosis of ALS. The FA value is more sensitive, and the posterior limb of the internal capsule may be the best place to evaluate the damage of the pyramidal tract. In a word, DTI can objectively and quantitatively evaluate the lesions of pyramidal tract, provide valuable information for the diagnosis of ALS, and hope to provide help for the establishment, diagnosis, evaluation, prognosis, scientific research and drug test of ALS motor nerve injury. INDEX TERMS Diffusion tensor imaging (DTI), amyotrophic lateral sclerosis (ALS), motor neuron injury (MN), evaluation parameters.

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Parkinsonian traits in amyotrophic lateral sclerosis (ALS): a prospective population-based study

Cited by 26 publications

References 33 publications

Coexisting Lewy body disease and clinical parkinsonism in amyotrophic lateral sclerosis

Coexisting Lewy body disease and clinical parkinsonism in amyotrophic lateral sclerosis

Role of the nigrosome 1 absence as a biomarker in amyotrophic lateral sclerosis

Evaluation of Motor Neuron Injury in ALS by Different Parameters of Diffusion Tensor Imaging

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