Relevance of the pyramidal syndrome in amyotrophic lateral sclerosis

Álvarez, Nuria; Díez, Laura; Avellaneda-Gómez, C.; Serra, Maria Sala; Rubio, Miguel A.

doi:10.1016/j.nrleng.2016.04.005

Cited by 6 publications

(5 citation statements)

References 15 publications

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“…This study replicated prior imaging research findings in other CNS disorders that PBA is nonlocalizing to any specific brain region, and this study shows new findings that PBA is also nonspecific for neuroinflammation or neuroaxonal loss mechanisms in ALS . This study replicated previously published findings that spasticity was a less frequently occurring UMN sign compared to hyperreflexia in ALS cohorts (Alvarez et al 69% had hyperreflexia versus 17% had spasticity), which is likely also the experience of many neurologists in clinical ALS practice. In this study, while hyperreflexia was universal in at least one segment, less than half of the participants were noted to have spasticity in any spinal segment.…”

Section: Discussionsupporting

confidence: 88%

Comparison of Two Clinical Upper Motor Neuron Burden Rating Scales in ALS Using Quantitative Brain Imaging

Makary

Weerasekara

Rodham

et al. 2021

ACS Chem. Neurosci.

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Several clinical upper motor neuron burden scales (UMNSs) variably measure brain dysfunction in amyotrophic lateral sclerosis (ALS). Here, we compare relationship of two widely used clinical UMNSs in ALS (Penn and MGH UMNSs) with (a) neuroimaging markers of brain dysfunction and (b) neurological impairment status using the gold-standard functional measure, the revised ALS Functional Rating Scale (ALSFRS-R). MGH UMNS measures hyperreflexia alone, and Penn UMNS measures hyperreflexia, spasticity, and pseudobulbar affect. Twenty-eight ALS participants underwent both Penn and MGH UMNSs, at a matching time-point as a simultaneous [11C]PBR28 positron emission tomography (PBR28-PET)/Magnetic Resonance scan and ALSFRS-R. The two UMNSs were compared for localization and strength of association with neuroimaging markers of: (a) neuroinflammation, PBR28-PET and MR Spectroscopy metabolites (myo-inositol and choline) and (b) corticospinal axonal loss, fractional anisotropy (FA), and MR Spectroscopy metabolite (N-acetylaspartate). Among clinical UMN manifestations, segmental hyperreflexia, spasticity, and pseudobulbar affect occurred in 100, 43, and 18% ALS participants, respectively. Pseudobulbar affect did not map to any specific brain regional dysfunction, while hyperreflexia and spasticity subdomains significantly correlated and colocalized neurobiological changes to corticospinal pathways on whole brain voxel-wise analyses. Both UMNS total scores showed significant and similar strength of association with (a) neuroimaging changes (PBR28-PET, FA, MR Spectroscopy metabolites) in primary motor cortices and (b) severity of functional decline (ALSFRS-R). Hyperreflexia is the most frequent clinical UMN manifestation and correlates best with UMN molecular imaging changes in ALS. Among Penn UMNS’s subdomains, hyperreflexia carries the weight of association with neuroimaging markers of biological changes in ALS. A clinical UMN scale comprising hyperreflexia items alone is clinically relevant and sufficient to predict the highest yield of molecular neuroimaging abnormalities in ALS.

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Section: Discussionsupporting

confidence: 88%

Comparison of Two Clinical Upper Motor Neuron Burden Rating Scales in ALS Using Quantitative Brain Imaging

Makary

Weerasekara

Rodham

et al. 2021

ACS Chem. Neurosci.

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“…In our group of patients, at the time of the electrophysiological examination, 77% exhibited frank pyramidal signs (predominant UMN and balanced UMN/LMN forms) (Table ), with spasticity in 27%, Babinski or Hoffman signs in 35% and frank hyperreflexia in 73% cases, which is quite similar to the cohort presented by Álvarez et al . . This indicates that our population was representative of a classic ALS population in which hyperreflexia is a common clinical feature.…”

Section: Discussionmentioning

confidence: 82%

“…These signs tend to remain unchanged over time, although they appear at later stages or even disappear over time in some patients (Álvarez et al . ). In our group of patients, at the time of the electrophysiological examination, 77% exhibited frank pyramidal signs (predominant UMN and balanced UMN/LMN forms) (Table ), with spasticity in 27%, Babinski or Hoffman signs in 35% and frank hyperreflexia in 73% cases, which is quite similar to the cohort presented by Álvarez et al .…”

Section: Discussionmentioning

confidence: 97%

Absence of hyperexcitability of spinal motoneurons in patients with amyotrophic lateral sclerosis

Marchand‐Pauvert

Peyre

Lackmy-Vallée

et al. 2019

The Journal of Physiology

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Key points •Amyotrophic lateral sclerosis (ALS) motoneurons become hypoexcitable with disease progression in experimental models, raising questions about the neural hyperexcitability supported by clinical observations. •A variant of the ∆F method, based on motor unit discharge frequency modulations during recruitment and derecruitment, has been developed to investigate the motoneuron capacity to self‐sustained discharge in patients. •The modulation of motor unit firing rate during ramp contraction and vibration‐induced recruitment are modified in ALS, suggesting lower motoneuron capacity to self‐sustained discharge, which is a sign of hypoexcitability. •∆F‐D decreases with functional impairment and its reduction is more pronounced in fast progressors. •In patients with ALS, motoneurons exhibit hypoexcitability, which increases with disease progression. Abstract Experimental models have primarily revealed spinal motoneuron hypoexcitability in amyotrophic lateral sclerosis (ALS), which is contentious considering the role of glutamate‐induced excitotoxicity in neurodegeneration and clinical features rather supporting hyperexcitability. This phenomenon was evaluated in human patients by investigating changes in motor unit firing during contraction and relaxation. Twenty‐two ALS patients with subtle motor deficits and 28 controls performed tonic contractions of extensor carpi radialis, triceps brachialis, tibialis anterior and quadriceps, aiming to isolate a low‐threshold unit (U1) on the electromyogram (EMG). Subsequently, they performed a stronger contraction or tendon vibration was delivered, to recruit higher threshold unit (U2) for 10 s before they relaxed progressively. EMG and motor unit potential analyses suggest altered neuromuscular function in all muscles, including those with normal strength (Medical Research Council score at 5). During the preconditioning tonic phase, U1 discharge frequency did not differ significantly between groups. During recruitment, the increase in U1 frequency (∆F‐R) was comparable between groups both during contraction and tendon vibration. During derecruitment, the decrease in U1 frequency (∆F‐D) was reduced in ALS regardless of the recruitment mode, particularly for ∆F‐R <8 Hz in the upper limbs, consistent with the muscle weakness profile of the group. ∆F‐D was associated with functional disability and its reduction was more pronounced in patients with more rapid disease progression rate. This in vivo study has demonstrated reduced motoneuron capacity for self‐sustained discharge, and further supports that motoneurons are normo‐ to hypoexcitable in ALS patients, similar to observations in experimental models.

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“…Signs of CSTD were defined as the presence of a Babinski sign in at least one extremity or hyperreflexia in at least two extremities. ( 21 ) We considered that signs of CSTD were present when identified in all clinical evaluations. Information regarding other neurological signs, symptoms or syndromes was recorded from the ECR.…”

Section: Methodsmentioning

confidence: 99%

Critical COVID-19 and neurological dysfunction - a direct comparative analysis between SARS-CoV-2 and other infectious pathogens

Teixeira-Vaz¹,

Rocha²,

Reis³

et al. 2022

RBTI

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Objective To evaluate whether critical SARS-CoV-2 infection is more frequently associated with signs of corticospinal tract dysfunction and other neurological signs, symptoms, and syndromes, than other infectious pathogens. Methods This was a prospective cohort study with consecutive inclusion of patients admitted to intensive care units due to primary infectious acute respiratory distress syndrome requiring invasive mechanical ventilation > 48 hours. Eligible patients were randomly assigned to three investigators for clinical evaluation, which encompassed the examination of signs of corticospinal tract dysfunction. Clinical data, including other neurological complications and possible predictors, were independently obtained from clinical records. Results We consecutively included 54 patients with acute respiratory distress syndrome, 27 due to SARS-CoV-2 and 27 due to other infectious pathogens. The groups were comparable in most characteristics. COVID-19 patients presented a significantly higher risk of neurological complications (RR = 1.98; 95%CI 1.23 - 3.26). Signs of corticospinal tract dysfunction tended to be more prevalent in COVID-19 patients (RR = 1.62; 95%CI 0.72 - 3.44). Conclusion Our study is the first comparative analysis between SARS-CoV-2 and other infectious pathogens, in an intensive care unit setting, assessing neurological dysfunction. We report a significantly higher risk of neurological dysfunction among COVID-19 patients. As such, we suggest systematic screening for neurological complications in severe COVID-19 patients.

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Relevance of the pyramidal syndrome in amyotrophic lateral sclerosis

Cited by 6 publications

References 15 publications

Comparison of Two Clinical Upper Motor Neuron Burden Rating Scales in ALS Using Quantitative Brain Imaging

Comparison of Two Clinical Upper Motor Neuron Burden Rating Scales in ALS Using Quantitative Brain Imaging

Absence of hyperexcitability of spinal motoneurons in patients with amyotrophic lateral sclerosis

Critical COVID-19 and neurological dysfunction - a direct comparative analysis between SARS-CoV-2 and other infectious pathogens

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