Upper motor neuron and extra-motor neuron involvement in amyotrophic lateral sclerosis: A clinical and brain imaging review

Graaff, M.M. van der; Jong, J.M.B.V. de; Baas, Frank; Visser, Marjolein

doi:10.1016/j.nmd.2008.10.002

Cited by 69 publications

(39 citation statements)

References 86 publications

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“…ALS is a motor system disease, although extra-motor areas are also involved (van der Graaff et al, 2009). Magnetic resonance imaging (MRI) analysis of regional volumetric changes in ALS patients, particularly voxel-based morphometry (VBM), has indicated that brain atrophy occurs not only in motor areas, but also in non-motor areas, including frontal, temporal, and parietal lobes of both hemispheres (Agosta et al, 2010; Turner and Modo, 2010; Mezzapesa et al, 2013).…”

Section: Upper Motor Neuron In Amyotrophic Lateral Sclerosismentioning

confidence: 99%

Motoneuron firing in amyotrophic lateral sclerosis (ALS)

Carvalho

Eisen

Krieger

et al. 2014

Front. Hum. Neurosci.

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Amyotrophic lateral sclerosis is an inexorably progressive neurodegenerative disorder involving the classical motor system and the frontal effector brain, causing muscular weakness and atrophy, with variable upper motor neuron signs and often an associated fronto-temporal dementia. The physiological disturbance consequent on the motor system degeneration is beginning to be well understood. In this review we describe aspects of the motor cortical, neuronal, and lower motor neuron dysfunction. We show how studies of the changes in the pattern of motor unit firing help delineate the underlying pathophysiological disturbance as the disease progresses. Such studies are beginning to illuminate the underlying disordered pathophysiological processes in the disease, and are important in designing new approaches to therapy and especially for clinical trials.

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Section: Upper Motor Neuron In Amyotrophic Lateral Sclerosismentioning

confidence: 99%

Motoneuron firing in amyotrophic lateral sclerosis (ALS)

Carvalho

Eisen

Krieger

et al. 2014

Front. Hum. Neurosci.

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“…Patients with ALS experience a progressive loss of motor neurons in the spinal cord and brain stem, and may become completely paralyzed toward the later stages of the disease (Gordon, 2013). ALS was traditionally considered to be a pure motor disorder (van der Graaff et al, 2009), but is now considered to be a multisystem neurodegenerative disease including frontal lobe, basal ganglia, and substantia nigra, and the cerebellum (Cellura, 2011; Mochizuki et al, 2012; Williams, 2013). The involvement of the cerebellum in ALS was recently reviewed by Prell and Grosskreutz (2013).…”

Section: Introductionmentioning

confidence: 99%

Overexpression of Human SOD1 Leads to Discrete Defects in the Cerebellar Architecture in the Mouse

et al. 2017

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The human superoxide dismutase 1 (SOD1) gene is responsible for neutralizing supercharged oxygen radicals within the cell. Mutation in SOD1 gene causes amyotrophic lateral sclerosis (ALS). Recent studies have shown involvement of the cerebellum in ALS, although the cerebellar contribution in SOD1 transgenic mice remains unclear. Using immunohistopathology, we investigated the Purkinje cell phenotype in the vermis of the SOD1 transgenic mice cerebellum. Calbindin 1 (Calb1) and three well-known zone and stripe markers, zebrin II, HSP25, and PLCβ4 have been used to explore possible alteration in zone and stripe. Here we show that Calb1 expression is significantly reduced in a subset of the Purkinje cells that is almost aligned with the cerebellar zones and stripes pattern. The Purkinje cells of SOD1 transgenic mice display a pattern of Calb1 down-regulation, which seems to proceed to Purkinje cell degeneration as the mice age. The onset of Calb1 down-regulation in Purkinje cells begins from the central zone and continues into the nodular zone, however it has not been observed in the anterior and posterior zones. In a subgroup of SOD1 transgenic mice in which gait unsteadiness was apparent, down-regulation of Calb1 is seen in a subset of PLCβ4+ Purkinje cells in the anterior zone. These observations suggest that the Calb1− subset of Purkinje cells in the anterior zone, which receives somatosensory input, causes unsteady gait. Our data suggest that human SOD1 overexpression leads to Calb1 down-regulation in the zone and strip pattern and raise the question of whether SOD1 overexpression leads to Purkinje cells degeneration.

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“…[1] Though disorders of the efferent visual system were first reported in the literature in 1978,[2,3] data to support afferent visual system involvement was not reported until twenty years later, when Munte et al described reduced visual evoked potentials in ALS patients. [4,5] Altered afferent visual function has also been documented using functional MRI,[6] and high and low contrast visual acuity (VA) measures, though these latter observations have not been confirmed.…”

Section: Introductionmentioning

confidence: 99%

High and Low Contrast Visual Acuity Are Not Affected in Amyotrophic Lateral Sclerosis

et al. 2016

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The afferent visual system may be affected by neuro-degeneration in amyotrophic lateral sclerosis (ALS) based on observations of visual function impairment and retinal inclusions on histopathology in ALS patients. To test the hypothesis that visual acuity is impaired in ALS, we compared three measures of visual acuity in ALS patients (n = 25) attending a multidisciplinary ALS clinic and age matched control subjects (n = 25). Bilateral monocular and binocular visual acuities were assessed using high contrast (black letters on white background) and low contrast (2.5%, 1.25% grey letters on white background) visual acuity charts under controlled lighting conditions following refraction. Binocular summation was calculated as the difference between binocular and best monocular acuity scores. There were no associations between binocular or monocular high contrast visual acuity or low contrast visual acuity and amyotrophic lateral sclerosis diagnosis (generalized estimating equation models accounting for age). Binocular summation was similar in both amyotrophic lateral sclerosis and control subjects. There was a small magnitude association between increased duration of ALS symptoms and reduced 1.25% low contrast visual acuity. This study does not confirm prior observations of impaired visual acuity in patients with amyotrophic lateral sclerosis and does not support this particular measure of visual function for use in broad scale assessment of visual pathway involvement in ALS patients.

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Upper motor neuron and extra-motor neuron involvement in amyotrophic lateral sclerosis: A clinical and brain imaging review

Cited by 69 publications

References 86 publications

Motoneuron firing in amyotrophic lateral sclerosis (ALS)

Motoneuron firing in amyotrophic lateral sclerosis (ALS)

Overexpression of Human SOD1 Leads to Discrete Defects in the Cerebellar Architecture in the Mouse

High and Low Contrast Visual Acuity Are Not Affected in Amyotrophic Lateral Sclerosis

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