The size distribution of neurons in the motor cortex in amyotrophic lateral sclerosis

Toft, Mette; Gredal, Ole; Pakkenberg, Bente

doi:10.1111/j.1469-7580.2005.00465.x

Cited by 39 publications

(22 citation statements)

References 34 publications

(39 reference statements)

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“…Heterogeneity of motor phenotypes is a clinically well-recognized fundamental aspect of ALS [2,4,6]. In the present series, 8 ALS patients showed LMN-UMN equally affected and 5 cases had predominantly LMN.…”

Section: Discussionmentioning

confidence: 62%

“…Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder characterized by rapid deterioration and selective death of motor neurons (MNs) in the cerebral cortex, brain stem, and spinal cord [1][2][3]. Clinical features are attributable to the superimposition of motor deficits occurring in the upper motor neuron (UMN) and lower motor neuron (LMN).…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Intrathecal delivery of bone marrow stem cells in ALS: A preliminary report

Martínez¹,

Gómez‐Almaguer²,

Jaime‐Pérez³

et al. 2017

Transplant Open

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Section: Discussionmentioning

confidence: 62%

Section: Introductionmentioning

confidence: 99%

Intrathecal delivery of bone marrow stem cells in ALS: A preliminary report

Martínez¹,

Gómez‐Almaguer²,

Jaime‐Pérez³

et al. 2017

Transplant Open

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“…Analyses in human ALS cases have reported that upper and lower motoneurons are significantly shrunken compared with controls (40), whereas no change in neuronal perikarya was found in another investigation on the motor cortex of ALS cases (41). Variations in parameters measured, region analyzed, and differing measuring techniques could account for discrepancies in human studies, but corticospinal neuron degeneration is considered a hallmark of ALS in humans (2).…”

Section: Corticospinal Neuronsmentioning

confidence: 97%

Gene, Cell, and Axon Changes in the Familial Amyotrophic Lateral Sclerosis Mouse Sensorimotor Cortex

Kassa¹,

Mariotti²,

Bonaconsa³

et al. 2009

J Neuropathol Exp Neurol

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Lower motoneuron abnormalities have been extensively documented in the murine model of familial amyotrophic lateral sclerosis, whereas information on corticospinal neurons in these mice is very limited. We investigated 1) mRNA levels of inflammation-related molecules in the deep layers in which corticospinal neurons reside, 2) corticospinal neurons labeled from tracer injections in the corticospinal tract at the cervical level, 3) axonal damage revealed by A-amyloid precursor protein accumulation, and 4) glial cell activation in the sensorimotor cortex of presymptomatic and endstage superoxide dismutase (SOD)-1 (G93A) mice. We demonstrated induction of inflammatory gene transcripts in the deep layers, early and progressive shrinkage of corticospinal cell bodies and activation of surrounding astrocytes and microglia with upregulation of major histocompatibility complex class I antigen. Accumulation of A-amyloid precursor protein in proximal axonal swellings indicating axonal injury was also evident at the terminal stage in the motor cortex and internal capsule. Glial and axon changes were not observed elsewhere in the cortex. These data reveal that the entire motor circuit is affected in this murine amyotrophic lateral sclerosis model as it is in human amyotrophic lateral sclerosis. Sensorimotor cortical inflammation and progressive corticospinal cell body and fiber damage may reflect transsynaptic signaling of damage from lower motoneurons.

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“…The data obtained in each disector and section were then summed to provide the total number of profiles observed ( P Q À ). According also to preceding studies on the same medullary nuclei (Machaalani and Waters, 2003;Kiryu-Seo et al, 2005), neurons were distinguished from glial cells on the basis of their larger size, clearly defined neuronal cytoplasm, with Nissl substance, and membrane-bound nucleus with a clear nucleolus (Toft et al, 2005). For each medullary nucleus, the neuronal density was calculated as follows:…”

Section: Methodsmentioning

confidence: 99%

Morphometric Analysis of Infant and Adult Medullary Nuclei Through Optical Disector Method

Porzionato

Macchi

Parenti

et al. 2009

The Anatomical Record

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In the literature, comprehensive and comparative morphometric studies of infant and adult medullary nuclei performed with unbiased methods are still lacking. In this study, the unbiased quantitative method of the optical disector was applied to analyze neuronal densities, nuclear volumes, and total neuron numbers of the hypoglossal nucleus (XII), dorsal motor nucleus of the vagus (DMNV), nucleus tractus solitarii (NTS), medial vestibular nucleus (MedVe), cuneate nucleus (Cu), nucleus of the spinal trigeminal tract, principal inferior olivary nucleus (PION), medial inferior olivary nucleus (MION), and dorsal inferior olivary nucleus (DION) in adults (16 male, six female; mean age: 37 years) and infants (five male, five female; mean age: 5 months). In both infants and adults, higher neuronal densities were found in the more ventrally located nuclei of the spinal trigeminal tract (mean values (coefficient of variation): 20,947 (0.29) and 8,990 (0.18) neurons/mm 3 , respectively) and inferior olivary complex (PION: 20,010 (0.15) and 9,076 (0.10); MION: 18,667 (0.20) and 9,989 (0.13); DION: 22,424 (0.17) and 10,986 (0.20), respectively) than in the nuclei of the medullary tegmentum, that is, XII (2,747 (0.39) and 1,026 (0.31)), DMNV (2,876 (0.19) and 1,553 (0.26)), NTS (7,993 (0.17) and 2,877 (0.13)), MedVe (7,010 (0.17) and 2,918 (0.12)), and Cu (2,563 (0.23) and 1,038 (0.16)). All the medullary nuclei showed higher volumes and lower neuronal densities in adults than in infants, without statistically significant differences in total neuron numbers, probably because of postnatal development of the neuropil and microvascularization. Anat Rec, 292:1619Rec, 292: -1629Rec, 292: , 2009. V V C 2009 Wiley-Liss, Inc.Key words: morphometry; optical disector; medulla oblongata; human; infantThe medulla oblongata is involved in many different and complex functions as it contains nuclei involved in cardiac and respiratory control, nervous centers providing sensory and motor innervation of peripheral structures, groups of neurons receiving inputs related to the special senses of hearing, vestibular function and taste, or providing/receiving efferent/afferent cerebellar projections. There have been a few studies in the literature detailing morphometric parameters, such as neuronal density, nuclear volume, and total neuron number, in the medullary nuclei considered in this study (e.g

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The size distribution of neurons in the motor cortex in amyotrophic lateral sclerosis

Cited by 39 publications

References 34 publications

Intrathecal delivery of bone marrow stem cells in ALS: A preliminary report

Intrathecal delivery of bone marrow stem cells in ALS: A preliminary report

Gene, Cell, and Axon Changes in the Familial Amyotrophic Lateral Sclerosis Mouse Sensorimotor Cortex

Morphometric Analysis of Infant and Adult Medullary Nuclei Through Optical Disector Method

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