scholarly journals Inherited neuroaxonal dystrophy in dogs causing lethal, fetal-onset motor system dysfunction and cerebellar hypoplasia

2010 ◽  
Vol 518 (18) ◽  
pp. 3771-3784 ◽  
Author(s):  
John C. Fyfe ◽  
Raba' A. Al-Tamimi ◽  
Rudy J. Castellani ◽  
Diana Rosenstein ◽  
Daniel Goldowitz ◽  
...  
Keyword(s):  
Motor System ◽  
Neuroaxonal Dystrophy ◽  
Cerebellar Hypoplasia

scholarly journals Neuroimaging of Pediatric Cerebellum in Inherited Neurodegenerative Diseases

2021 ◽  
Vol 11 (18) ◽  
pp. 8522
Author(s):  
Luisa Chiapparini ◽  
Marco Moscatelli
Keyword(s):  
Neurodegenerative Disorder ◽  
Pediatric Population ◽  
Cerebellar Atrophy ◽  
Mitochondrial Diseases ◽  
Point Of View ◽  
Neuroaxonal Dystrophy ◽  
Cerebellar Hypoplasia ◽  
Degenerative Diseases ◽  
Cerebellar Ataxias ◽  
Magnetic Resonance Imaging Mri

In the study of cerebellar degenerative diseases, morphologic imaging (computed tomography, CT and magnetic resonance imaging, MRI) is the most common examination. From the clinical and genetic point of view, cerebellar degenerative diseases include heterogeneous conditions in which MRI may show isolated cerebellar atrophy or cerebellar atrophy associated with other cerebellar or supratentorial abnormalities. Neuroradiological progression is often observed. In congenital disorders of glycosylation (CDG), for example, MRI may be normal, may demonstrate mild cerebellar atrophy or, in the advanced stages of the disease, marked atrophy of the cerebellar hemispheres and vermis associated with the abnormal signal intensity of the cerebellar cortex and white matter and brainstem hypotrophy. In spinal cerebellar ataxias (SCAs), very rare in the pediatric population, MRI may demonstrate isolated cerebellar atrophy or cerebellar and brainstem atrophy. MRI shows characteristic findings in other diseases, strongly suggesting a distinct disorder, such as neuroaxonal dystrophy, ARSACS, ataxia-telangiectasia, or precise mitochondrial diseases. An example of neurodegenerative disorder with prenatal onset is pontocerebellar hypoplasia (PCH). PCH represents a group of neurodegenerative disorders characterized by microcephaly, early cerebellar hypoplasia, and variable atrophy of the cerebellum and ventral pons, genetically divided into several subtypes. Cerebellar hypoplasia visible on MRI is often the first sign that suggests the clinical diagnosis. In most cases, the PCH subtype may demonstrate a characteristic pattern distinguishable at MRI. Selective involvement of the cerebellum, sometimes accompanied by brainstem or supratentorial abnormalities in different combinations, may help restrict the differential diagnosis and may address the specific molecular screening.

The Role of Mitochondria in the Genesis of Neuroaxonal Dystrophy in the Brains of Aging Mice

1975 ◽  
Vol 33 ◽  
pp. 372-373
Author(s):  
J.E. Johnson
Keyword(s):  
Electron Microscopy ◽  
Present Report ◽  
Light And Electron Microscopy ◽  
Dorsal Column ◽  
Neuroaxonal Dystrophy ◽  
Nucleus Gracilis ◽  
Dystrophic Axons ◽  
The Brain ◽  
Nucleus Cuneatus

Although neuroaxonal dystrophy (NAD) has been examined by light and electron microscopy for years, the nature of the components in the dystrophic axons is not well understood. The present report examines nucleus gracilis and cuneatus (the dorsal column nuclei) in the brain stem of aging mice.Mice (C57BL/6J) were sacrificed by aldehyde perfusion at ages ranging from 3 months to 23 months. Several brain areas and parts of other organs were processed for electron microscopy.At 3 months of age, very little evidence of NAD can be discerned by light microscopy. At the EM level, a few axons are found to contain dystrophic material. By 23 months of age, the entire nucleus gracilis is filled with dystrophic axons. Much less NAD is seen in nucleus cuneatus by comparison. The most recurrent pattern of NAD is an enlarged profile, in the center of which is a mass of reticulated material (reticulated portion; or RP).

Does the Motor System Facilitate Spatial Imagery?

2017 ◽  
Vol 49 (3) ◽  
pp. 127-137 ◽  
Author(s):  
Markus Krüger ◽  
Horst Krist
Keyword(s):  
Motor System ◽  
Third Graders ◽  
External Support ◽  
New Paradigm ◽  
Perceptual Condition ◽  
Imagery Condition ◽  
Passive Rotation ◽  
Perception Condition ◽  
Motor Condition ◽  
Dynamic Imagery

Abstract. Recent studies have ascertained a link between the motor system and imagery in children. A motor effect on imagery is demonstrated by the influence of stimuli-related movement constraints (i. e., constraints defined by the musculoskeletal system) on mental rotation, or by interference effects due to participants’ own body movements or body postures. This link is usually seen as qualitatively different or stronger in children as opposed to adults. In the present research, we put this interpretation to further scrutiny using a new paradigm: In a motor condition we asked our participants (kindergartners and third-graders) to manually rotate a circular board with a covered picture on it. This condition was compared with a perceptual condition where the board was rotated by an experimenter. Additionally, in a pure imagery condition, children were instructed to merely imagine the rotation of the board. The children’s task was to mark the presumed end position of a salient detail of the respective picture. The children’s performance was clearly the worst in the pure imagery condition. However, contrary to what embodiment theories would suggest, there was no difference in participants’ performance between the active rotation (i. e., motor) and the passive rotation (i. e., perception) condition. Control experiments revealed that this was also the case when, in the perception condition, gaze shifting was controlled for and when the board was rotated mechanically rather than by the experimenter. Our findings indicate that young children depend heavily on external support when imagining physical events. Furthermore, they indicate that motor-assisted imagery is not generally superior to perceptually driven dynamic imagery.

Higher-order representations of newly learnt action sounds in the human motor system

2011 ◽  
Author(s):  
F. Waszak ◽  
S. Schuetz-Bosbach ◽  
C. Weiss ◽  
L. Ticini
Keyword(s):  
Motor System ◽  
Higher Order ◽  
Human Motor

EEG Investigation of Motor System Activity During Language Comprehension

2013 ◽  
Author(s):  
Julie Carranza ◽  
Matthew Bachman ◽  
Michael P. Kaschak ◽  
Edward M. Bernat ◽  
John L. Jones ◽  
...  
Keyword(s):  
Language Comprehension ◽  
Motor System ◽  
System Activity

PONTO-CEREBELLAR HYPOPLASIA: CLINICAL STUDY OF 4 CASES

2006 ◽  
Vol 37 (S 1) ◽  
Author(s):  
F Kammoun ◽  
C Triki ◽  
A Masmoudi ◽  
C Mhiri
Keyword(s):  
Clinical Study ◽  
Cerebellar Hypoplasia

An Inverter-Driven Induction Motor System with a Deadlock Breaking Capability

2012 ◽  
Vol 132 (8) ◽  
pp. 802-807
Author(s):  
Takuto Ichikawa ◽  
Toshiya Yoshida ◽  
Osamu Miyashita
Keyword(s):  
Induction Motor ◽  
Motor System

Effects of sorbinil, dietary myo-inositol supplementation, and insulin on resolution of neuroaxonal dystrophy in mesenteric nerves of streptozocin-induced diabetic rats

Diabetes ◽  
1991 ◽  
Vol 40 (5) ◽  
pp. 574-582 ◽  
Author(s):  
R. E. Schmidt ◽  
S. B. Plurad ◽  
B. D. Coleman ◽  
J. R. Williamson ◽  
R. G. Tilton
Keyword(s):  
Diabetic Rats ◽  
Neuroaxonal Dystrophy
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