Onion bulb formation in the initial complex of neurons in human dorsal root ganglion: their significance and alterations in amyotrophic lateral sclerosis

1991 ◽  
Vol 82 (6) ◽  
pp. 462-470 ◽  
Author(s):  
S. Murayama ◽  
T. W. Bouldin ◽  
K. Suzuki
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Dorsal Root Ganglion ◽  
Dorsal Root ◽  
Onion Bulb ◽  
Bulb Formation ◽  
Onion Bulb Formation ◽  
Lateral Sclerosis

scholarly journals Accumulation of Misfolded SOD1 in Dorsal Root Ganglion Degenerating Proprioceptive Sensory Neurons of Transgenic Mice with Amyotrophic Lateral Sclerosis

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Javier Sábado ◽  
Anna Casanovas ◽  
Olga Tarabal ◽  
Marta Hereu ◽  
Lídia Piedrafita ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Dorsal Root Ganglion ◽  
Sensory Neurons ◽  
Dorsal Root ◽  
Ventral Horn ◽  
Spinal Cord Dorsal Horn ◽  
Spinal Cord Dorsal ◽  
Neuronal Types ◽  
Misfolded Sod1 ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is an adult-onset progressive neurodegenerative disease affecting upper and lower motoneurons (MNs). Although the motor phenotype is a hallmark for ALS, there is increasing evidence that systems other than the efferent MN system can be involved. Mutations ofsuperoxide dismutase 1(SOD1) gene cause a proportion of familial forms of this disease. Misfolding and aggregation of mutant SOD1 exert neurotoxicity in a noncell autonomous manner, as evidenced in studies using transgenic mouse models. Here, we used theSOD1G93Amouse model for ALS to detect, by means of conformational-specific anti-SOD1 antibodies, whether misfolded SOD1-mediated neurotoxicity extended to neuronal types other than MNs. We report that large dorsal root ganglion (DRG) proprioceptive neurons accumulate misfolded SOD1 and suffer a degenerative process involving the inflammatory recruitment of macrophagic cells. Degenerating sensory axons were also detected in association with activated microglial cells in the spinal cord dorsal horn of diseased animals. As large proprioceptive DRG neurons project monosynaptically to ventral horn MNs, we hypothesise that a prion-like mechanism may be responsible for the transsynaptic propagation of SOD1 misfolding from ventral horn MNs to DRG sensory neurons.

Hypertrophic neurofibrosis with onion bulb formation in an isolated element of the brachial plexus

Neurosurgery ◽  
1981 ◽  
Vol 8 (3) ◽  
pp. 397???9 ◽  
Author(s):  
R A de los Reyes ◽  
J L Chason ◽  
J S Rogers ◽  
J I Ausman
Keyword(s):  
Brachial Plexus ◽  
Onion Bulb ◽  
Bulb Formation ◽  
Onion Bulb Formation

Intraneural perineurioma of the third cranial nerve: occurrence and identification

2006 ◽  
Vol 104 (5) ◽  
pp. 824-827 ◽  
Author(s):  
Rami Almefty ◽  
Bruce L. Webber ◽  
Kenan I. Arnautović
Keyword(s):  
Schwann Cells ◽  
Cranial Nerve ◽  
S100 Protein ◽  
Cranial Nerves ◽  
Onion Bulb ◽  
Subtotal Resection ◽  
Bulb Formation ◽  
Intraneural Perineurioma ◽  
Onion Bulb Formation ◽  
Third Cranial Nerve Palsy

✓Intraneural perineurioma is a true but rare neoplasm that originates from perineurial cells and mainly affects peripheral nerves. It must be distinguished from other hypertrophic neuropathies that are either inflammatory or demonstrate an onion-bulb formation that originates from Schwann cells. Complying with this strict definition, only three additional cases of cranium-related perineurioma have been identified: two lesions arose extracranially and involved cranial nerves, and one occurred intracranially but did not involve a nerve. The authors describe a 27-year-old woman who presented with left third cranial nerve palsy and was found to harbor a mass lesion in the superior orbital fissure and cavernous sinus. After subtotal resection had been performed, pathological studies confirmed the presence of perineurial tumor cells in a pseudo–onion bulb formation. The cells stained positively for epithelial membrane antigen but not for S100 protein, clearly distinguishing the disease from one that originates in Schwann cells.

“Onion bulb” formation associated with a solitary neoplasm of the eighth nerve sheath

1981 ◽  
Vol 2 (4) ◽  
pp. 307-313 ◽  
Author(s):  
J. Gail Neely ◽  
Dawna Armstrong ◽  
John Benson ◽  
Charles Neblett
Keyword(s):  
Onion Bulb ◽  
Eighth Nerve ◽  
Bulb Formation ◽  
Nerve Sheath ◽  
Onion Bulb Formation

scholarly journals Clinical and Pathological Variation of Charcot-Marie-Tooth 1A in a Large Chinese Cohort

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Rui Wu ◽  
He Lv ◽  
Wei Zhang ◽  
Zhaoxia Wang ◽  
Yuehuan Zuo ◽  
...  
Keyword(s):  
Nerve Fiber ◽  
Onion Bulb ◽  
Myelinated Fiber ◽  
Fiber Density ◽  
Charcot Marie Tooth ◽  
Bulb Formation ◽  
Significant Difference ◽  
Axonal Sprouts ◽  
Onion Bulb Formation ◽  
Nerve Fiber Density

Charcot-Marie-Tooth 1A (CMT1A) caused by peripheral myelin protein 22 (PMP22) gene duplication is the most common form of hereditary polyneuropathy. Twenty-four genetically confirmed CMT1A patients with sural nerve biopsies were enrolled in this study. The clinical picture included a great variability of phenotype with mean onset age of 22.2±14.5 years (1–55 years). Pathologically, we observed a severe reduction in myelinated fiber density showing three types of changes: pure onion bulb formation in 3 cases (12.5%), onion bulb formation with axonal sprouts in 10 cases (41.7%), and focally thickened myelin with onion bulb formation or/and axonal sprouts in 11 cases (45.8%). We observed no significant correlation between nerve fiber density and disease duration. There was no significant difference between the 3 pathological types in terms of clinical manifestations, nerve fiber density, and g-ratio. Our study indicates that there is marked variability in the age of onset of CMT1A, as well as significant pathological changes without deterioration with the development of the disease. Focally thickened myelin is another common morphological feature of demyelination.

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