Successful generation of peripheral neuropathy with onion-bulb formation in the macrophage scavenger receptor classA knockout mouse treated with isoniazid

✓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.

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

American Journal of Otolaryngology ◽

10.1016/s0196-0709(81)80040-3 ◽

1981 ◽

Vol 2 (4) ◽

pp. 307-313 ◽

Cited By ~ 9

Author(s):

J. Gail Neely ◽

Dawna Armstrong ◽

John Benson ◽

Charles Neblett

Keyword(s):

Onion Bulb ◽

Eighth Nerve ◽

Bulb Formation ◽

Nerve Sheath ◽

Onion Bulb Formation

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

BioMed Research International ◽

10.1155/2017/6481367 ◽

2017 ◽

Vol 2017 ◽

pp. 1-6 ◽

Cited By ~ 2

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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