[Localized hypertrophic neuropathy (LHN) of the brachial plexus: diagnosis and presurgical evaluation with MRI].

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[Localized hypertrophic neuropathy (LHN) of the brachial plexus: diagnosis and presurgical evaluation with MRI].

Journal Article•

[Localized hypertrophic neuropathy (LHN) of the brachial plexus: diagnosis and presurgical evaluation with MRI].

M. Saguintaah, C. Veyrac, C. Baud, Couture A

01 Jun 2002-Journal De Radiologie (J Radiol)-Vol. 83, Iss: 6, pp 745-747

TL;DR: L'aspect IRM est evocateur de la neuropathie hypertrophique localisee qui doit etre recherchee au meme titre qu'une compression, une tumeur nerveuse ; les atteintes virales et idiopathiques (IRM normale) restent un diagnostic d'elimination.

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Abstract: The authors report a case of localized hypertrophic neuropathy diagnosed by MRI. This 10-year-old girl presented with painless progressive deltoid atrophy. Electrodiagnostic studies demonstrated chronic denervation. An MRI performed with T1, T2, T1 with gadolinium and fat saturation sequences showed a linear 5-cm long segment of hypertrophic brachial plexus cord, with moderate high T2, intermediate T1 signal, and marked post-Gadolinium enhancement. Progressive improvement followed surgical resection with nerve graft repair. Histological study confirmed LHN (perineurial cell proliferation), a rare cause of peripheral neurological deficit. MRI findings may suggest LHN, that should be suspected as well as peripheral nerve compression or tumor; inflammatory neuritis and idiopathic lesion (with normal MRI findings) remain a diagnosis of exclusion. MR aids in the precise localization of the lesion before surgery.

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MRI neurography and diffusion tensor imaging of a sciatic perineuroma in a child

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Laura Merlini¹, Magalie Viallon², Geraldo De Coulon², Johannes Alexander Lobrinus², Maria Vargas² - Show less +1 more•Institutions (2)

University of Geneva¹, Geneva College²

26 Jun 2008-Pediatric Radiology

TL;DR: The MR protocol combining 3-D T2-W STIR SPACE, fat-saturated gadolinium-enhanced T1-W images, and diffusion tensor imaging with tractography was a valuable tool for depicting peripheral nerve and roots in order to plan surgical treatment.

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Abstract: Perineuroma, rare in children, presents as a painless mononeuropathy of a major nerve trunk. Resection of the lesion with end-to-end sural nerve grafting appears to be the treatment of choice. This technique is not recommended if the unhealthy segment of nerve is too long or if spinal roots are involved. However, in children, reports of direct MR evaluation of nerve trunks and of the exiting nerve roots are limited. We report a 7-year-old girl with an intramural sciatic nerve perineuroma in whom the diagnosis was made by MRI and confirmed by biopsy. The MR protocol combining 3-D T2-W STIR SPACE, fat-saturated gadolinium-enhanced T1-W images, and diffusion tensor imaging with tractography was a valuable tool for depicting peripheral nerve and roots in order to plan surgical treatment.

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25 citations

Journal Article•DOI•

Resection and Graft Repair for Localized Hypertrophic Neuropathy

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Suzie C. Tindall

01 Jul 1998-Neurosurgery

18 citations

Journal Article•DOI•

Localized hypertrophic neuropathy of the sciatic nerve in children: MRI findings.

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Adrien Roux, Catherine Tréguier, Bertrand Bruneau, Franck Marin, Laurent Riffaud, Philippe Violas, Anne Michel, Yves Gandon, Jean-Yves Gauvrit - Show less +5 more

26 Jul 2012-Pediatric Radiology

TL;DR: MRI of the whole sciatic nerve is the method of choice for diagnosing localized hypertrophic neuropathy of the Sciatic nerve in children with a dedicated protocol covering the lumbar spine and the lower limb.

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Abstract: Background Localized hypertrophic neuropathy (LHN) of the sciatic nerve in children is a rare condition characterized by a painless neurological deficit in the sciatic nerve territory.

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14 citations

Cites background or methods from "[Localized hypertrophic neuropathy ..."

...[14] described the benefit and methodology for MRI in LHN of the brachial plexus in a 10year-old child....
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...Abnormal contrast enhancement was most helpful in demonstrating the extension of the lesion, but vessels may be difficult to separate from enhancing nerves [14]....
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...A few reports have emphasized the benefit of MRI in the characterization of LHN [2, 10, 11, 13, 14, 16, 18, 19]....
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Journal Article•DOI•

MRI of intraneural perineurioma of the brachial plexus.

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Efrat Saraf Lavi, Allan D. Levi, Erica K. Schallert¹, Andrew D. Brown², Michael D. Norenberg - Show less +1 more•Institutions (2)

University of Miami¹, Florida International University²

01 Jan 2012-Radiology Case Reports

TL;DR: An 18-year old female with progressive right-hand weakness, numbness, and severe atrophic changes of the hand secondary to an intraneural perineurioma involving the right brachial plexus is reported.

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6 citations

Journal Article•DOI•

Clinical Features, Natural History, and Outcomes of Intraneural Perineuriomas: A Systematic Review of the Literature.

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Karina A. Lenartowicz¹, Anshit Goyal¹, Michelle L. Mauermann¹, Thomas J. Wilson², Robert J. Spinner¹ - Show less +1 more•Institutions (2)

Mayo Clinic¹, Stanford University²

01 Oct 2021-World Neurosurgery

TL;DR: The most common sites were the sciatic nerve or its branches (41.9%), median nerve (13.5%), radial nerve (12.2%), and brachial plexus ( 12.2%) as discussed by the authors.

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4 citations

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From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation.

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Mark D. Murphey¹, Mark D. Murphey², Mark D. Murphey³, W. Sean Smith¹, W. Sean Smith³, Stacy E. Smith², Stacy E. Smith³, Mark J. Kransdorf⁴, H. Thomas Temple⁵, H. Thomas Temple¹, H. Thomas Temple⁶ - Show less +7 more•Institutions (6)

Uniformed Services University of the Health Sciences¹, University of Maryland, Baltimore², Armed Forces Institute of Pathology³, Mayo Clinic⁴, University of Miami⁵, Walter Reed Army Institute of Research⁶

01 Sep 1999-Radiographics

TL;DR: Although differentiation of neurilemoma from neurofibroma and of benign from malignant PNST is problematic, recognition of the radiologic appearances of neurogenic tumors often allows prospective diagnosis and improves clinical management of patients.

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Abstract: Numerous neurogenic tumors can affect the musculoskeletal system, including traumatic neuroma, Morton neuroma, neural fibrolipoma, nerve sheath ganglion, neurilemoma, neurofibroma, and malignant peripheral nerve sheath tumors (PNSTs). The diagnosis of neurogenic tumors can be suggested from their imaging appearances, including lesion shape and intrinsic imaging characteristics. It is also important to establish lesion location along a typical nerve distribution (eg, plantar digital nerve in Morton neuroma, median nerve in neural fibrolipoma, large nerve trunk in benign and malignant PNSTs). Traumatic and Morton neuromas are commonly related to an amputation stump or are located in the intermetatarsal space, respectively. Neural fibrolipomas show fat interspersed between nerve fascicles and are often associated with macrodactyly. Nerve sheath ganglion has a cystic appearance and commonly occurs about the knee. Radiologic characteristics of neurilemoma, neurofibroma, and malignant PNST at computed tomography (CT), ultrasonography, and magnetic resonance imaging include fusiform shape, identification of entering and exiting nerve, low attenuation at CT, target sign, fascicular sign, split-fat sign, and associated muscle atrophy. Although differentiation of neurilemoma from neurofibroma and of benign from malignant PNST is problematic, recognition of the radiologic appearances of neurogenic tumors often allows prospective diagnosis and improves clinical management of patients.

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497 citations

"[Localized hypertrophic neuropathy ..." refers background in this paper

...Cela différencie la NHL des autres syndromes tumoraux des nerfs périphériques, principalement le névrome post traumatique (masse fusiforme ou ronde centrée sur le nerf, en hypersignal T2 hétérogène fasciculaire, traitée par chirurgie en cas d’échec des traitements locaux) ; le neurofibrome et le schwannome, proliférations schwanniennes (PS1OO +) (masse fusiforme encapsulée centrée sur le nerf, graisseuse en périphérie en T1, en « cible » avec hyposignal central en T2) (24)....
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Journal Article•DOI•

Intraneural perineurioma: A clonal neoplasm associated with abnormalities of chromosome 22

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Theresa S. Emory¹, Bernd W. Scheithauer¹, T. Hirose¹, Michael B. Wood¹, Burton M. Onofrio¹, Robert B. Jenkins¹ - Show less +2 more•Institutions (1)

Mayo Clinic¹

01 Jun 1995-American Journal of Clinical Pathology

TL;DR: Eight clinically and morphologically typical perineuriomas were studied by histologic, immunohistochemical and ultrastructural methods, finding that each of 16 metaphases was abnormal; the tumor cells appeared to be homozygously deficient for the region 22q11.

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Abstract: The nature of perineurioma, variably termed "localized hypertrophic neuropathy," "intraneural neurofibroma," and "hypertrophic interstitial neuritis" has long been an issue of contention. Most authors consider it a neoplasm, but some a reactive process. Eight clinically and morphologically typical perineuriomas were studied by histologic, immunohistochemical and ultrastructural methods. One perineurioma was subject to tissue culture and cytogenetic study and another to fluorescence in situ hybridization (FISH) analysis. The patients, 3 males and 5 females, ranged in age from 11 to 38 years. All tumors were intraneural, and involved extremities (2 sciatic, 1 median, 1 femoral, 1 peroneal, 1 brachial plexus, 1 ulnar, and 1 radial). Neurologic symptoms, motor in all cases and sensory in 4, were present from 1 month to 7 years (mean 1.2 years). Fusiform, segmental nerve enlargement was clinically apparent in only two patients, but was evident on MRI in five of eight patients. Lesion length ranged from 3.5 to 30 cm, the largest involving the sciatic nerve from the obturator foramen to the knee. One lesion involved two nerve roots, but no association with a phakomatosis was noted. Treatment consisted of biopsy in six cases and resection in two cases. Histologically, pseudo-onion bulbs composed of epithelial membrane antigen-reactive, S-100 protein-negative perineurial cells surrounded myelinated or nonmyelinated nerve fibers. Many were accompanied by their S-100 protein-positive Schwann sheaths. Some whorls lacked a central axon. A single mitosis was noted in one case. The MIB-1 antigen labelling index ranged from 4% to 17%. Staining for p53 antigen in six cases showed no (2 of 6), rare (2 of 6), or scattered (2 of 6) immunoreactive nuclei. Cytogenetic analysis in one case demonstrated a chromosomally abnormal clone. Each of 16 metaphases was abnormal; the tumor cells appeared to be homozygously deficient for the region 22q11.2qter. In another case, 53% of interphase nuclei showed three FISH signals with a chromosome 14/22 probe, thus suggesting either monosomy for the centromere of chromosome 14 or that of chromosome 22.(ABSTRACT TRUNCATED AT 400 WORDS)

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208 citations

"[Localized hypertrophic neuropathy ..." refers background in this paper

...Des facteurs traumatiques ou toxiques ont été envisagés (9), certains (3, 21) la considèrent comme une affection tumorale....
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...Peu d’articles ont décrit la NHL en IRM, la plupart ne mentionnant qu’un aspect de « gros nerf » (3, 14, 19, 20)....
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...L’apparition des symptômes est le plus souvent insidieuse car indolore (2,5 mois à 25 ans avant le diagnostic dans la série de Gruen (4), 1 mois à 7 ans chez Emory (3))....
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...Emory TS, Scheithauer BW, Hirose T, Wood M, Onofrio B, Jenkins RB. Intraneural perineurioma: a clonal neoplasm associated with abnormalities of chromosome 22....
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...Les deux séries les plus importantes (Gruen (4) 15 cas, Emory (3) 8 cas) retrouvent des patients de 4 à 43 ans....
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Journal Article•DOI•

Immunohistochemical detection of epithelial membrane antigen in normal perineurial cells and perineurioma.

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Aurelio Ariza¹, Juan M. Bilbao, Juan Rosai•Institutions (1)

Yale University¹

01 Sep 1988-The American Journal of Surgical Pathology

TL;DR: The detection of an epithelial marker in perineurial cells is in agreement with the concept of a “perineural epithelium” and seems to support a common embryologic origin for the perineuial cell and the equally EMA-positive arachnoidal cap cell.

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Abstract: The use of epithelial membrane antigen (EMA) as an immunohistochemical marker for normal and neoplastic perineurial cells is described. Normal perineurial cells react strongly for this antigen, which is also expressed by the cells of perineurioma. Instead, neurofibromas and schwannomas only show some peripheral or entrapped layers of EMA-positive cells. In traumatic and Morton's neuromas, bundles of neural fibers are wrapped in layers of EMA-positive perineurial cells. Neurothekeoma and granular cell tumor show no EMA reactivity. The detection of an epithelial marker in perineurial cells is in agreement with the concept of a "perineural epithelium" and seems to support a common embryologic origin for the perineurial cell and the equally EMA-positive arachnoidal cap cell. The availability of an immunohistochemical marker for the perineurial cell provides an easy and convenient tool for the evaluation of the participation of this cell in a variety of pathologic processes.

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166 citations

"[Localized hypertrophic neuropathy ..." refers background in this paper

...746 M Saguintaah et al. : Neuropathie hypertrophique localisée (NHL) du plexus brachial : diagnostic et bilan préoperatoire par l’IRM J Radiol 2002;83 exprimant l’EMA et présence accessoire de quelques éléments Schwanniens PS 100 +)....
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...La NHL, sur le plan histologique, correspond à une prolifération « en bulbe d’oignon » de cellules périneurales exprimant l’EMA (Epithelial Membrane Antigen), et négatives pour la protéine PS 100 (marqueur des cellules de Schwann) (1, 3, 9, 14, 18, 22)....
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...Quelques cellules PS 100 + peuvent toutefois être retrouvées au centre de la lésion (15) comme dans notre observation, en rapport avec des cellules de Schwann constituant les gaines de myéline....
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Journal Article•

Perineurioma (localized hypertrophic neuropathy).

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Juan M. Bilbao, Khoury Nj, Hudson Ar, Briggs Sj

01 Jul 1984-Archives of Pathology & Laboratory Medicine

TL;DR: It is concluded that LHN is not a disorder caused by hyperplasia of Schwann's cells but a benign peripheral nerve tumor of perineurial cell origin, and it is suggested that perineURial and Schwann’s cells may not derive from the same precursor element.

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Abstract: Three patients with localized hypertrophic neuropathy ( LHN ) had a painless, slowly progressive mononeuropathy over many years, resulting in severe focal neurologic deficit. Grossly, the affected nerves showed a fusiform enlargement. Histologically, there was loss of nerve fibers, disorganization of the fascicular pattern, and proliferation of elongated cells with whorl formation. These cells exhibited no immunoreactivity for S-100 protein, and ultrastructural features set them apart from Schwann's cells. We conclude that LHN is not a disorder caused by hyperplasia of Schwann's cells but a benign peripheral nerve tumor of perineurial cell origin. This study also suggests that perineurial and Schwann's cells may not derive from the same precursor element.

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83 citations

"[Localized hypertrophic neuropathy ..." refers background in this paper

...746 M Saguintaah et al. : Neuropathie hypertrophique localisée (NHL) du plexus brachial : diagnostic et bilan préoperatoire par l’IRM J Radiol 2002;83 exprimant l’EMA et présence accessoire de quelques éléments Schwanniens PS 100 +)....
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...La NHL, sur le plan histologique, correspond à une prolifération « en bulbe d’oignon » de cellules périneurales exprimant l’EMA (Epithelial Membrane Antigen), et négatives pour la protéine PS 100 (marqueur des cellules de Schwann) (1, 3, 9, 14, 18, 22)....
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...Quelques cellules PS 100 + peuvent toutefois être retrouvées au centre de la lésion (15) comme dans notre observation, en rapport avec des cellules de Schwann constituant les gaines de myéline....
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...La neuropathie hypertrophique localisée (NHL) est une entité rare et peu connue, dont moins d’une soixantaine de cas ont été décrits dans la littérature (1-21)....
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Journal Article•DOI•

Perineurioma as the cause of localized hypertrophic neuropathy.

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Hiroshi Mitsumoto¹, Asa J. Wilbourn¹, Hershel Goren¹•Institutions (1)

Cleveland Clinic¹

01 Sep 1980-Muscle & Nerve

TL;DR: Clinical, electrodiagnostic, and morphological studies were performed on three patients with localized hypertrophic neuropathy and found that the tumor is a perineurioma, a rare tumor that can occur both in generalized neurofibromatosis and in isolated form and appears to be the cause of localized hypertrophe neuropathy.

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Abstract: Clinical, electrodiagnostic, and morphological studies were performed on three patients with localized hypertrophic neuropathy (LHN). LHN is characterized clinically by slowly progressive motor mononeuropathy without significant pain or numbness. Nerve conduction studies and needle electrode examination show severe focal motor and sensory axonal loss. Morphological findings in the localized areas of enlarged nerves are different from those of generalized hypertrophic neuropathy, entrapment neuropathy, and neurofibroma, and consist of primary perineurial cell hyperplasia. The tumor is thus a perineurioma. This rare tumor can occur both in generalized neurofibromatosis and in isolated form and appears to be the cause of localized hypertrophic neuropathy.

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79 citations