scholarly journals Necrotizing Lymphocytic Vasculitis Limited to the Peripheral Nerves: Report of Six Cases and Review

2009 ◽  
Vol 2009 ◽  
pp. 1-5 ◽  
Author(s):  
José Félix Restrepo ◽  
Federico Rondón ◽  
Eric L. Matteson ◽  
Carlos H. Colegial ◽  
Gerardo Quintana ◽  
...  

Background. The systemic vasculitides are syndromes characterized by inflammation and injury (necrosis or thrombosis) of blood vessels, resulting in clinical manifestations according to the affected vascular bed, but not classically in stocking-glove neuropathy.Objective. To describe a form of primary vasculitis affecting strictly peripheral nerves manifesting as stocking-glove neuropathy.Methods. Case series of 110 patients seen in three centers in Bogotá who presented with symptoms and signs of polyneuropathy and/or were identified with vasculitis affecting only the peripheral nerves, and who underwent sural nerve biopsy.Results. Six patients had a vasculitis affecting only the peripheral nerves diagnosed on sural nerve biopsy which demonstrated a mixed infiltrate of monocytes/macrophages and lymphocytes especially in the small epineurial blood vessels. Over time, all had worsening of symptoms, with grip weakness and motor deficits in the hand and feet. Serologies and acute phase reactants were normal in all patients. Treatment response to immunosuppression was satisfactory in 5 patients; 1 patient had progressive neurologic damage.Conclusions. There is a distinct form of primary vasculitis of the peripheral nervous system characterized by distal sensory polyneuropathy with stocking-glove distribution with good prognosis, few and minor relapses and good response to treatment even after delayed diagnosis.

2003 ◽  
Vol 61 (3A) ◽  
pp. 542-546 ◽  
Author(s):  
Marcos R.G. de Freitas ◽  
Osvaldo J.M. Nascimento ◽  
Ernestina A.M. Quaglino ◽  
Andréia Oliveira ◽  
Myrian D. Hahn

Leprosy is one of the most common diseases of the peripheral nerves. In some cases there is only neural involvement without skin changes (neuritic form). The neuropathy has often a distal stocking and glove distribution with thermal and pinprick anesthesia and preservation of proprioception. There is no weakness, the tendon reflexes may be preserved and sometimes the nerves are thickened. We reported 17 patients with a predominantly small-fiber polyneuropathy due to leprosy. All patients had distal temperature and pain anesthesia with different individual variations. The tendon reflexes were normal in seven patients and in eight there was thickening of the nerves. The nerve conduction was normal in three patients. Sural nerve biopsy consisted of: 1) inflammatory infiltrates, 2) vacuolated "foamy" cells, 3) fibrosis of endoneurium, perineurium, and epineurium, 4) partial or total loss of nerve fibers, 5) large number of bacilli. We concluded that in countries where leprosy is frequent, nerve biopsy is an obligatory procedure in patients with predominantly small-fiber polyneuropathy.


2015 ◽  
Vol 86 (11) ◽  
pp. e4.60-e4
Author(s):  
Aisling Carr ◽  
Zane Jaunmuktane ◽  
Sebastian Brandner ◽  
J Kothari ◽  
Julian Blake ◽  
...  

BackgroundUK WM Guidelines recognise 2 mechanisms of nervous system involvement either direct tumour invasion of the CNS or antibody mediated damage to peripheral nerves. We present 5 cases illustrating an extended diversity of PNS involvement in WM with implications for treatment and outcome.CasesA 73 year-old man (IgMκ WM) developed progressively disabling tremor with stable WM. Neurophysiology was demyelinating with disproportionate DMLs; anti-MAG antibodies were strongly positive. He was treated with DRC with symptom stabilisation.A 64 year-old woman (IgMκ) with typical anti-MAG neuropathy developed severe episodic foot pain and ulceration. Skin biopsy confirmed leucocytoclastic vasculitis. She was treated with DRC but died from sepsis.A 43 year-old man (IgMκ WM) with rapidly progressive sensorimotor neuropathy had lymphplasmacytoid cellular infiltrate in sural nerve. He was successfully treated with IDARAM.A 59 year-old man (IgMλ WM) developed small then large fibre axonal neuropathy with autonomic symptoms within 2 years. A SAP scan showed moderate kidney uptake. MRI neurography directed biopsy and confirmed amyloid. Palliative treatment was offered.A 70 year-old woman (IgMλ WM) developed severe burning pains in her feet and became ataxic 2 months later. Sural nerve biopsy confirmed endoneurial amyloid. DRC was given.DiscussionThese cases highlight the importance of accurate diagnosis in WM-associated neuropathy. We propose a novel diagnostic algorithm.


Author(s):  
John L. Beggs ◽  
Peter C. Johnson ◽  
Astrid G. Olafsen ◽  
C. Jane Watkins

The blood supply (vasa nervorum) to peripheral nerves is composed of an interconnected dual circulation. The endoneurium of nerve fascicles is maintained by the intrinsic circulation which is composed of microvessels primarily of capillary caliber. Transperineurial arterioles link the intrinsic circulation with the extrinsic arterial supply located in the epineurium. Blood flow in the vasa nervorum is neurogenically influenced (1,2). Although a recent hypothesis proposes that endoneurial blood flow is controlled by the action of autonomic nerve fibers associated with epineurial arterioles (2), our recent studies (3) show that in addition to epineurial arterioles other segments of the vasa nervorum are also innervated. In this study, we examine blood vessels of the endoneurium for possible innervation.


2021 ◽  
Author(s):  
Thomas Schneider ◽  
Stephan Frank ◽  
Amrei Beuttler ◽  
Suzie Diener ◽  
Kirsten Mertz ◽  
...  

1987 ◽  
Vol 10 (3) ◽  
pp. 246-262 ◽  
Author(s):  
Ruurd Schoonhoven ◽  
Ronald L. L. A. Schellens ◽  
Dick F. Stegeman ◽  
Anneke A. W. M. Gabreëls-Festen

1983 ◽  
Vol 13 (1) ◽  
pp. 65-68 ◽  
Author(s):  
Martin Pollock ◽  
Hitoshi Nukada ◽  
Peter Taylor ◽  
Ivan Donaldson ◽  
Grant Carroll

2019 ◽  
Vol 65 (6) ◽  
pp. 767-770 ◽  
Author(s):  
Renata Carolina Schlögel de Freitas ◽  
Carlos Augusto Zanardini Pereira ◽  
Nadia Tannous Muri ◽  
Valéria Aparecida Zanela Franzon ◽  
Carlos Alberto Camorim Fatuch

SUMMARY Leprosy is a chronic infectious disease caused by Mycobacterium leprae, which affects mainly the skin and peripherical nerves. Brasil has not yet achieved its goal of elimination of the number of cases of this disease, ranking second in terms of absolute numbers worldwide, with India occupying the first position. Primary Neural Leprosy is considered to be a challenge in diagnosis, since it affects the peripherical nerve system with the absence of skin lesions, thus mimicking rheumatological disorders, like in the case presented. A male, 31, with no previous comorbidities, five years ago, started feeling severe pain in the left ankle as well as morning hand pain and stiffness. After many years of being submitted to intense rheumatological disease investigation, they all proved to be negative. Upon physical examination, the patient presented no skin lesions, symmetric polyarthritis in metacarpophalangeal joints and thickness of the left sural nerve. Lab exams showed no alterations and bacilloscopy was negative. Ultrasonography was used to investigate the thickness of the left sural nerve. Biopsy showed a minimal amount of perineural lymphocytes and positive AFB testing. Based on the electroneuromyography, the conclusion was multiple mononeuropathy, and multibacillary polychemotherapy was started. Leprosy remains a public health problem in Brasil. Due to the high prevalence of the disease, our medical colleagues must be alert and trained to recognize this clinical presentation of leprosy. Correct referral to Reference Centers accelerates research, contributing to an accurate diagnosis, classification, and treatment, thus preventing irreversible sequelae with severe functional disability.


2005 ◽  
Vol 7 (1) ◽  
pp. 19-24 ◽  
Author(s):  
Siew-Na Lim ◽  
Chin-Chang Huang ◽  
Hung-Chou Kuo ◽  
Yu-Chen Hsieh ◽  
Chun-Che Chu

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