Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 7317  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Resource Links
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (1,788 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this Article
   Article Figures

 Article Access Statistics
    PDF Downloaded37    
    Comments [Add]    
    Cited by others 2    

Recommend this journal


Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 6  |  Page : 653-655

A case of nonsystemic vasculitic neuropathy presenting with multiple cranial neuropathies

1 Department of Neurology, Kwandong University College of Medicine, Myongji Hospital, Goyang, Korea
2 Department of Pathology,Ewha Womans University School of Medicine,Seoul, Korea

Date of Web Publication29-Dec-2012

Correspondence Address:
Jee Young Kim
Department of Neurology, Kwandong University College of Medicine, Myongji Hospital, Goyang
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.105210

Rights and Permissions

How to cite this article:
Kim JY, Kim DS, Ku BD, Han HJ, Koo H. A case of nonsystemic vasculitic neuropathy presenting with multiple cranial neuropathies. Neurol India 2012;60:653-5

How to cite this URL:
Kim JY, Kim DS, Ku BD, Han HJ, Koo H. A case of nonsystemic vasculitic neuropathy presenting with multiple cranial neuropathies. Neurol India [serial online] 2012 [cited 2021 Mar 1];60:653-5. Available from:


Vasculitic neuropathy is an ischemic nerve damage caused by infiltration of inflammatory cells around blood vessels. [1],[2] "It can be due to systemic vasculitis. [1] However, some vasculitis affect selectively the peripheral nerves without manifestations of other organ damage." It is referred as nonsystemic vasculitic neuropathy (NSVN), vasculitis selectively involving the peripheral nerve. [2],[3] The report presents a patient of NSVN with multiple cranial neuropathies as the initial presenting feature.

A 62-year-old woman presented with left facial weakness in August 2010, and 4 months ago, she received steroids for orbital pain and left III, IV, V, and VI neuropathies. At that time, her orbit computer tomography (CT) was negative. In October 2010, she developed right peripheral facial palsy with no other deficit and nerve conduction studies (NCS) were normal. Brain magnetic resonance imaging (MRI) was normal. Although erythrocyte sedimentation rate (ESR) was elevated (47 mm/h), all serologic markers of autoimmune or connective tissue diseases were unremarkable. With oral steroid therapy, facial palsy got better. In January 2011, she noticed progressive, asymmetric weakness and gait difficulty with pain. One month later, she was hospitalized for quadriparesis with severe burning pain and allodynia. Systemic and skin examination was normal. Neurologic examination revealed left 3 rd -7 th cranial nerve neuropathies and flaccid quadriparesis (Medical Research Council [MRC] scale of strength (3, right/4, left) in upper limbs and MRC (1, right/2, left) in lower limbs) with muscular atrophy and hyporeflexia. All sensory modalities were decreased with a distal to proximal gradient. NCS data were suggestive of mononeuritis multiplex. Serologic markers including anti-neutrophil cytoplasmic antibodies (ANCA), anti-nuclear, paraneoplastic, and anti-Ro/La antibodies, the immunologic markers for syphilis, hepatitis B and C, human immunodeficiency virus (HIV), and serum and urine protein electrophoresis were unremarkable. Cerebrospinal fluid (CSF) study was normal. Sural nerve biopsy showed perivascular infiltration of mononuclear cells, endoneurial fibrosis, and marked degenerated myelinated axons [Figure 1]. After intravenous steroid pulse therapy (1 g/day for 5 days), her arm weakness improved and severe pain subsided gradually with steroid tapering. However, steroid therapy had to be discontinued due to recurrent infection and candidiasis. About 2 months after discontinuation, respiratory difficulty developed and limb weakness got aggravated. Because of aspiration pneumonia, intravenous immunoglobulin was administered for 5 days instead of steroid pulse therapy. After pneumonia improved, oral azathioprine (2 mg/kg) was administered with steroid tapering. Currently she is able to stand and make a few steps, holding hands of a caregiver.
Figure 1: (a) Light microscopic examination of H‑E stained longitudinal section shows multifocal perivascular infiltration of mononuclear cells, decreased myelinated axons, and endoneurial fibrosis (original magnification, 10) (b) Immunostain with CD8 antibody shows scattered groups of positive cells in perivascular areas (original magnification, 4) (c) Electron microscopic examination shows a markedly distorted large myelinated fiber with irregular myelin sheath, abnormal myelin compaction, and vacuolization of axoplasm. Endoneurial collagen deposition is also noted (original magnification, 10,000) (d) Electron microscopic examination shows a Schwann cell cytoplasm containing fragmented myelin and degenerated organelles (original magnification, 15,000)

Click here to view

The typical manifestation of NSVN is a subacute or acute, asymmetric sensorimotor neuropathy with severe neuropathic pain. [1],[2],[3] Because the course of NSVN is indolent and can present a symmetric polyneuropathy, sometimes early differential diagnosis might be difficult. This patient presented initially with cranial neuropathy subsequently developed subacute painful asymmetrical sensorymotor neuropathy and sural nerve showed features of vasculitic neuropathy. Based on these evidences, the diagnosis in this case would be NSVN. However, this case is worthy of notice because it presented multiple cranial neuropathies in the early stage. Cranial neuropathies as the initial presenting feature in NSVN is extremely rare. [3],[4] In the series of 12 patients with NSVN reported by Dyck et al., [4] only one patient "had cranial neuropathies involving trigeminal, axiilary, trigeminal, axillary, and vagus nerves". The initial manifestations in this patient were painful external ophthalmoplegia and recurrent facial palsies. The course of NSVN is often mild and non-fatal. [1],[2],[3] However, our patient had relatively aggressive course in a relatively short period. It is controversial whether NSVN is a tissue-specific vasculitis affecting peripheral nerves or a mild form of systemic vasculitis. [2],[4],[5],[6] The findings in this patient suggest that cranial neuropathy can be the initial manifestation of NSVN. We feel probably the first documentation of such presentation in NSVN.

  References Top

1.Collins MP, Periquet MI. Non-systemic vasculitic neuropathy. Curr Opin Neurol 2004;17:587-98.  Back to cited text no. 1
2.Davies L, Spies JM, Pollard JD, McLeod JG. Vasculitis confined to peripheral nerves. Brain 1996;119:1441-8.  Back to cited text no. 2
3.Collins MP, Periquet MI, Mendell JR, Sahenk Z, Nagaraja HN, Kissel JT. Nonsystemic vasculitic neuropathy: Insights from a clinical cohort. Neurology 2003;61:623-30.  Back to cited text no. 3
4.Dyck PJ, Benstead TJ, Conn DL, Stevens JC, Windebank AJ, Low PA. Nonsystemic vasculitic neuropathy. Brain 1987;110:843-53.  Back to cited text no. 4
5.Kararizou E, Davaki P, Karandreas N, Davou R, Vassilopoulos D. Nonsystemic vasculitic neuropathy: A clinicopathological study of 22 cases. J Rheumatol 2005;32:853-8.  Back to cited text no. 5
6.Said G, Lacroix-Ciaudo C, Fujimura H, Blas C, Faux N. The peripheral neuropathy of necrotizing arteritis: A clinicopathological study. Ann Neurol 1988;23:461-5.  Back to cited text no. 6


  [Figure 1]

This article has been cited by
1 The nonsystemic vasculitic neuropathies
Michael P. Collins,Robert D. Hadden
Nature Reviews Neurology. 2017; 13(5): 302
[Pubmed] | [DOI]
2 Clinical and Electrodiagnostic Characteristics of Vasculitic Neuropathy
Joshua Doo Yong Park,Joshua Jee Young Kim
Jouranl of Korean Association of EMG Electrodiagnostic Medicine. 2015; 17(1): 1
[Pubmed] | [DOI]


Print this article  Email this article
Online since 20th March '04
Published by Wolters Kluwer - Medknow