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Churg-Strauss syndrome (CSS) is a rare cause of vasculitic neuropathy. Although rare and potentially fatal, Churg-Strauss syndrome (CSS) is easily diagnosable and treatable. The presence of bronchial asthma with peripheral neuropathy in a patient alerts a physician to this diagnosis. This is vividly illustrated by the presented two cases who had neuropathy associated with bronchial asthma, eosinophilia, sinusitis, and positive perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) test, which improved with administration of steroids.

Background: Vasculitic neuropathy can be confirmed by demonstrating vasculitis in a nerve biopsy, but it is uncertain to what extent combined (i.e. nerve/muscle) biopsy improves the yield. Methods: A random-effects meta-analysis was performed to assess the additional yield of combined biopsy in vasculitic neuropathy. Medline, Embase, LILACS and ISI were searched from January 1980 until January 2009 for relevant articles on the yield of nerve, muscle or combined biopsy to diagnose vasculitic neuropathy. Fourteen (15%) studies were included. Methodological quality was scored using a modified Quality Assessment for Diagnostic Accuracy Studies tool. Results: In patients clinically suspected of vasculitic neuropathy, the additional yield of definite vasculitis in combined biopsy was 5.1% (95% CI 1.1–9.2%; P = 0.013). In patients diagnosed with vasculitic neuropathy, the additional yield of definite vasculitis in combined biopsy was 15% (95% CI 2.1–28%; P = 0.023). Conclusions: There is a modest additional yield of definite vasculitis in combined biopsy compared to nerve biopsy alone. Because of methodological flaws in analysed studies, the findings should be validated in a prospective study.

A woman presented with severe multifocal acute axonal neuropathy due to necrotizing vasculitis. Six years later, electrophysiological examination revealed features suggestive of persistent conduction block (CB) with increased temporal dispersion (ITD) affecting the right median and tibial nerves. A clinical case characterized by multifocal CBs that were observed several years after an initial episode of vasculitic neuropathy was recently reported. The occurrence of CBs paralleled clinical deterioration and was attributed to superimposed antiganglioside antibody-mediated demyelination. In contrast, our patient was clinically stable and did not have antiganglioside antibodies. Nerve conduction studies showed pseudo-CBs rather than true CBs. We suggest that pseudo-CBs with ITD can reveal heterogeneous axonal regeneration as a sequel of a severe multifocal vasculitic neuropathy. This condition should be distinguished from the occurrence of pseudo-CB in the acute phase of vasculitis or from superimposed immune-mediated demyelination. Muscle Nerve 40: 290-293, 2009.

Neuropathic symptoms in patients with diabetes occur commonly and are most often a consequence of the diabetes. Up to 10% of patients with diabetes and neuropathy have an etiology other than diabetes as a cause of their nerve dysfunction. Herein we present a case of vasculitic neuropathy initially misdiagnosed as diabetic neuropathy that led to separate amputations of two toes. This case emphasizes the importance of considering alternative, potentially treatable, causes of peripheral neuropathy in patients with diabetes. (J Am Podiatr Med Assoc 98(4): 322–325, 2008)

Patient: Female, 63 Final Diagnosis: Non-systemic vasculitic peripheral neuropathy Symptoms: Paresthesia Medication: — Clinical Procedure: Sural nerve biopsy Specialty: Rheumatology Objective: Challenging differential diagnosis Background: Non-systemic vasculitic peripheral neuropathy is a rare condition characterized by necrotizing inflammation resulting in luminal narrowing of the vasa nervorum, leading to ischemic injury to peripheral nerves. Here, we present the case of 63-year-old woman with subacute onset of severe hyperesthesia of the lower extremities accompanied by foot drop. Case Report: A 63-year-old woman with prolonged history of uncontrolled diabetes mellitus presented with subacute onset of severe bilateral lower extremity hyperesthesia and motor weakness along with left-sided foot drop. She had multiple emergency room visits with no relief of her symptoms. High doses of analgesics were insufficient to control pain. Laboratory tests were positive only for high erythrocyte sedimentation rate and C-reactive protein. A skin biopsy obtained 5 cm above the left lateral malleolus revealed medium-sized dermal vasculitis with dense mononuclear infiltrate. Electromyography showed peripheral neuropathy. A nerve biopsy was needed to reveal the exact diagnosis. Conclusions: Diagnosis of non-systemic vasculitic peripheral neuropathy can be delayed or missed in patients with uncontrolled diabetes mellitus, leading to significant morbidity. Elevated markers of inflammation in the absence of a possible explanation should prompt the clinician to perform a nerve biopsy; however, it is an invasive procedure and is associated with complications of post-neuropathic pain and delayed wound healing. Magnetic resonance angiography of the lower limbs, if combined with skin biopsy, can save the patient from undergoing nerve biopsy.

Introduction Early comprehensive geriatric assessment (CGA) with good history-taking is essential in assessing the older adult. Case presentation Our patient, a 75-year-old Caucasian woman, was originally admitted to hospital for investigation of iron deficiency anemia. During admission, she developed pneumonia and new intermittent atrial fibrillation in association with a right-sided weakness, which was felt to be new at the time. Following this episode, she was treated for a further chest infection and, despite clinical improvement, her inflammatory markers failed to settle satisfactorily. She was transferred to her local hospital for a period of rehabilitation where further neurological findings made the diagnosis of solely stroke questionable; these findings prompted further history-taking, investigations and input from other disciplines, thereby helping to arrive at a working diagnosis of vasculitic neuropathy. Conclusions The case aims to highlight the importance of taking a good history and performing an early comprehensive assessment in the older adult.

Background: Paraneoplastic vasculitic neuropathy (PVN) is a rare paraneoplastic syndrome. It is characterized by non-systemic subacute vasculitic neuropathy. It is most commonly associated with small cell lung cancers (SCLC) and lymphomas. PVN presents as a painful symmetrical or asymmetrical sensorimotor axonal neuropathy. The neurological symptoms may predate the tumor and may be the initial manifestations, or they may develop after a tumor is diagnosed. Recognition of this entity is important because of its potential treatability. Aim: To study the clinical features of PVN and briefly review the literature. Materials and Methods: The data was collected retrospectively from the medical records of our hospital. Results: Of the 14 cases of paraneoplastic neuropathies, 4 had a PVN. The age of onset was more than 50 years and there was no sex preponderance. Pain was seen in three patients. Two patients were previously treated for a thymoma. Two patients, following their presentation with PVN, were diagnosed with a colonic carcinoma and lung carcinoma, respectively. Conclusions: The recognition of PVN is important as this syndrome may respond to immunosuppression and tumor removal.

The chronic autoimmune neuropathies are a diverse group of disorders, whose diagnosis and classification is based on the clinical presentations and results of ancillary tests. In chronic inflammatory demyelinating polyneuropathy, controlled therapeutic trials demonstrated efficacy for intravenous gamma-globulins, corticosteroids, and plasmaphereis. In multifocal motor neuropathy, intravenous gamma-globulins have been shown to be effective. In the other immune-mediated neuropathies, there are no reported controlled therapeutic trials, but efficacy has been reported for some treatments in non-controlled trials on case studies. Choice of therapy in individual cases is based on reported efficacy, as well as severity, progression, coexisting illness, predisposition to developing complications, and potential drug interactions.

Objective Rituximab is approved for the treatment of granulomatosis with polyangiitis and microscopic polyangiitis. Our objective was to review published clinical evidence on the efficacy of rituximab in the treatment of eosinophilic granulomatosis and polyangiitis (EGPA). Methods We describe a case of refractory EGPA with severe vasculitic neuropathy, which responded impressively to B-cell-depleting therapy. A systematic search of the English literature was also performed to capture all available clinical evidence on the use of rituximab in EGPA. Results We identified a total of 73 EGPA patients who have been treated with rituximab, all data coming from case series or isolated case reports. The majority of patients (85.1%) were treated for refractory or relapsing disease; a mean (SD) of 2.1 (0.9) different immunosuppressive agents were used prior to rituximab administration. Efficacy of RTX therapy was significant in the majority of cases and in a wide variety of disease manifestations; however, a lack of standardized assessment of disease activity before and after treatment was observed in many reports. Overall, 54.0% of patients were treated with a single cycle of rituximab and only 10.8% experienced relapses of the disease. Few significant side effects were observed during a highly variable period of follow-up (3 months to 5 years), mainly severe infections and allergic reactions. Conclusions RTX seems to be effective in cases of severe EGPA refractory to standard of care immunosuppressive treatment, although support comes from case reports and non-controlled studies.

A 58-year-old man with a 15-year history of idiopathic pulmonary fibrosis was hospitalized for rapid progression of muscle weakness to bilateral foot drop. Although laboratory data revealed high titers of myeloperoxidase anti-neutrophil cytoplasmic antibody (489 EU), the patient was diagnosed as polyarteritis nodosa limited to the lower portions of the legs. Despite of the treatment with large doses of corticosteroids and cyclosporin A, his symptoms barely improved during the following two months.

It is with great regret that I have to say good-bye to my CoChief Editor Professor Gerard Said, who after 3 years has decided to step down. His successors, of whom there are two, are Michael Strupp and Massimo Filippi. Gerard took on this role with myself and Thomas Brandt back in January 2009, and since then the journal has continued to grow in terms of the number of submissions it receives and its impact factor, in no small part because of Gerard’s skills and his well-established and leading role in the European Neurological Society (ENS), to which the journal is linked. Gerard qualified in neurology from the medical school of the Salpetriere back in 1971 and since then has become an international academic neurologist of great repute as well as being highly influential in many different areas of neurological practice and training, both nationally and internationally. Apart from being the Professor of Neurology and Chief of Service de Neurologie at the Centre Hospitalier Universitaire de Bicetre, Universite Paris-Sud from 1987 to 2007, he was also a co-founder of the ENS in 1986 with the late P.K. Thomas and Anita Harding. This new society was born out of a sense that there was nothing equivalent to the American Academy of Neurology in Europe. Gerard became the first Secretary General of the society until 2007 and has been instrumental in organising all of their yearly congresses. He became President of the ENS in 2010. His main field of investigation has been in the area of peripheral neuropathies, where he has made many seminal contributions, and he is perhaps best known for his work on diabetic and vasculitic neuropathy. However, his work extends to many aspects of the peripheral nervous system, with influential work on infectious causes of peripheral nerve dysfunction such as HIV-associated neuropathies. This work has led not only to a better description and recognition of these conditions, but also to better investigation and treatment. This ability to master the whole spectrum of peripheral nerve disease has led to him taking an international role in this field of neurology. He has been Director of the Research Group on Neuromuscular Disorders of the World Federation of Neurology, and between 1998 and 2000 he was also President of the Peripheral Nerve Society (PNS). During his 3 years at the helm of the Journal of Neurology Gerard has been instrumental in speeding up the review process, in bringing in new initiatives, and in R. A. Barker (&) Cambridge Centre for Brain Repair, University of Cambridge, The E.D. Adrian Building, Forvie Site, Robinson Way, Cambridge CB2 2PY, UK e-mail: rab46@cam.ac.uk

Nutrient arteries arise from major vessels and penetrate the nerve trunk roughly perpendicular to its axis. These vessels are highly variable in caliber (up to 1 mm) and in location, both between and within (from side to side) individuals. The median and ulnar nerves, for example, have 1–11 (mean 3.24) and 2–19 (mean 7.75) such nutrient arteries in their forearm segments, respectively. After entering the nerve trunk, the nutrient vessels divide into longitudinal ascending and descending branches and supply several arrays of vessels which run along the nerve and interconnect via oblique or laterally directed channels.

Conditions leading to vasculitis of peripheral nerves are heterogeneous. They can be primary or secondary and systemic or nonsystemic. Thus, immune pathology is most likely diverse and differs between diseases. This article focuses on processes of the cellular and humoral immune system related to vasculitis, with an emphasis on those diseases where knowledge has accumulated in recent years. These are the antineutrophil cytoplasmic autoantibody-associated vasculitides, vasculitis associated with cryoglobulinemia and hepatitis C, and nonsystemic vasculitic neuropathy.

A substantial percentage of vasculitic neuropathy patients remain undiagnosed, even after combined nerve/muscle biopsy. In Masuda's article in this issue of the journal, the addition of a simultaneous skin biopsy yielded a mere 12% increase in the diagnostic accuracy; however, this procedure is worth considering because of its non-invasive nature, and future detailed morphological analyses of biopsied skin specimens might further widen the diagnostic window of vasculitic neuropathy.

Chronic immune-mediated neuropathies show high clinical variability. Diagnosis is based on clinical and neurophysiological studies, but recently ultrasound (US) of peripheral nerves has been shown to provide useful morphological information. US has already been shown to crucially influence diagnosis and clinical care in entrapment neuropathies, in traumatic nerve lesions and in tumors. The role of US in the evaluation of polyneuropathies is still not clearly defined, but increasing attention has recently been focused on the immune-mediated neuropathies and specific US measures (namely the intra- and inter-nerve cross-sectional area variability) have been developed. The aim of the current paper is to make a review of the available nerve US studies and provide data from personal observations in the most common chronic immune-mediated neuropathies.

Peripheral neuropathy due to vasculitis without any complications of vasculitis in other organs was first reported in 1987. This condition was termed non-systemic vasculitic neuropathy (NSVN). Although vasculitis is believed to develop in small arteries and arterioles in this disease, the level of vascular involvement has not been fully established. We present a case of NSVN followed up by MR angiography, which was thought to be useful to assess the level as well as the state of vascular lesions in this condition.

Vasculitic neuropathy is a heterogeneous disorder that usually occurs in systemic diseases, but less commonly appears as nonsystemic vasculitic neuropathy (NSVN). This review is intended to highlight recent developments in the field of vasculitic neuropathies.A Peripheral Nerve Society guideline provides data-driven consensus recommendation on classification of vasculitic neuropathies and diagnosis/treatment of NSVN. NSVN is sometimes accompanied by subclinical inflammation of adjacent skin. Amyotrophic lateral sclerosis with sensory involvement can mimic NSVN. Systemic vasculitides with neuropathy include polyarteritis nodosa, microscopic polyangiitis (MPA), rheumatoid vasculitis, Churg-Strauss syndrome (CSS), and hepatitis C-related mixed cryoglobulinemic vasculitis (MCV). At autopsy, MPA affects limb nerves diffusely, with maximal damage in proximal/middle segments. CSS can be accompanied by antineutrophil cytoplasmic antibodies (ANCAs), but most patients with neuropathy lack ANCAs. Cryoglobulinemic neuropathies are usually caused by vasculitis, irrespective of phenotype. Two randomized trials revealed rituximab to be noninferior to cyclophosphamide for inducing remission in ANCA-associated vasculitis. Many reports also document efficacy of rituximab in MCV.Consensus guidelines on NSVN should be evaluated prospectively. MPA-associated vasculitic neuropathy results from vasculitic lesions distributed diffusely throughout peripheral extremity nerves. Rituximab is effective for ANCA-associated and cryoglobulinemic vasculitis with neuropathy.

Sjogren’s syndrome (SjS) is a chronic autoimmune disorder characterized by dysfunction and destruction of exocrine glands, principally the salivary and lacrimal glands. Vasculitis, one of the major extraglandular manifestations of SjS, frequently heralds the need for systemic therapy and serves as an indicator of a risk for poor outcomes in many patients. Poor outcomes associated with the development of ­vasculitis may include direct complications of the vascular inflammation, such as complications of cutaneous ulceration and vasculitic neuropathy, as well as the side effects of the intensive treatment sometimes required to manage vasculitis. The occurrence of vasculitis along with certain other clinical and serologic features (e.g., salivary gland enlargement and low C4 levels) are markers for patients at increased risk for B cell lymphoma development.

Paraneoplastic vasculitic neuropathy has been recently described. We report the first case of this disorder which responded both clinically and electrophysiologically to cyclophosphamide treatment. A 54hyphen;year-old women with a history of metastatic endometrial carcinoma in remission had asymmetrical polyneuropathy, electrophysiological findings consistent with a diffuse axonal neuropathy, high sedimentation rate and spinal fluid protein, and microvasculitis with axonal degeneration on nerve biopsy. The patient was treated with 150 mg of cyclophosphamide daily with gradual clinical and electrophysiological improvement.

Since vasculitic neuropathy is treatable and potentially debilitating, clinicians should develop an approach to neuropathy that increases the likelihood of uncovering existing systemic or nonsystemic vasculitis. The presence of a connective tissue disease, systemic vasculitis, asymmetric or non--length-dependent axonal polyneuropathy, or multiple axonal mononeuropathies should heighten suspicion, but vasculitic neuropathy can also present as a distal symmetric polyneuropathy with or without other organ involvement. Electrodiagnostic testing utilizing extensive nerve conductions may be helpful in identifying features suggestive of vasculitic neuropathy and in selecting an abnormal nerve and muscle for biopsy confirmation. An array of laboratory tests may lead to identification of a systemic disorder that is either characterized by or predisposes to vasculitic neuropathy. The mainstays of treatment are corticosteroids and cyclophosphamide, but other drugs are used in specific conditions. With early diagnosis and careful monitoring of treatment regimens, the prognosis is usually good.

Objectives: To evaluate the diagnostic yield of nerve biopsy in patients with peripheral neuropathy of undetermined cause despite extensive diagnostic workup. Methods: From November 2001 through January 2004, 38 patients underwent nerve biopsy because of unclassified neuropathy. Results: The etiology of the neuropathies could be defined in 14 patients (37%), i.e. in 15% of chronic symmetric, 30% of chronic asymmetric, 50% of subacute symmetric and 62.5% of subacute asymmetric neuropathies. The biopsy was diagnostic in 6 patients (16%), where it showed a vasculitis, and supportive in 8 patients (21%). Conclusions: The contribution of nerve biopsy to the diagnosis of peripheral neuropathy was highest in acute and subacute asymmetric forms of neuropathy and lowest in chronic symmetric forms. The main indication for nerve biopsy remains the diagnosis of vasculitic neuropathy, a potentially treatable disorder.

Leucocytoclastic vasculitis (LCV) is a clinicopathological entity that preferentially involves capillaries or small vessels rather than the medium sized or large arteries typical of polyarteritis nodosa. Its histopathological features are characterised by the presence of perivascular polymorphonuclear leucocytes with fragmented nuclear debris (leucocytoclasis).1 It may be limited to skin lesions such as erythematous macules, purpuric papules, and haemorrhagic vesiculobullous lesions. Although LCV often involves organ systems other than the skin, it is rarely associated with neurological complications, unlike polyarteritis nodosa or Churg-Strauss syndrome. We report a patient who presented with leucocytoclastic vasculitic neuropathy without skin lesions, and discuss the value of doing a combined biopsy of the skin, nerves, and muscle in this patient for the detection of vasculitis. A 44 year old man was admitted to our hospital because of progressive asymmetrical paraesthesiae in all four limbs. Eight months before admission, a tingling sensation had begun in the second finger of his left hand, but it disappeared spontaneously within one month. Over the next seven months, he experienced dysaesthesia which spread gradually on the lateral sides of his feet, and later to his left hand, and he developed drop foot on the right. Two weeks before admission, he had dysaesthesia in his right hand and …

Vasculitic neuropathy usually presents with painful mononeuropathies or an asymmetric polyneuropathy of acute or subacute onset. The disorder should be classified as being systemic or non-systemic. Systemic vasculitis should be further classified into one of the primary and secondary forms. Although specific treatment regimens vary among neurologists, basic principles can be applied. Corticosteroids and cytotoxic drugs have been the mainstay of treatment for most forms of vasculitic neuropathy. Here we discuss dosing, potential side-effects, and management recommendations of conventional treatments. New treatments showing promise include intravenous immunoglobulin and biological agents and trials of the newest treatments are being reviewed. Future trials should compare commonly used treatment regimens and better establish the efficacy of newer, potentially safer, treatments.

Influenza seasonal vaccination is widely performed and clearly justified on public healthy grounds. However, neurological complications have been reported following influenza vaccinations1 […] Vasculitic neuropathy following influenza seasonal vaccination

Peripheral neuropathy can be a manifestation of small-vessel vasculitides such as antineutrophil cytoplasmic antibody-associated vasculitis. Diagnosing vasculitic neuropathy is, however, difficult in many cases. Early treatment focused on achieving remission of the underlying vasculitic process is important as chronic neuropathy has a major influence on a patient's quality of life.

A 46 year old Asian male with previously treated lepromatous leprosy developed a stepwise multifocal sensory disturbance 25years later. Neurophysiology demonstrated marked deterioration from previous studies. Sural nerve biopsy disclosed a vasculitic process superimposed on inactive lepromatous leprosy. Immunocytochemical stains for mycobacterial antigen showed deposits within nerve and vessel walls. A delayed vasculitic neuropathy precipitated by persisting mycobacterial antigen is proposed.

We describe a patient presenting with vasculitic neuropathy associated with cutaneous vasculitis in the absence of other clinical or laboratory evidence of the underlying systemic vasculitis, and showing a favourable prognosis. Although the early proposed criteria for the diagnosis of non-systemic vasculitic neuropathy (NSVN) exclude the involvement of extraneural tissues, the condition observed in our patient might represent a variant of NSVN, with the vasculitis confined to the small vessels in both the peripheral nerve and the skin.

We report a patient with neurosarcoidosis who developed bilateral benign paroxysmal positional vertigo (BPPV) of the posterior canals, deafness, and absent responses to conventional caloric and rotational vestibular testing. Additional rotation in the planes of the vertical semicircular canals revealed relative sparing of vertical canal function. This vertical-horizontal canal dissociation explains the presence of BPPV and suggests that the vestibular damage in this patient is secondary to a vasculitic neuropathy.