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Introduction: Vasculitic neuropathy is a condition in a patient when there is inflammation in the blood vessels supplying peripheral nerves, often causing both sensory and motor dysfunction, showing up as unusual sensations, numbness, pain and weakness of the muscles in the limbs. Usual medical treatment is to use Corticosteroids for a limited period, and recovery may take several months to years from case to case. Yoga Prana Vidya healing protocols were used as complementary medicine in the case presented in this paper to achieve speedy and effective recovery. Method: This paper used case study method going through patient’s medical records, healer’s records and patient feedback. Results: Patient started feeling relief in pain as healing started, and felt relaxed. There were no other noticeable side effects of medicines. The patient fully recovered within 6 months of YPV healing and further healings were discontinued. Conclusions: The YPV healings enabled the patient complementary to the prescribed medication to recover from vasculitis condition physically and emotionally, and also reducing drug induced side effects. Further research is recommended. It will be beneficial to frontline health workers to acquire some working knowledge of YPV healing to complement their specialties to holistically heal the patients.

After initial investigation of patients presenting with symptoms suggestive of neuropathy, a clinical decision is made for a minority of patients to undergo further assessment with nerve biopsy. Many nerve biopsies do not demonstrate a definitive pathological diagnosis and there is considerable cost and morbidity associated with the procedure. This highlights the need for appropriate selection of patients, nerves and neuropathology techniques. Additionally, concomitant muscle and skin biopsies may improve the diagnostic yield in some cases. Several advances have been made in diagnostics in recent years, particularly in genomics. The indications for nerve biopsy have consequently changed over time. This review explores the current indications for nerve biopsies and some of the issues surrounding its use. Also included are comments on alternative diagnostic modalities that may help to supplant or reduce the use of nerve biopsy as a diagnostic test. These primarily include extraneural biopsy and neuroimaging techniques such as magnetic resonance neurography and nerve ultrasound. Finally, we propose an algorithm to assist in deciding when to perform nerve biopsies.

Purpose of review This article discusses peripheral neuropathies associated with vasculitis (isolated or in the setting of systemic vasculitis) and autoimmune connective tissue disease and provides a brief overview of their diagnostic evaluation and management. Recent findings The classification of systemic vasculitic neuropathy and nonsystemic vasculitic neuropathy continues to evolve. Classification according to the presence of antineutrophil cytoplasmic antibodies and their subtypes facilitates prognostication and management. Recent research on antineutrophil cytoplasmic antibody-associated vasculitis has added to our understanding of its neurologic complications. The treatment of vasculitis is also evolving, and new nonsystemic vasculitic neuropathy classification has impacted the treatment and management of this disorder. New classification criteria for Sjogren syndrome (which commonly causes neurologic complications) facilitate accurate and timely diagnosis. Summary Vasculitis and autoimmune connective tissue disease are underrecognized and treatable causes of peripheral neuropathy. Furthermore, peripheral neuropathy may reveal an underlying rheumatologic or vasculitic disorder. Rapid recognition and treatment are essential. Familiarity with the diagnosis and treatment of neuropathies in the setting of connective tissue disease and vasculitis reduces morbidity and, in some cases, mortality.

We herein report a 33-year-old woman who was an asymptomatic hepatitis B virus (HBV) carrier and presented with distal muscle weakness in the legs and asymmetrical paresthesia in the distal extremities. A nerve biopsy specimen revealed fibrinoid necrosis associated with inflammatory infiltration in the perineural space, and deposition of hepatitis B core antigen and C4d complement was detected in the vascular endothelial cells as well as around the vessels. She was diagnosed with HBV-related vasculitic neuropathy and treated with intravenous immunoglobulin (IVIG). Her symptoms completely subsided after eight weeks. Vasculitic neuropathy rarely develops in the chronic inactive stages of HBV infection. This is the first report of an HBV-inactive carrier with vasculitic neuropathy successfully treated with IVIG.

Vasculitis is a clinically diverse group of diseases with histopathological signs of blood vessel inflammation, which contributes to vascular damage and ischemic damage to the affected tissues. Vasculitic neuropathy is a common complication of the primary systemic vasculitides, such as polyartertis nodosa and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, systemic diseases of the connective tissue - systemic lupus erythematosus and Sjogren syndrome, vasculitis associated with infection, most often viral hepatitis C and B and non - systemic vasculitis neuropathy. Vessels of medium and small caliber are involved in the pathological process in these diseases. With all vasculitis, except for those caused by the direct effect of the infectious trigger on the blood vessel walls, the main pathogenetic mechanism is an autoimmune process with the development of vasa nervorum vasculitis - small arteries and vessels that supply peripheral nerves, and the outcome - nerve ischemia. The classic clinical presentation is an acute or subacute painful multifocal neuropathy that has a predilection for the lower extremities, affects two or more named nerves, and progresses in a step wise manner. However, vasculitic neuropathy can manifest in a variety of ways, including asymmetric polyneuropathies and distal symmetric sensory neuropathies, and it also can be slowly progressive, particularly in cases of nonsystemic vasculitic neuropathy (NSVN), a form of vasculitis that clinically remains restricted to peripheral nerves. Nerve biopsy can help establish the diagnosis of a systemic vasculitis, particularly when other organ involvement is not clinically apparent, and is required for diagnosis of NSVN. Neuropathy due to systemic vasculitis should be treated in accordance with the recommendations for the treatment of the underlying disease. In NSVH, the main medicine of choice are glucocrticoids, and in severe/progressive cases, pulse therapy with cyclophosphamide.Поражение периферической нервной системы при системных васкулитах - вопросы диагностики и лечения Васкулиты представляют собой клинически разнообразную группу заболеваний с гистопатологическими признаками воспаления кровеносных сосудов, которое способствует поражению сосудов и ишемическому повреждению пораженных тканей. Наиболее часто васкулитная нейропатия является осложнением таких первичных системных васкулитов, как узелковый полиартериит, и ассоциированных с антинейтрофильными цитоплазматическими антителами (АНЦА) васкулитов, системных заболеваний соединительной ткани - системной красной волчанки и синдрома Шегрена, васкулита, ассоциированного с инфекцией (чаще всего это вирусные гепатиты С и В) и несистемной васкулитной нейропатии (НСВН). При этих заболеваниях в патологический процесс вовлекаются сосуды среднего и малого калибра. При всех васкулитах, кроме тех, которые обусловлены непосредственным влиянием инфекционного триггера на стенки сосудов, основной патогенетический механизм - это аутоиммунный процесс с развитием васкулита vasa nervorum (мелких артерий и сосудов, которые кровоснабжают периферические нервы), а исход - ишемия нервов. Классическая клиническая картина ишемической нейропатии - это острая или подострая болезненная мультифокальная нейропатия с преимущественным поражением нервных структур нижних конечностей, поражающая два или более крупных нерва и прогрессирующая поэтапно. Однако васкулитная нейропатия может манифестировать различно, включая асимметричные полинейропатии и дистальные симметричные сенсорные нейропатии; кроме того, она может быть медленно прогрессирующий, особенно в случаях НСВН, которая клинически остается ограниченной периферическими нервами. Биопсия нерва может помочь установить диагноз системного васкулита, особенно когда поражение других органов не является клинически очевидным, и требуется для диагностики НСВН. Нейропатии, обусловленные системным васкулитом, следует лечить в соответствии с рекомендациями по лечению основного заболевания. При НСВН основными препаратами выбора являются глюкокортикоиды, а в тяжелых/прогрессирующих случаях используется пульс - терапия с циклофосфамидом.

Rheumatoid vasculitis which affects small-to-medium-sized vessels is a rare and late complication of rheumatoid arthritis. It is defined histologically as immune complex deposition in venules, capillaries and arterioles.1 Vasculitis in the vasa nervorum leads to infarction of peripheral nerves which leads to neuropathy. We present a case of mononeuritis multiplex due to rheumatoid vasculitis.

Single-organ vasculitis of the peripheral nervous system (PNS) is often designated nonsystemic vasculitic neuropathy (NSVN). Several variants or subtypes have been distinguished, including migratory sensory neuropathy, postsurgical inflammatory neuropathy, diabetic radiculoplexus neuropathies, skin-nerve vasculitides, and, arguably, neuralgic amyotrophy. NSVN often presents as nondiabetic lumbosacral radiculoplexus neuropathy (LRPN). This review updates classification, clinical features, epidemiology, and imaging of these disorders.A recent study showed the annual incidence of LRPN in Olmstead County, Minnesota to be 4.16/100 000:2.79/100 000 diabetic and 1.27/100 000 nondiabetic. This study was the first to determine the incidence or prevalence of any vasculitic neuropathy. In NSVN, ultrasonography shows multifocal enlargement of proximal and distal nerves. In neuralgic amyotrophy, MRI and ultrasound reveal multifocal enlargements and focal constrictions in nerves derived from the brachial plexus. Histopathology of these chronic lesions shows inflammation and rare vasculitis. Diffusion tensor imaging of tibial nerves in NSVN revealed decreased fractional anisotropy in one study.Single-organ PNS vasculitides are the most common inflammatory neuropathies. Neuralgic amyotrophy might result from PNS vasculitis, but further study is necessary. The usefulness of focal nerve enlargements or constrictions in understanding pathological mechanisms, directing biopsies, and monitoring disease activity in NSVN should be further investigated.

espanolObjetivo Revisar la eficacia y seguridad del rituximab en neuropatia vasculitica (NV). Metodos Se realizo una busqueda en la literatura de Medline y Embase hasta 2017. Los terminos incluidos guardaron relacion con «vasculitis», «neuropatia vasculitica» y «rituximab». Dicha busqueda fue realizada por 2 revisores. El resultado principal fue la eficacia del rituximab. Resultados Tras seleccionar inicialmente 702 articulos, 5 de ellos permanecieron con un nivel de evidencia de entre 1+ y 3, y un grado de recomendacion variable. En el unico ensayo clinico incluido, el rituximab fue superior a la terapia convencional para vasculitis crioglobulinemica, mostrando NV un incremento en la tasa de retencion farmacologica (64,3 vs. 3,5%; p Conclusiones Rituximab parece ser un tratamiento eficaz y seguro para NV, en el contexto de vasculitis crioglobulinemica. Se carece de evidencia sobre la eficacia especifica de NV en el contexto de otras vasculitis. EnglishObjective To review the efficacy and safety of rituximab in vasculitic neuropathy (VN). Methods A literature search was performed on Medline and Embase up until 2017. It included terms related to “vasculitis”,“vasculitic neuropathy” and “Rituximab”. Research was carried out by two reviewers. The main outcome was rituximab efficacy. Results Of an initial selection of 702 articles, 5 remained with a level of evidence between 1+ and 3 and variable recommendation degree. In the only clinical trial included, rituximab was superior to conventional therapy for cryoglobulinemic vasculitis with VN showing an increase in drug retention rate (64.3% vs. 3.5%; P Conclusions Rituximab seems an effective and safe treatment for VN in the context of cryoglobulinemic vasculitis. Evidence for specific efficacy in VN in the context of other types of vasculitis is lacking.

This article discusses the chronic immune-mediated polyneuropathies, a broad category of acquired polyneuropathies that encompasses chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), the most common immune-mediated neuropathy, the CIDP variants, and the vasculitic neuropathies. Polyneuropathies associated with rheumatological diseases and systemic inflammatory diseases, such as sarcoidosis, will also be briefly covered. These patients' history, examination, serum studies, and electrodiagnostic studies, as well as histopathological findings in the case of vasculitis, confirm the diagnosis and differentiate them from the more common length-dependent polyneuropathies. Prompt identification and initiation of treatment is imperative for these chronic immune-mediated polyneuropathies to prevent disability and even death.

Immune axonal neuropathies are a particular group of immune-mediated neuropathies that occasionally accompany systemic autoimmune rheumatic diseases such as connective tissue dissorders and primary systemic vasculitides. Apart from vasculitis of vasa nervorum, various other mechanisms are involved in their pathogenesis, with possible therapeutic implications. Immune axonal neuropathies have highly heterogeneous clinical presentation and course, ranging from mild chronic distal sensorimotor polyneuropathy to severe subacute mononeuritis multiplex with rapid progression and constitutional symptoms such as fever, malaise, weight loss and night sweats, underpinning a vasculitic process. Sensory neuronopathy (ganglionopathy), small fiber neuropathy (sensory and/or autonomic), axonal variants of Guillain-Barré syndrome and cranial neuropathies have also been reported. In contrast to demyelinating neuropathies, immune axonal neuropathies show absent or reduced nerve amplitudes with normal latencies and conduction velocities on nerve conduction studies. Diagnosis and initiation of treatment are often delayed, leading to accumulating disability. Considering the lack of validated diagnostic criteria and evidence-based treatment protocols for immune axonal neuropathies, this review offers a comprehensive perspective on etiopathogenesis, clinical and paraclinical findings as well as therapy guidance for assisting the clinician in approaching these patients. High quality clinical research is required in order to provide indications and follow up rules for treatment in immune axonal neuropathies related to systemic autoimmune rheumatic diseases.

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Multiple myeloma (MM) is a clonal proliferation of plasma cells in the bone marrow resulting in the production of paraproteins. It is more common in elderly adults and presents with nonspecific symptoms like bone pain, pathological fracture, fatigue, and signs of hypercalcemia. Peripheral neuropathy is an atypical presentation. We present a rare case of vasculitic neuropathy (VN) who was also diagnosed with MM at the same time. Nerve conduction study and biopsy showed findings suggestive of demyelinating VN. His serum protein electrophoresis and bone marrow aspirate were consistent with MM. The association between these two conditions remains understudied. So far there is no strong evidence suggesting an association between MM and VN. If VN was just coincidental or a presenting symptom of MM remains a question and warrants further studies.

Due to its rarity and the limited literature, the clinicopathological characteristics of peripheral nerve involvement in immunoglobulin G4 (IgG4)-related disease are unknown. We present two cases of IgG4-related disease, accompanied by peripheral neuropathy, presenting as unilateral ptosis (case 1) and sclerosing cholangitis (case 2), respectively. In both cases, sural nerve biopsy indicated vasculitis as the underlying pathophysiology; the peripheral neuropathy was refractory to corticosteroid therapy. In contrast to the previously proposed pathomechanism of IgG4-related neuropathy (direct lymphoplasmacytic infiltration), the pathological findings in our cases suggest that vasculitis occurs secondary to systemic autoimmune conditions.

Asymmetric and rapidly progressive painful sensorimotor disturbances raise concern for vasculitic neuropathy, the most common cause of which is non-systemic vasculitic neuropathy (NSVN). Nerve damage occurs by ischemic injury via mechanisms that differ between each process. The diagnosis is confirmed by history, examination, electrodiagnostic studies, laboratory findings, and likely nerve/muscle biopsy. Treatment is dependent on the underlying cause, and diagnosis is focused on evaluation for systemic vasculitides that can result in significant morbidity. In the case of NSVN, corticosteroids and consideration of other immunosuppressants are necessary to avoid further injury and disability.

Nerve biopsy represents the conclusive step in the diagnostic work-up of peripheral neuropathies, and its diagnostic yield is still debated. The aim of this study is to consider the impact of nerve biopsy on reaching a useful diagnosis in different peripheral neuropathies and its changing over time. We retrospectively analyzed 1,179 sural nerve biopsies performed in the period 1981–2017 at Neurological Clinic of Policlinico San Martino (Genoa). We relied on medical records and collected both clinical and pathological data in a database. Biopsy provided univocal diagnoses in 53% of cases (with an increase over time), multiple diagnostic options in 14%, while diagnosis was undetermined in 33% (undetermined reports decreased during the years). In 57% of patients, the pre-biopsy suspicion was confirmed, while in 43% sural biopsy modified the clinical diagnosis. The highest yield was in axonal neuropathies (29% undetermined reports vs. 40% in demyelinating and 48% in mixed neuropathies). In 68% of patients with vasculitic neuropathy, this etiology was already suspected, whereas in 32% nerve biopsy modified the clinical diagnosis. During the years, the number of annually performed biopsies decreased significantly (p = 0.007), with an increase in the mean age of patients (p < 0.0001). The percentage of hereditary neuropathies had a significant decrease (p = 0.016), while the rate of vasculitic and chronic inflammatory neuropathies increased (p < 0.0001). This is the largest Italian study addressing the yield of sural nerve biopsy. During the years, we observed a progressive refinement of the indication of this procedure, which confirms its utility for interstitial neuropathies, particularly if non-systemic vasculitic neuropathy is suspected.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.

Introduction The aim of this study was to determine the diagnostic usefulness of skin punch biopsies with emphasis on visualization and quantification of T-cells and macrophages in patients with polyneuropathies. Methods: We quantified inflammatory cells in skin samples (lower leg, upper thigh) in 187 patients and compared data with counts in their sural nerve biopsies and with skin biopsies from 32 healthy volunteers. Results: Vessel-bound T-cells and macrophages were increased in proximal and distal skin samples of neuropathy patients compared with controls (P

We herein report a case of peripheral neuropathy following exposure to large amounts of glyphosate-based herbicide. A 70-year-old man suffered from pain and purpura in the left sole following exposure to glyphosate-based herbicide. Pain and purpura spread to the opposite side and increased in severity. Mild weakness of the lower limbs was also observed. A sural nerve biopsy revealed the infiltration of lymphocytes around small vessels in the epineurium with numerous eosinophils, deposition of hemosiderins and focal axonal degeneration, compatible with findings of vasculitic neuropathy. Glyphosate-based herbicides should be recognized as a causative agent of vasculitic neuropathy.

A 21-year-old female born of nonconsanguineous marriage presented with gradually progressive quadriplegia for 3 years. Weakness was distal more than proximal, with wasting. She also had moderate pain in extremities. She had significant weight loss. Clinically, she had lower motor neuron flaccid quadriplegia with absent deep tendon jerks. Sensations of touch and pain were diminished in bilateral feet till ankle joints. Peripheral nerves were not thickened. Nerve conduction study was suggestive of pure axonal sensorimotor polyneuropathy. All routine hematological and biochemical parameters, erythrocyte sedimentation rate, antinuclear antibodies, and antinuclear cytoplasmic antibodies were negative. Cerebrospinal fluid showed no cells, normal sugar, and raised proteins. Biopsy of sural nerve was suggestive of vasculitic neuropathy. This was a case of nonsystemic vasculitic neuropathy that responded to steroids.

Renaut corpuscles are cylindrical hyaline structures that arise from the peripheral nerve perineurium and project into the endoneurium. Despite their earlier accurate description in the French and German literature, Kernohan and Woltman (1938) reported very similar structures as “nerve infarcts” in a case series of vasculitic neuropathy. Krücke (1955) deserves credit for discovering this error and further explaining how peripheral nerves react differently (from brain parenchyma) to ischemia. We tried to elucidate the reason why Kernohan and Woltman, and others, made this scientific error by describing the historical evolution of our understanding of the structure and function of Renaut corpuscles.

Vasculitic neuropathy is a rare debilitating but treatable peripheral neuropathy. The classic manifestation is a mononeuropathy multiplex pattern of sensorimotor disturbances, but it can also present as a symmetric length-dependent polyneuropathy. Nerve and muscle biopsies are important in the diagnostic workup, with the combination increasing the diagnostic yield. Specific pathologic findings include fibrinoid necrosis and transmural inflammation. Long-term combined immunosuppressive therapy is often required. We present a case of a woman who presented with a subacute onset of progressive painful sensory and motor disturbances without systemic manifestations. Laboratory and pathological findings were confirmatory for a lupus-associated vasculitic neuropathy.

Peripheral neuropathies have a significant population prevalence which is likely to increase in the future. They can impose a significant burden on the quality of life. The use of nerve biopsy is an important diagnostic tool in the assessment of patients with neuropathies. Newer techniques have emerged; reducing the reliance on nerve biopsies to assist with diagnosis. Nonetheless, the information obtained from histopathology may still help to distinguish clinical phenotypes which in turn can potentially modify the clinical management of patients. However, no clear guidelines currently exist to assist clinicians considering nerve biopsies for their patients. During this Master’s project, a comprehensive database was created based on the analysis of clinical and pathological information derived from the medical records of patients who were referred for nerve biopsy. This neuromuscular database was analysed to study the diagnostic utility of nerve biopsies in the clinical workup of selected types of neuropathies. Patients with suspected vasculitic neuropathy were studied to determine the clinical parameters that influenced the presence of neuropathological findings supportive of a diagnosis of vasculitic neuropathy. Stepwise clinical progression, the presence of both sensory and motor features, and asymmetric or multifocal presentation best differentiated systemic and non-systemic vasculitic neuropathy from other conditions. Additionally, parameters that best differentiated systemic vasculitic neuropathy from other conditions included the presence of selected autoantibodies and elevated inflammatory markers. Nerve conduction studies (NCS) in patients with pathologically confirmed vasculitic neuropathy frequently demonstrated a pure axonal neuropathy with sensorimotor abnormalities. In contrast, a diagnostic exclusion of vasculitis was highest in patients with normal NCS, patients with upper limb dominant symptoms, and in patients with cerebrospinal fluid (CSF) pleocytosis or CSF protein above 110mg/dL. Nerve biopsies in patients with symptoms that were purely sensory or chronic and symmetric as well as patients with dominant demyelinating features on NCS were unlikely to demonstrate evidence of vasculitis. Patients with suspected paraproteinaemic neuropathy were studied to determine the clinical parameters that influenced the presence of neuropathological findings supportive of a diagnosis of paraproteinaemic neuropathy. The presence of an IgM paraprotein and absence of an IgG paraprotein best differentiated biopsy findings consistent with paraproteinaemic neuropathy from other diagnoses. Patients with an eventual clinical diagnosis of paraproteinaemic neuropathy were studied separately and it was determined that regardless of whether the nerve biopsy report suggested a diagnosis of paraproteinaemic neuropathy, there was no difference in the prevalence of changes to the management plan or to clinical outcomes. Overall, this thesis has established that in a cohort of patients referred for nerve biopsy, several clinical parameters influenced the pathological outcomes of nerve biopsy. Consequently, decision aids were created for clinicians to assist in deciding whether a referral for nerve biopsy was appropriate.

Monoeuropathies, mononeuropathy multiplex, and other neuropathies are all caused by damage to the peripheral nervous system. Monoeuropathies are characterized by neuropathic pain and motor disturbances in the distribution of one affected nerve. Mononeuropathy multiplex affect nerve trunks that cause dysesthesias and paresthesias and are usually caused by ischemia to the vasa nervorum.

Vaskulitische Neuropathien sind immunvermittelte entzundliche Gefaserkrankungen des peripheren Nervensystems. Diese kommen bei den primaren systemischen Vaskulitiden wie der Panarteriitis nodosa, den ANCA-assoziierten Vaskulitiden (z. B. Eosinophile Granulomatose mit Polyangiitis, Granulomatose mit Polyangiitis, mikroskopische Polyangiitis), der kryoglobulinamischen Vaskulitis sowie bei den sekundaren Vaskulitiden bei Kollagenosen (systemischer Lupus erythematodes, primares Sjogren Syndrom, systemische Sklerose) sowie der rheumatoiden Arthritis, Infektionen, Malignomen oder Medikamenten vor. Die vaskulitische Neuropathie ist bei den genannten Erkrankungen eine haufige Organmanifestation und teilweise syndromdefinierend. Typisch fur die Neuropathie sind eine asymmetrische sensomotorische oder multifokale (Multiplex-Typ), haufig schmerzhafte Polyneuropathie mit einem axonalen Schadigungsmuster durch eine ischamische Nervenschadigung. Letztlich kommen aber alle Polyneuropathiemuster vor, in der Regel mit einem progressiven, teils schubformigen, in der Regel jedoch nicht selbstlimitierendem Verlauf. Die immunologischen Mechanismen sind vielfaltig und fuhren haufig zu einer Vaskulitis der Vasa vasorum der Nerven mit der Notwendigkeit zu einer suffizienten immunsuppressiven Therapie, welche zu einer Besserung der Neuropathie fuhrt.

Introduction: The additional value of peripheral nerve ultrasound in acquired immune-mediated neuropathies has recently been reported. Its impact in vasculitic neuropathy is yet to be defined. We report electrophysiological and nerve ultrasound studies in a patient with nonsystemic vasculitic neuropathy at first diagnosis and in response to immunosuppression. Case Report: A 44-year-old female presented with painful neuropathy and weakness of the intrinsic hand muscles. Electrodiagnostic studies revealed severe axonal neuropathy of the nerves of the left arm. On nerve ultrasound, massive and patchy swelling of these nerves was detected. Clinical, laboratory, and radiological evidence of nonneuromuscular involvement and systemic vasculitic diseases was absent. Hence, nonsystemic vasculitic neuropathy was diagnosed without the possibility of histological verification. After 6 months of systemic immunosuppression with steroids and cyclophosphamide, clinical symptoms improved in parallel with neurosonography. In contrast, electrophysiological studies remained pathological despite clinical improvement. Conclusions: Neurosonography studies in nonsystemic vasculitic neuropathy are rare but might be an ancillary technique to guide noninvasive diagnosis and therapeutic monitoring. Morphological analysis of nerves and changes in response to treatment could be well visualized. Additionally, neurosonography might be useful to target nerve biopsy.

Objective: To describe clinical, electrophysiological, and histopathological profile of vasculitic neuropathy in elderly subjects aged 65 years or more. Design: Retrospective chart review. Setting: Departments of Neurology and Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India. Patients and Methods: Elderly subjects, diagnosed vasculitic neuropathy by nerve biopsy over one decade, were studied. Results: The cohort consisted of 46 subjects. Symptom duration was 21.54 ± 33.53 months. Onset was chronic in majority (82.6%). Key features included paresthesias (89%), weakness (80%), sensory loss (70%), wasting (63%), and relapsing-remitting course (6.5%). Most Common clinico-electrophysiological patterns were distal symmetrical sensorimotor polyneuropathy - 19, mononeuritis multiplex - 9, and asymmetric sensorimotor neuropathy - 10. Diagnosis of vasculitis was not suspected before biopsy in 31 (67.3%). Nerve biopsy revealed definite vasculitis - 12, probable - 10, and possible - 24. Treatment included immunomodulatory agents (41), symptomatic medications only (9), and antiretroviral therapy (1). Twenty-four patients were followed up for mean period of 6.5 months. Outcome at last follow-up was improved (13), unchanged (8), and worsened (3). Conclusion: Vasculitis is an important, treatable cause of neuropathy in elderly. Nerve biopsy should be used judiciously for early diagnosis and appropriate treatment.

Background: We sought to determine the usefulness of sonography in the detection of nerve involvement in patients with vasculitic neuropathy. Methods: We enrolled 16 consecutive patients with vasculitic neuropathy (11 systemic vasculitis and 5 single organ peripheral nerve vasculitis), who met the diagnostic criteria of the Peripheral Nerve Society, and 16 disease controls with noninflammatory axonal polyneuropathy (10 cryptogenic, 4 metabolic, 2 hereditary). Patients underwent standardized nerve conduction studies and assessment of muscle strength (Medical Research Council scale), in addition to sonography of large arm and leg nerves, and brachial plexus. Nerves were evaluated bilaterally at predetermined sites for nerve size (cross-sectional area) and presence of hypervascularization. Results: We found enlarged nerves at common sites of nerve compression in all vasculitic and control patients. Multifocal enlargement in arm nerves, proximal to common sites of nerve compression, was sensitive (94%) and specific (88%) for vasculitic neuropathy. Sonography showed nerve enlargement in 51% of clinically or electrodiagnostically unaffected nerves. Sonography of the brachial plexus was normal. We found hypervascularization in 3 patients with systemic vasculitis. Conclusions: Sonographic enlargement of arm nerves proximal to sites of nerve compression with sparing of the brachial plexus may indicate a pattern characteristic of patients with vasculitic neuropathy. Sonography may represent a sensitive and specific technique for the detection of inflammatory neuropathy. Classification of evidence: This study provides Class III evidence that sonographic enlargement of arm nerves proximal to sites of nerve compression accurately identifies patients with vasculitic neuropathy.

Introduction: We performed a retrospective analysis of the clinical, pathological, and electrophysiological features of 21 cases of Asymptomatic vasculitic neuropathy (AsVN). Methods: Among 270 patients with biopsy-proven vasculitic neuropathy, we identified 21 (7.8%) who had asymptomatic neuropathy. Results: Of the 21 patients with AsVN, 11 were women and 10 were men. Their mean age was 62.5 years. Referring physicians suspected systemic vasculitis on the basis of clinical and laboratory features, but none of the patients had neuropathy by examination. Screening nerve conduction studies identified neuropathy in all patients, leading us to perform a sural nerve biopsy, which confirmed the diagnosis of vasculitis. Twelve patients had active (type I), 6 had inactive (type II), and 3 had probable (type III) vasculitis. Vasculitis was primary in 10 patients and secondary in 11. Conclusions: Nerve conduction study is an important tool for identifying AsVN, a subtype of vasculitic neuropathy. Muscle Nerve 52: 34–38, 2015

Objective Combined nerve/muscle biopsy is widely carried out to improvethe diagnostic sensitivity for vasculitic neuropathy. However, an additionalyield of muscle biopsy is modest. Therefore, we investigated whether skinbiopsy in combination with nerve/muscle biopsy increases the detectionrate of vasculitis.Methods A total of 25 combined nerve/muscle/skin biopsy samples frompatients with biopsy-proven necrotizing vasculitis (n = 16) or clinically prob-able vasculitic neuropathy (n = 9) were reviewed. Specimens from the suralnerve, peroneus brevis muscle and skin were obtained simultaneously by asingle incision.Results Skin biopsy substantially contributed to diagnosis of vasculitic neu-ropathy. Additional skin biopsy enhanced diagnostic sensitivity from 88% to100% in patients with pathologically confirmed vasculitis (n = 16). Vasculitiswas detected only in skin specimens from two of the entire cohort (n = 25).There were no complications related to skin collection, such as anastomoticleakage or wound infection.Conclusion Combined nerve/muscle/skin biopsy could be a less invasiveoption to improve the diagnostic sensitivity for vasculitis.IntroductionVasculitic neuropathy should be considered in thedifferential diagnosis of progressive axonal neuropa-thies with multifocal or asymmetric symptoms, andtimely diagnosis and early treatment before exten-sive Wallerian degeneration can improve the prog-nosis. Peripheral neuropathy can occur

Objective:To investigate the mechanisms of vasculitis in nonsystemic vasculitic neuropathy (NSVN) and microscopic polyangiitis (MPA), focusing on complement- and antineutrophil cytoplasmic antibody (ANCA)-associated pathogenesis.Methods:Sural nerve biopsy specimens taken from twenty-four patients with NSVN and 37 with MPA-associated neuropathy (MPAN) were examined. Twenty-two patients in the MPAN group tested positive for ANCA.Results:Immunostaining for complement component C3d deposition showed more frequent positive staining of epineurial small vessels in NSVN than in MPAN (p = 0.002). The percentages of C3d-positive blood vessels were higher in the NSVN group than those in the ANCA-positive MPAN and ANCA-negative MPAN groups (p = 0.002 and p = 0.009, respectively). Attachment of neutrophils to the endothelial cells of epineurial small vessels was frequently observed in the MPAN groups, irrespective of the presence or absence of ANCA, but was scarce in the NSVN group. Immunohistochemistry using antimyeloperoxidase (MPO) antibodies revealed that the number of MPO-positive cells attached to the endothelial cells of epineurial vessels was lower in the NSVN group than that in the ANCA-positive MPAN and ANCA-negative MPAN groups (p < 0.001 and p = 0.011, respectively).Conclusions:NSVN and MPA have distinct mechanisms of vasculitis. In MPA, the attachment of neutrophils to vascular endothelial cells seems to be an initial lesion of vasculitis, regardless of the presence or absence of ANCA. Complement participated in the pathogenesis of vasculitis in NSVN.

This article discusses the clinical features, pathophysiology, and management of primary and secondary acquired immune axonal neuropathies.Although there are many collagen vascular disorders associated with vasculitic neuropathy, a quarter of cases have been described to be due to nonsystemic vasculitis of the peripheral nervous system. Enhanced surveillance and aggressive treatment of conditions such as cryoglobulin-related vasculitic neuropathy with cyclophosphamide, rituximab, and alfa interferons has led to improved morbidity and mortality, however, many cases of immune axonal acquired neuropathy are still associated with poor outcomes. Acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) are well-characterized variants of Guillain-Barré syndrome.Characterizing the clinical and electrophysiologic phenotype can help diagnose conditions such as nonsystemic vasculitic neuropathy, AMAN, AMSAN, and immune small fiber neuropathy, while careful evaluation of systemic features is key to identifying secondary immune axonal neuropathies such as vasculitic neuropathy related to collagen vascular disease. Additional research is needed to determine the exact immune pathogenesis and optimized treatment regimens for all acquired immune axonal neuropathies.

医学部神経内科学教室 内山真一郎教授退任記念特別号

The nomenclature and classification of vasculitis has been difficult and controversial for many decades. This is problematic for both research on vasculitis as well as clinical care of patients with vasculitis. The first (1994) International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC) proposed names and definitions for the most common forms of vasculitis. Since then, there have been substantial advances in our understanding of vasculitis and changes in medical terminology. In addition, CHCC 1994 did not propose a nomenclature for some relatively common forms of vasculitis, such as vasculitis secondary to other diseases. To address these issues, a second International Chapel Hill Consensus Conference was held in 2012. The goals were to change names and definitions as appropriate, and add important categories of vasculitis not included in CHCC 1994. This overview summarizes the 2012 CHCC and points out the changes compared to the 1994 CHCC. Notable changes include the introduction of new terms such as granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and IgA vasculitis, and the inclusion of categories for variable vessel vasculitis and secondary forms of vasculitis.

A 43-year-old female was referred to our clinic for an electromyographic (EMG) examination due to weakness in both the lower extremities. Conduction blocks were indicated through nerve conduction studies (NCS). Because of demyelinating polyneuropathy findings and elevated protein levels in the cerebrospinal fluid (CSF), the patient was diagnosed with acute inflammatory demyelinating polyneuropathy (AIDP) and was treated with intravenous immunoglobulin. In the follow-up examination, a distal quadriparesis was found that was markedly pronounced in the lower left extremity, and a second EMG examination showed asymmetrical worsening. These findings combined with the lack of demyelination suggested axonal degeneration, and the partial conduction blocks observed during the first NCS were determined to be pseudo-blocks. Vasculitic syndromes were considered and the associated laboratory tests were performed. Following a sural nerve biopsy, non-infectious necrotizing arteritis was found and the patient was diagnosed with polyarteritis nodosa (PAN). Therefore, clinicians should be aware that conduction blocks observed in nerve conduction studies may potentially be pseudo-blocks and an indication of vasculitic neuropathy.

Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) is reported to be highly specific to vasculitis compared to myeloperoxidase (MPO)-ANCA. We report a case of a 19-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) with high PR3-ANCA positivity. The patient responded well to intravenous immunoglobulin plus oral steroid, and showed no signs of systemic vasculitis during the subsequent 10 months of follow-up. Our present case suggests that CIDP may accompany high PR3-ANCA levels, which should be differentiated from axonal neuropathy due to vasculitis.