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Inflammopharmacology 2020 Jun 29. Epub 2020 Jun 29.

Department of Anesthesiology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, 289, Jianguo Rd., Sindian District, New Taipei City, 23142, Taiwan.

Unfortunately, Fig. 5 was incorrectly published in the original publication. The complete corrected Fig. Read More

Ter Arkh 2019 Dec 15;91(12):63-69. Epub 2019 Dec 15.

Clinic of modern rheumatology.

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. Read More

Am J Respir Crit Care Med 2020 Jun 25. Epub 2020 Jun 25.

University of Alberta, Medicine, Edmonton, Alberta, Canada.

An international consensus on anti-neutrophil cytoplasm antibodies (ANCA) testing in eosinophilic granulomatosis with polyangiitis (EGPA) is presented. ANCA, specific for myeloperoxidase (MPO), can be detected in 30-35% of EGPA patients. MPO-ANCA should be tested with antigen-specific immunoassays in any patient with eosinophilic asthma and clinical features suggesting EGPA, including constitutional symptoms, purpura, polyneuropathy, unexplained heart, gastrointestinal or kidney disease, and/or pulmonary infiltrates or hemorrhage. Read More

Rheumatology (Oxford) 2020 May;59(Supplement_3):iii55-iii59

Department of Neurology, Royal Free Hospital, London, UK.

Peripheral neuropathy is a common feature of systemic vasculitis and can also occur when vessel wall inflammation is confined to the vasa nervorum, as a tissue-specific condition-non-systemic vasculitic neuropathy (NSVN). Typically, the clinical picture in both systemic and non-systemic cases is of a lower limb predominant, distal, asymmetric or multifocal neuropathy, which is painful and subacute in onset. For NSVN, nerve biopsy is required to make the diagnosis, and nerve biopsy also has a role when vasculitic neuropathy is suspected and a systemic process has not yet declared itself. Read More

Am J Trop Med Hyg 2020 Apr 13. Epub 2020 Apr 13.

Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.

Identification of DNA by polymerase chain reaction (PCR) is a reliable and an affordable method to confirm leprosy. DNA from 87 nerve samples (56 from paraffin blocks and 26 fresh samples) was extracted. DNA was amplified by PCR from 80/87 (92%) specimens. Read More

Int J Rheum Dis 2020 May 7;23(5):697-701. Epub 2020 Apr 7.

Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, India.

A 57-year old gentleman had presented a year back with inflammatory oligoarthritis and vasculitic neuropathy, diagnosed as unclassifiable vasculitis, initiated on oral corticosteroids and intravenous cyclophosphamide (monthly X 6). His disease stabilized and he had been maintained on azathioprine, which had to be stopped due to acute pancreatitis with subsequent pseudocyst formation, requiring percutaneous drainage suspecting infection. Within a week of pseudocyst drainage, he developed sudden onset pain in left upper limb, with absent left upper limb pulses, loss of motor function of left hand, myocardial ischemia, and extensive thrombosis of the left upper limb arteries. Read More

Internist (Berl) 2020 Mar;61(3):261-269

Friedrich-Baur-Institut, Neurologische Klinik, Ludwig-Maximilians-Universität München, Ziemssenstr. 1, 80336, München, Deutschland.

Vasculitic neuropathies result from inflammation of the vasa nervorum followed by ischemia and destruction of the peripheral nerve. The inflammation can be systemic or localized, i.e. Read More

Medicine (Baltimore) 2020 Jan;99(5):e19036

Department of Neurology, Showa University Fujigaoka Hospital, Fujigaoka Aoba-ku, Yokohama-shi, Kanagawa.

Rationale: Monoclonal gammopathy of undetermined significance (MGUS) is a plasma cell proliferative disorder that consistently precedes multiple myeloma. Peripheral neuropathy in patients with IgG-MGUS tends to vary in clinical phenotype. We report a rare case of a patient with IgG-MGUS who had nonsystemic vasculitic neuropathy (NSVN). Read More

Cureus 2019 Dec 19;11(12):e6423. Epub 2019 Dec 19.

Internal Medicine, John H Stroger Jr Hospital of Cook County, Chicago, USA.

Cryoglobulinemic vasculitis (CV) is a systemic inflammatory syndrome involving small- to medium-sized vessels. Almost half of hepatitis C-infected patients have detectable cryoglobulins levels, but only very few develop clinical manifestations. In this case report, we bring forth a diagnostic challenge of CV. Read More

Front Neurol 2019 22;10:1218. Epub 2019 Nov 22.

Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics and Maternal/Child Sciences, University of Genoa, Genoa, Italy.

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). Read More

BMJ Case Rep 2019 Nov 26;12(11). Epub 2019 Nov 26.

Division of Rheumatology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA

Systemic sclerosis (SSc) is characterised by non-inflammatory vasculopathy, autoimmunity and widespread fibrosis. While the presence of antineutrophil cytoplasmic antibodies (ANCAs) has been reported in SSc, their association with ANCA-associated vasculitis is exceedingly rare. Myeloperoxidase ANCA is more common than proteinase-3 ANCA, and glomerulonephritis is the most common clinical presentation of ANCA-associated vasculitis in SSc. Read More

J Community Hosp Intern Med Perspect 2019 1;9(5):430-432. Epub 2019 Nov 1.

Department of Internal Medicine, Greater Baltimore Medical Center, Towson, Maryland, USA.

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. Vasculitis in the vasa nervorum leads to infarction of peripheral nerves which leads to neuropathy. Read More

Neurol Sci 2020 Feb 24;41(2):341-346. Epub 2019 Oct 24.

Università Cattolica del Sacro Cuore, Rome, Italy.

Introduction: Nerve biopsy has been widely used to investigate patients with peripheral neuropathy, and in many centers, it is still a useful diagnostic tool in this setting. In this study, we reviewed the histopathological spectrum of the nerve biopsies performed in our center in a 30-year period and we analyzed their relevance in the clinical setting.

Materials And Methods: Retrospective analysis of the retrieved data was done for cases of nerve biopsies performed in our institute between 1988 and 2018. Read More

Semin Neurol 2019 10 22;39(5):608-619. Epub 2019 Oct 22.

Department of Neurology, Virginia Commonwealth University, Richmond, Virginia.

Vasculitic neuropathies are disorders that result from inflammation in the peripheral nerves' vascular supply, resulting in ischemic injury. These disorders may be a result of systemic inflammation or may be confined to the peripheral nervous system. Causative etiologies include primary systemic vasculitis, vasculitis secondary to other conditions such as primary connective tissue disorders, infectious, paraneoplastic, and drug-induced conditions, and nonsystemic vasculitic neuropathy. Read More

Presse Med 2019 Sep 26;48(9):948-955. Epub 2019 Sep 26.

CHU de Toulouse, Hôpital Purpan, université de Toulouse, service de médecine interne, Pavillon URM, UMR Inserm 1027, 31059 Toulouse cedex 9, France.

GCA ischemic complications occur generally in patients with a yet undiagnosed or uncontrolled disease. When disease control is fair, ischemic complications may be due mostly to atheromatosis. Ophtalmic complications are most frequent and are dominated by anterior ischemic optic neuropathy. Read More

Neurol Neuroimmunol Neuroinflamm 2019 11 20;6(6). Epub 2019 Sep 20.

From the Immunology Clinic and Department of Neurology (A.B.), Departments of Medicine, Biomedicine and Clinical Research, University Hospital Basel; Department of Rheumatology (V.K.J., T.D.), University Hospital Basel, Switzerland; Department of Neurology (R.D.M.H.), King's College Hospital, London; Nuffield Department of Orthopedics (R.A.L., A.C.), Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, University of Oxford, United Kingdom; Department of Neurology (A.-K.P.), University Hospital Basel & Clinical Neuroimmunology; Department of Biomedicine (A.-K.P.), University of Basel, Switzerland; Division of Rheumatology and Department of Biostatistics, Epidemiology, and Informatics (P.A.M.), University of Pennsylvania, Philadelphia; Department of Rheumatology (R.S.), Auckland District Health Board, New Zealand; and Department of Neurology (M.P.C.), Medical College of Wisconsin, Milwaukee, WI.

Objective: Reported prevalence of vasculitic neuropathy (VN) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is highly variable, and associations with other organ manifestations have not been studied systematically while accounting for diagnostic certainty of VN.

Methods: Data of all patients with AAV within the Diagnostic and Classification criteria for primary systemic VASculitis study were analyzed cross-sectionally. VN was categorized as definite (histology proven), probable (multiple mononeuropathy or nerve biopsy consistent with vasculitis), or possible (all others). Read More

J Clin Neuromuscul Dis 2019 Sep;21(1):1-6

Department of Neurology, UPMC, Pittsburgh, PA.

Hereditary transthyretin amyloidosis (hATTR) is a rare cause of severe neuropathy, typically with progressive sensorimotor and autonomic manifestations. The clinical course is marked by progressive worsening with typical survival of 7-11 years following the onset of symptoms. The phenotype may resemble other types of neuropathy, and dysautonomia may be absent at onset delaying the diagnosis. Read More

Yonsei Med J 2019 Aug;60(8):705-712

Division of Rheumatology, Department of Internal Medicine, and Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Korea.

Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. Read More

Curr Opin Neurol 2019 10;32(5):684-695

Department of Neurology, King's College Hospital, Denmark Hill, London, UK.

Purpose Of Review: 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). Read More

J Assoc Physicians India 2019 Apr;67(4):96-97

Professor,Medical College, Kolkata, West Bengal.

Rheumatoid arthritis (RA) is a multisystem disease with a variety of manifestations. Vasculitis and myositis are two very rare complications of RA. However, the coexistence of both of these complications in the same patient is extremely rare in medical literature. Read More

Rheumatology (Oxford) 2020 01;59(1):251-252

Department of Autoimmune Diseases, Vasculitis Research Unit.

Inflammopharmacology 2019 Aug 22;27(4):713-722. Epub 2019 May 22.

Department of Anesthesiology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, 289, Jianguo Rd., Sindian District, New Taipei City, 23142, Taiwan.

Vasculitic peripheral neuropathy (VPN) is characterized by acute-to-subacute onset of painful sensory and motor disturbances that result from inflammatory obliteration of nerve blood vessels and subsequent ischaemic injury. Endoplasmic reticulum (ER) stress has been implicated in the pathogenesis of various peripheral neuropathies, and 4-phenylbutyric acid (4-PBA) is a chemical chaperone that inhibits ER stress signaling. We investigated the effects of 4-PBA on neuropathic pain associated with VPN induced by ischaemia-reperfusion (IR) and its underlying mechanisms. Read More

Clin Exp Pharmacol Physiol 2019 08 29;46(8):770-779. Epub 2019 May 29.

Department of Anesthesiology, Buddhist Tzu Chi Medical Foundation, Taipei Tzu Chi Hospital, New Taipei City, Taiwan.

Vasculitic peripheral neuropathy (VPN) arises from an inflammatory obstruction in the blood vessels supplying peripheral nerves and subsequent ischaemic insults, which exhibits the clinical features of neuropathic pain and impaired peripheral nerve function. VPN induced by ischaemia-reperfusion (IR) has been reported to involve nuclear factor-κB (NF-κB)-mediated neuroinflammation. Recent studies have suggested that endoplasmic reticulum (ER) stress has been implicated in the development of peripheral neuropathies. Read More

Ann Indian Acad Neurol 2019 Apr-Jun;22(2):180-186

Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.

Objective: Vasculitic neuropathy can be either restricted to the peripheral nerves or associated with systemic involvement of other organs. The objective of this study was to analyze the nerve biopsies reported as "vasculitic neuropathy" with clinical features.

Materials And Methods: All cases diagnosed with vasculitic neuropathy were retrospectively analyzed and categorized as systemic vasculitis and nonsystemic vasculitic neuropathy based on the clinical features. Read More

Neurol Clin 2019 05 18;37(2):303-333. Epub 2019 Mar 18.

Department of Neurology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA. Electronic address:

"The vasculitic neuropathies encompass a wide range of disorders characterized by ischemic injury to the vasa nervorum. Patients with vasculitic neuropathies develop progressive, painful sensory or sensorimotor deficits that are typically multifocal or asymmetric. Depending on the underlying etiology, the vasculitis may be confined to the peripheral nervous system; may be one manifestation of a primary systemic vasculitis; or one manifestation of a systemic vasculitis that is secondary to underlying connective tissue disease, drug exposure, viral infection, or paraneoplastic syndrome. Read More

Muscle Nerve 2019 06 14;59(6):E44-E46. Epub 2019 Mar 14.

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Oxf Med Case Reports 2019 Feb 16;2019(2):omy131. Epub 2019 Feb 16.

Department of Nephrology, St Vincent's Hospital Melbourne, Fitzroy, Victoria, Australia.

A 64-year-old gentleman initially presented with nephrotic syndrome and membranous nephropathy with positive staining for C1q, which was suspicious for lupus membranous nephritis. Investigation led to the simultaneous diagnosis of colorectal cancer (CRC). The CRC was surgically excised and the patient's nephrotic syndrome resolved. Read More

J Neurol 2019 Apr 14;266(4):975-981. Epub 2019 Feb 14.

Department of Radiology, University of Cologne, Cologne, Germany.

Background: Diagnosis and disease monitoring of non-systemic vasculitic neuropathy (NSVN) are based on electrophysiological and clinical measures. However, these methods are insensitive to detect subtle differences of axonal injury. We here assessed the utility of a multiparametric MRI protocol to quantify axonal injury and neurogenic muscle damage in NSVN. Read More

Muscle Nerve 2019 06 23;59(6):643-649. Epub 2019 Feb 23.

Brain and Mind Centre, University of Sydney, Sydney, New South Wales, Australia.

Introduction: To improve diagnostic accuracy, in this study we compared prebiopsy clinical parameters with subsequent pathological confirmation of peripheral nerve vasculitis.

Methods: Clinical, laboratory, and neurophysiological parameters were analyzed for consecutive patients referred for nerve biopsy with suspected vasculitis. Patients were assigned pathological categories of definite, probable, possible, or absent vasculitis using validated guidelines. Read More

Reumatol Clin 2019 May - Jun;15(3):173-178. Epub 2019 Jan 25.

Servicio de Reumatología, Instituto de Investigación Biomédica de Málaga (IBIMA), Hospital Regional Universitario de Málaga, Universidad de Málaga, Málaga, España.

Objective: 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. Read More

Neurol India 2019 Jan-Feb;67(Supplement):S62-S70

Department of Neurochemistry, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.

Aim: To describe the clinical and laboratory findings of patients with biopsy proven vasculitic neuropathy.

Introduction: Peripheral neuropathies form one of the most common disorders of the nervous system. However, more than 50% of them are labelled as 'idiopathic' and, therefore, treatment options become less. Read More

Rinsho Shinkeigaku 2018 Dec 29;58(12):745-749. Epub 2018 Nov 29.

Department of Neurology, Seirei Mikatahara General Hospital.

We present the unique case of a patient with lumbosacral radiculoplexus neuropathy (LRPN) and a small intramedullary lesion, probably an infarction, in the spinal cord. A previously healthy 58-year-old female developed acute severe pain involving the entire right lower limb, followed by weakness of the right lower limb a few hours later. The patient could not walk and was referred to our clinic. Read More

Curr Opin Rheumatol 2019 01;31(1):40-45

Division of Rheumatology, Department of Medicine, University of California, San Francisco, San Francisco, California, USA.

Purpose Of Review: Vasculitis of medium-sized and small vessels commonly affects peripheral nerves and can occur in context of a systemic vasculitis with multiorgan involvement or a nonsystemic vasculitis limited to the peripheral nervous system. This review summarizes the clinical and pathological features of systemic and nonsystemic vasculitis of the peripheral nervous system.

Recent Findings: Vasculitis of peripheral nerves is a diffuse process that affects the vasa nervorum along the entire length of affected nerves but appears to cause injury primarily in a zone in the proximal-middle of the nerve that is particularly susceptible to ischemic injury. Read More

BMC Pulm Med 2018 Nov 19;18(1):170. Epub 2018 Nov 19.

Centre for Experimental Medicine, Queen's University Belfast, 97 Lisburn Road, Belfast, BT9 7BL, Northern Ireland.

Background: Non-Tuberculous Mycobacterial-pulmonary disease (NTM-PD) is increasing in incidence and prevalence. Mycobacterium abscessus (M.abscessus) is a rapid growing multi-resistant NTM associated with severe NTM-PD requiring prolonged antibiotic therapy. Read More

Fortschr Neurol Psychiatr 2018 09 24;86(9):566-574. Epub 2018 Sep 24.

Friedrich-Baur-Institut, Neurologische Klinik und Poliklinik, Klinikum der LMU München.

This paper is a practical survey of immune-mediated, inflammatory and hereditary neuropathies along with recommendations for diagnostic procedures. The large group of immune-mediated, inflammatory neuropathies includes the Guillain-Barré syndrome and chronic-inflammatory demyelinating polyradiculoneuropathy and their subtypes, vasculitic, paraneoplastic and paraproteinemic neuropathies as well as neuropathies resulting from connective tissue disorders. Besides clinical features such as time-dependent progression and distribution of sensorimotor deficits, characteristic electroneurographic findings and antibody profiles are considered. Read More

BMJ Case Rep 2018 Sep 19;2018. Epub 2018 Sep 19.

Hull and East Yorkshire Hospitals NHS Trust, Hull, UK.

A retired businessman presented to the infectious diseases department with a history of ongoing fevers and myalgia and raised inflammatory markers. This continued despite adequate antibiotic treatment of an epididymo-orchitis. Extensive investigations, including bone marrow and liver biopsies and a positron emission tomography, did not reveal a cause but showed reactive change in the bone marrow. Read More

Mayo Clin Proc Innov Qual Outcomes 2017 Sep 1;1(2):192-197. Epub 2017 Sep 1.

Department of Neurology, Mayo Clinic, Rochester, MN.

Recently, guidelines have been outlined for management of immune-related adverse events occurring with immune checkpoint inhibitors in cancer, irrespective of affected organ systems. Increasingly, these complications have been recognized as including diverse neuromuscular presentations, such as demyelinating and axonal length-dependent peripheral neuropathies, vasculitic neuropathy, myasthenia gravis, and myopathy. We present 2 cases of brachial plexopathy developing on anti-programmed cell death-1 checkpoint inhibitor therapies (pembrolizumab, nivolumab). Read More

Indian J Pathol Microbiol 2018 Jul-Sep;61(3):339-344

Department of Pathology, Institute of Human Behaviour and Allied Sciences, New Delhi, India.

Background: Nerve biopsy has been widely used to investigate patients with peripheral neuropathy and in many centers, it is still a part of the diagnostic armamentarium. In this study, the histopathological spectrum of the nerve biopsies received is being revisited to analyze the various clinical and pathologic features and also to assess their relevance.

Materials And Methods: Retrospective analysis of the data retrieved was done for 74 cases of nerve biopsies. Read More

J Peripher Nerv Syst 2018 09 24;23(3):174-177. Epub 2018 Jul 24.

Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy.

Neurofilament light chain (NFL) levels reflect axonal damage in different inflammatory and neurodegenerative central nervous system conditions, in correlation with disease severity. Our aim was to determine the possible diagnostic and prognostic value of serum and cerebrospinal fluid (CSF) NFL levels in subjects with different forms of acquired peripheral neuropathies (PN). Paired serum and CSF samples of 25 patients with acquired PN were analysed for NFL using an ultrasensitive technique (Quanterix, Simoa, Lexington, MA, USA) and compared with a group of 25 age-matched healthy subjects. Read More

Oman Med J 2018 May;33(3):253-255

Department of Internal Medicine, Government Medical College Kozhikode, Kerala, India.

Necrotizing vasculitic neuropathy in polyarteritis nodosa can rarely present acutely and may mimic acute inflammatory neuropathies. A 53-year-old male presented with an acute neurological illness characterized by paresthesia and weakness of both lower limbs lasting six-days. He also had mild paresthesia of both hands. Read More

Pain Physician 2018 03;21(2):111-120

Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Foundation Trust, UK.

Background: Neuropathic pain (NP) is a common symptom caused by lesions or diseases of the somatosensory nervous system. Acute/subacute peripheral neuropathies (APN) are rare, however can be particularly painful.

Objectives: The aim of this systematic review was to estimate the incidence of NP in APN and overview the various etiologies of such neuropathies. Read More

J Clin Neuromuscul Dis 2018 Mar;19(3):138-141

Department of Neurology, Lahey Hospital and Medical Center, Burlington, MA.

We describe 2 patients presenting with multiplex mononeuritis, associated with skin manifestation, secondary to minocycline-induced vasculitis. One of the cases is associated neither with lupus nor polyarteritis nodosa. An extensive laboratory workup ruled out any possible underlying immunologic disorder. Read More

Allergy Asthma Clin Immunol 2017 13;13:49. Epub 2017 Dec 13.

Department of Internal Medicine, IJsselland Hospital, Capelle aan den IJssel, The Netherlands.

Background: Although influenza vaccines are generally safe and effective, a variety of autoimmune phenomena have been reported after vaccination over the past years, such as Guillain-Barre syndrome, rheumatoid arthritis, pemphigus vulgaris, psoriasis, giant cell arteritis and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV).

Case Report: We describe the case of a 67-year old man who presented with a myeloperoxidase-ANCA associated vasculitis with renal involvement and mononeuritis multiplex after seasonal influenza vaccination. He was initially treated with intravenous cyclophosphamide and high-dose prednisolone followed by maintenance treatment consisting of azathioprine and prednisolone. Read More

J Ultrasound Med 2018 Jun 21;37(6):1565-1574. Epub 2017 Nov 21.

Department of Neurology and Clinical Neurophysiology, Radboud University Medical Center, Donders Center for Neuroscience, Nijmegen, the Netherlands.

The differential diagnosis of upper extremity mononeuritis multiplex includes neuralgic amyotrophy, vasculitic neuropathy, and Lewis-Sumner syndrome. We describe 3 patients initially suspected of neuralgic amyotrophy, who had an extremely painful, protracted, progressive disease course, not fitting one of these established diagnoses. Nerve ultrasonography showed focal caliber changes of the roots, plexus, and limb nerves. Read More

Autoimmun Rev 2018 Jan 3;17(1):73-77. Epub 2017 Nov 3.

Department of Rheumatology and Lupus, Guy's and St. Thomas' NHS Foundation Trust, London, UK. Electronic address:

Objectives: We analysed the spectrum of clinical manifestations of cocaine associated pseudovasculitis.

Methods: Clinical, serological, radiological and histological features of 14 patients with cocaine pseudovasculitis syndromes were included.

Results: Twelve patients had significant sinus thickening or erosive disease. Read More

Neurologist 2017 Nov;22(6):234-236

2nd Neurology Department, AHEPA University Hospital, Thessaloniki, Greece.

X-linked Charcot-Marie-Tooth disease (CMT 1X) is the second most common form of inherited demyelinating neuropathy. It is established that patients suffering from CMT 1X can have episodes of hemiparesis, paraparesis, quadriparesis, ataxia, aphasia, and dysarthria, which can be fully reversible, and 'trigger' factors for these episodes are usually febrile illness, high altitudes, hyperventilation, and physical activity. We describe a 22-year-old patient with a history of viral infection and sleep deprivation who presented to our department because of acute difficulty in walking and neurophysiological findings suggesting Guillain-Barre syndrome. Read More

Neurol Neuroimmunol Neuroinflamm 2017 Nov 23;4(6):e407. Epub 2017 Oct 23.

Department of Neurology (M.T., H.K., S.I., Y.K., M.I., A.H., M.K., G.S.), and Research Division of Dementia and Neurodegenerative Disease (G.S.), Nagoya University Graduate School of Medicine, Japan.

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. Read More

Nat Rev Neurol 2017 Oct 15;13(10):599-611. Epub 2017 Sep 15.

Weill Cornell Medicine-Qatar, Education City, PO Box 24144, Doha, Qatar.

Distal symmetric polyneuropathy (DSPN) is the most common neuropathy to occur in diabetes mellitus. However, patients with diabetes can also develop inflammatory neuropathies, the most common and most treatable of which is chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Whether diabetes is a risk factor for CIDP remains under debate. Read More

JAMA Neurol 2017 10;74(10):1216-1222

Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Importance: Neurological complications are an increasingly recognized consequence of the use of anti-programmed death 1 (PD-1) antibodies in the treatment of solid-organ tumors, with an estimated frequency of 4.2%. To date, the clinical spectrum and optimum treatment approach are not established. Read More

Ann Rheum Dis 2018 07 25;77(7):1093-1094. Epub 2017 Jul 25.

Clinic for Rheumatology, Department of Medicine, University Hospital Basel, Basel, Switzerland.