Guillain-Barre Syndrome Publications (7701)

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Guillain-Barre Syndrome Publications

2015Sep
Neurosurg Focus
Neurosurg Focus 2015 Sep;39(3):E13
Departments of 1 Neurologic Surgery.

Intraneural perineurioma is a rare, benign slow-growing lesion arising from the perineurial cells that surrounds the peripheral nerve fibers. Typically it presents during childhood and young adulthood as a motor mononeuropathy. MRI plays an essential role in the diagnosis and localization of the lesion, which appears as a fusiform enlargement of the nerve fascicles that enhances intensely with gadolinium. Read More

Despite the typical clinical and radiological features, intraneural perineurioma remains largely underdiagnosed because of the lack of familiarity with this entity, but also as a result of technical limitations with conventional MRI that is typically performed as a screening test over a large field of view and without contrast sequences. The purpose of this article is to present the pitfalls and pearls learned from years of experience in the diagnosis and management of this relatively rare condition. Clinical suspicion and detailed neurological examination followed by high-quality electrophysiological studies (EPS) must lead to an adequate preimaging localization of the lesion and narrowing of the imaging area. The use of high-resolution (3-T) MRI combined with gadolinium administration will allow adequate visualization of the internal anatomy of the nerve and help in differentiating other causes of neuropathy. In cases where the lesion is not recognized but clinical suspicion is high, possible errors must be assessed, including the EPS localization, area of imaging, MRI resolution, and slice thickness.

2015Aug
Brain Dev.
Brain Dev 2015 Aug 28. Epub 2015 Aug 28.
Division of Child Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110029, India. Electronic address: sheffalig@yahoo.com.
2015Aug
Muscle Nerve
Muscle Nerve 2015 Aug 28. Epub 2015 Aug 28.
Brain & Mind Research Institute, University of Sydney, Sydney, Australia.

Bifacial weakness with paraesthesias (BFP) is a subtype of Guillain-Barré syndrome defined by rapidly progressive bilateral facial weakness in the absence of other cranial neuropathies, ataxia or limb weakness. Many patients also complain of distal limb paraesthesias and display diminished or absent deep tendon reflexes. BFP represents a localized form of Guillain-Barré syndrome and is thought to be exclusively caused by demyelinating rather than axonal type neuropathy. Read More

Patients with BFP do not display anti-ganglioside antibodies. Although rare, many physicians are unfamiliar with BFP as bilateral facial weakness is more commonly associated with sarcoidosis, Lyme disease or meningeal pathology. Many patients diagnosed with bilateral Bell's palsy may instead have BFP. In this review, we highlight the clinical features of BFP and outline diagnostic criteria. This article is protected by copyright. All rights reserved.

2015Aug
Int. Immunopharmacol.
Int Immunopharmacol 2015 Aug 23. Epub 2015 Aug 23.
Department of Neurology, Affiliated Hospital of Xuzhou Medical College, Xuzhou, Jiangsu, China. Electronic address: zy2003njmu@163.com.
2015Sep
J Clin Neuromuscul Dis
J Clin Neuromuscul Dis 2015 Sep;17(1):27-9
*Department of Neurology, Duke University Hospital, Durham, NC; †Department of Neurology, State University of New York/Upstate Medical University, Syracuse, NY; ‡Department of Neurology, Aurora Neuroscience Innovation Institute, St. Luke's Medical Center, Milwaukee, WI; and §Department of Neurology, Cooper Medical School of Rowan University, Camden, NJ.

We describe a patient with acute progressive weakness and areflexia. Both botulism and Miller-Fisher variant of Guillain-Barré syndrome were initial diagnostic considerations, and she was treated with intravenous immunoglobulin and botulinum antitoxin. A mouse bioassay was positive for botulinum toxin A, although her clinical course, electrodiagnostic studies, and cerebrospinal fluid findings supported Miller-Fisher syndrome. Read More

This patient's atypical features offer points of discussion regarding the evaluation of patients with acute neuromuscular weakness and emphasize the limitations of the botulism bioassay.

2015Aug
Acta Neurol Belg
Acta Neurol Belg 2015 Aug 23. Epub 2015 Aug 23.
Universitair Ziekenhuis Gent, Ghent, Belgium, tine.vandenbroucke@ugent.be.
2015Aug
Neurology
Neurology 2015 Aug 21. Epub 2015 Aug 21.
From the Nuffield Department of Clinical Neurosciences (Y.H., M.R., B.L., M.L., A.V.), John Radcliffe Hospital, University of Oxford; Children's Neurosciences (R.S., M.L.), Evelina London Children's Hospital at Guy's and St Thomas' NHS Foundation Trust, King's Health Partners Academic Health Science Centre, London; and Department of Paediatric Neurology (C.H.), Great Ormond Street Hospital for Children, London, UK. angela.vincent@ndcn.ox.ac.uk.

To assess the clinical and immunologic findings in children with voltage-gated potassium channel (VGKC)-complex antibodies (Abs).
Thirty-nine of 363 sera, referred from 2 pediatric centers from 2007 to 2013, had been reported positive (>100 pM) for VGKC-complex Abs. Medical records were reviewed retrospectively and the patients' condition was independently classified as inflammatory (n = 159) or noninflammatory (n = 204). Read More

Positive sera (>100 pM) were tested/retested for the VGKC-complex Ab-positive complex proteins LGI1 and CASPR2, screened for binding to live hippocampal neurons, and 12 high-titer sera (>400 pM) tested by radioimmunoassay for binding to VGKC Kv1 subunits with or without intracellular postsynaptic density proteins.
VGKC-complex Abs were found in 39 children, including 20% of encephalopathies and 7.6% of other conditions (p = 0.001). Thirty children had inflammatory conditions and 9 had noninflammatory etiologies but titers >400 pM (n = 12) were found only in inflammatory diseases (p < 0.0001). Four sera, including from 2 children with coexisting NMDA receptor Abs and one with Guillain-Barré syndrome and Abs to both LGI1 and CASPR2, bound to hippocampal neurons. None of the sera bound detectably to VGKC Kv1 subunits on live HEK cells, but 4 of 12 >400 pM sera immunoprecipitated VGKC Kv1 subunits, with or without postsynaptic densities, extracted from transfected cells.
Positive VGKC-complex Abs cannot be taken to indicate a specific clinical syndrome in children, but appear to be a nonspecific biomarker of inflammatory neurologic diseases, particularly of encephalopathy. Some of the Abs may bind to intracellular epitopes on the VGKC subunits, or to the intracellular interacting proteins, but in many the targets remain undefined.

2015Aug
BMC Neurol
BMC Neurol 2015 21;15:147. Epub 2015 Aug 21.
Division of Health and Social Care, King's College London, London, SE1 1UL, UK. jane.petty@kcl.ac.uk.

Inflammatory neuropathies such as Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and paraproteinaemic demyelinating neuropathy are a heterogenous group of peripheral nerve disorders that affect around one to two people per 100,000. Whilst treatments such as intravenous immunoglobulin, plasma exchange and corticosteroids have generally positive results, long-term residual symptoms and associated activity limitations are common. There is currently no standardised care for patients with ongoing activity limitation and participation restriction as a result of inflammatory neuropathy IN but data from observational studies and a randomised controlled trial suggest that exercise either alone or as part of a multidisciplinary rehabilitation programme may be beneficial in improving activity limitation. Read More

Tailoring the intervention for participants following physiotherapy assessment and incorporating patient preference for type and location of exercise may be important.
The current study is a pragmatic, prospective, parallel observer-blind, randomised controlled trial to evaluate the efficacy and cost-effectiveness of a twelve week tailored home exercise programme versus advice and usual care. Seventy adults with stable immune mediated inflammatory neuropathy IN will be recruited to the study from two main sources: patients attending selected specialist peripheral nerve clinics in the South East and West Midlands of England and people with who access the GAIN charity website or newsletter. Participants will be randomised to receive either advice about exercise and usual care or a 12 week tailored home exercise programme. The primary outcome of activity limitation and secondary outcomes of fatigue, quality of life, self-efficacy, illness beliefs, mood and physical activity will be assessed via self-report questionnaire at baseline, 12 weeks and 12 months post intervention. Cost effectiveness and cost utility will be assessed via interview at baseline and 12 months post intervention. Intention to treat analysis will be our primary model for efficacy analysis. Semi-structured interviews will be conducted with a selected sample of participants in order to explore the acceptability of the intervention and factors affecting adherence to the exercise programme.
This is the first randomised controlled trial to compare the efficacy and cost-effectiveness of tailored home exercise with advice about exercise and usual care for adults with inflammatory neuropathy.
Current Controlled Trials ISRCTN13311697.

2015Aug
Cell. Mol. Neurobiol.
Cell Mol Neurobiol 2015 Aug 21. Epub 2015 Aug 21.
Department of Neurology, Hospital of Chongqing Armed Police Force, Chongqing, 404000, China.

Wnt signaling has been implicated in developmental and regenerative myelination of the CNS and PNS. The present translational investigation was undertaken to assess whether a soluble factor like Wnt may be responsible for the critically important skeletal muscle neuromuscular junction-Schwann cell communication. Specifically, three key aspects were examined: (a) whether the expression of Daam2, disheveled-associated activator of morphogenesis, a key Wnt signaling downstream effector, and PIP5K is changed in the demyelinating conditions and under different stages of progress of clinical recovery of patients with Guillain-Barre syndrome; (b) whether critical protein interactions of Daam2 with disheveled and Arf6 are changed; and (c) whether expression of c-Jun/Krox, a key negative regulator of remyelination, is changed. Read More

Daam2 was elevated in acute presentation in GB syndrome. Reduction occurred with clinical improvement of the patients. With progressive clinical improvement, c-Jun/Krox expression significantly reduced with time. Wnt signaling likely causes immediate early gene activation and transcriptional shutdown of factors critical for formation and maintenance of myelination. Whether the findings of the present study are specific to pathophysiology of demyelination in acute infectious polyradiculopathy and multiple sclerosis or a generalized aspect of demyelinating diseases merits to be examined in future studies.

2015Aug
Rinsho Shinkeigaku
Rinsho Shinkeigaku 2015 Aug 18. Epub 2015 Aug 18.
Department of Neurology and Stroke Treatment, Kyoto First Red Cross Hospital.

A 49-year-old woman was admitted to our hospital with gradually progressive weakness of the limbs for about 20 days. She presented with weakness of the limbs, predominantly in the proximal portion, and slight dysesthesia of the limbs, predominantly in the distal portion. Repeated nerve conduction examination revealed axonopathy dominantly in the motor neurons. Read More

Therefore, we suspected her as having Guillain-Barré syndrome, and initiated intravenous administration of high-dose immunoglobulin. However, her symptoms progressed gradually and finally she found it difficult to walk. Her urine analysis simultaneously demonstrated albuminuria, and a kidney biopsy indicated focal segmental glomerulosclerosis. At that point, laboratory examination showed high levels of anti SS-A antibody and salivary gland biopsy revealed infiltration of a significant number of lymphocytes around the gland, which led to the diagnosis of Sjögren's syndrome. We considered the etiology of the neural and renal dysfunction as due to the inflammatory mechanism associated with Sjögren's syndrome. Therefore, we administered a second course of immunoglobulin therapy and steroid therapy, which included both pulse and oral administration. Her neurologic symptoms and albuminuria improved rapidly after steroid therapy. The present case indicates that both motor dominant neuropathy and focal segmental glomerulosclerosis can occur in patients with Sjögren's syndrome.

2015Aug
J. Neurol. Sci.
J Neurol Sci 2015 Aug 7. Epub 2015 Aug 7.
Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya, Malaysia.

Miller Fisher syndrome is characterised by the triad of ophthalmoplegia, ataxia and areflexia. However, facial palsy can occur during the course of the illness although development of facial palsy when other cardinal signs of Miller Fisher syndrome have reached nadir or improving, is unusual. This delayed appearance of facial palsy can be easily overlooked by the treating clinician. Read More

Here, we report four patients with Miller Fisher syndrome and delayed-onset facial palsy. We discuss the possible underlying reasons behind the delay in facial palsy.

2015Aug
Leuk Res Rep
Leuk Res Rep 2015 17;4(2):42-4. Epub 2015 Jul 17.
Hematology-Oncology Department, Hotel Dieu de France Hospital, Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.

Central nervous system (CNS) relapse is not a rare presentation in acute myeloid leukemia (AML) as its incidence ranges between 2% and 9%. It manifests with meningeal leukemia, cranial nerve palsies or cerebral mesenchymal myeloid sarcoma. We herein report the case of a 69 year-old female that presented a pseudo-Guillain-Barré syndrome masking an AML CNS relapse. Read More

Her symptoms completely resolved upon administration of a tailored treatment. This case suggests that puzzling neurological manifestations in patients with a history of AML should be considered as a CNS recurrence and investigated accordingly even in the context of normal imaging findings.

2015Aug
Acta Neuropathol.
Acta Neuropathol 2015 Aug 12. Epub 2015 Aug 12.
Neuromuscular Immunopathology Research Laboratory, Division of Neuromuscular Disease, Department of Neurology, The University of Alabama at Birmingham, 1720 7th Avenue South, Sparks Center 200, Birmingham, AL, 35294-0017, USA, ubogu@uab.edu.

Inflammatory neuropathies encompass groups of heterogeneous disorders characterized by pathogenic immune-mediated hematogenous leukocyte infiltration of peripheral nerves, nerve roots or both, with resultant demyelination or axonal degeneration or both. Inflammatory neuropathies may be divided into three major disease categories: Guillain-Barré syndrome (particularly the acute inflammatory demyelinating polyradiculoneuropathy variant), chronic inflammatory demyelinating polyradiculoneuropathy and nonsystemic vasculitic neuropathy (or peripheral nerve vasculitis). Despite major advances in molecular biology, pathology and genetics, the pathogenesis of these disorders remains elusive. Read More

There is insufficient knowledge on the mechanisms of hematogenous leukocyte trafficking into the peripheral nervous system to guide the development of specific molecular therapies for immune-mediated inflammatory neuropathies compared to disorders such as psoriasis, inflammatory bowel disease, rheumatoid arthritis or multiple sclerosis. The recent isolation and characterization of human endoneurial endothelial cells that form the blood-nerve barrier provides an opportunity to elucidate leukocyte-endothelial cell interactions critical to the pathogenesis of inflammatory neuropathies at the interface between the systemic circulation and peripheral nerve endoneurium. This review discusses our current knowledge of the classic pathological features of inflammatory neuropathies, attempts at molecular classification and genetic determinants, the utilization of in vitro and in vivo animal models to determine pathogenic mechanisms at the interface between the systemic circulation and the peripheral nervous system relevant to these disorders and prospects for future potential molecular pathology biomarkers and targets for specific therapeutic intervention.

2015Aug
Int J Clin Exp Pathol
Int J Clin Exp Pathol 2015 1;8(6):7614-6. Epub 2015 Jun 1.
Department of Implantology, Fonazione Policlinico IRCCS Cà Granda, University of Milan University of Milan School of Dentistry MI, IT.
2015Aug
Vestn. Akad. Med. Nauk SSSR
Vestn Ross Akad Med Nauk 2015 (2):183-7
2015Jul
Clin Neurophysiol
Clin Neurophysiol 2015 Jul 17. Epub 2015 Jul 17.
Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia. Electronic address: nortina7@gmail.com.
2015Jul
J. Autoimmun.
J Autoimmun 2015 Jul 27. Epub 2015 Jul 27.
Department of Bacteriology and Immunology, Haartman Institute, University of Helsinki, Helsinki, Finland; Research Program Unit, Immunobiology, University of Helsinki, Helsinki, Finland. Electronic address: seppo.meri@helsinki.fi.

Following the mass vaccinations against pandemic influenza A/H1N1 virus in 2009, a sudden increase in juvenile onset narcolepsy with cataplexy (NC) was detected in several European countries where AS03-adjuvanted Pandemrix vaccine had been used. NC is a chronic neurological disorder characterized by excessive daytime sleepiness and cataplexy. In human NC, the hypocretin-producing neurons in the hypothalamus or the hypocretin signaling pathway are destroyed by an autoimmune reaction. Read More

Both genetic (e.g. HLA-DQB1*0602) and environmental risk factors (e.g. Pandemrix) contribute to the disease development, but the underlying and the mediating immunological mechanisms are largely unknown. Influenza virus hemagglutinin is known to bind gangliosides, which serve as host cell virus receptors. Anti-ganglioside antibodies have previously been linked to various neurological disorders, like the Guillain-Barré syndrome which may develop after infection or vaccination. Because of these links we screened sera of NC patients and controls for IgG anti-ganglioside antibodies against 11 human brain gangliosides (GM1, GM2, GM3, GM4, GD1a, GD1b, GD2, GD3, GT1a, GT1b, GQ1b) and a sulfatide by using a line blot assay. Samples from 173 children and adolescents were analyzed: 48 with Pandemrix-associated NC, 20 with NC without Pandemrix association, 57 Pandemrix-vaccinated and 48 unvaccinated healthy children. We found that patients with Pandemrix-associated NC had more frequently (14.6%) anti-GM3 antibodies than vaccinated healthy controls (3.5%) (P = 0.047). Anti-GM3 antibodies were significantly associated with HLA-DQB1*0602 (P = 0.016) both in vaccinated NC patients and controls. In general, anti-ganglioside antibodies were more frequent in vaccinated (18.1%) than in unvaccinated (7.3%) individuals (P = 0.035). Our data suggest that autoimmunity against GM3 is a feature of Pandemrix-associated NC and that autoantibodies against gangliosides were induced by Pandemrix vaccination.

2015Aug
Immunol. Invest.
Immunol Invest 2015 ;44(6):566-77
Department of Neurology, Xiangya Hospital, Central South University , Changsha, Hunan , PR China .
2015Jul

To investigate cross-sectional areas (CSAs) of several peripheral nerves including the vagus nerve and the diameter of spinal nerves as measured by nerve ultrasound (NUS) and nerve conduction studies (NCS) in Guillain-Barré syndrome (GBS) patients over at least six months compared to healthy controls.
NUS and/or NCS of several nerves, the vagus nerve, and the 5th/6th cervical spinal nerves were performed in patients with GBS at days 2-3 after symptom onset, at days 10-14 after immunoglobulin therapy and after six months compared to healthy controls.
27 GBS-patients and 31 controls were included. Read More

Using NUS significant enlargement was found in all measured nerves (P<0.001), except the sural nerve (P=0.086) compared to the controls at onset. The vagus (median 3.0mm(2) vs. 2.0mm(2), P<0.0001) and the cervical spinal nerves were significantly enlarged (median 3.5/4.0mm vs. 2.6/3.2mm, p<0.0001), the vagus most obviously in patients with autonomic dysregulation (AD, 4.0mm(2)). Six months later, NCS showed persisting pathology in CMAP-amplitudes with amelioration of F-wave pathology. NUS showed restitution in the spinal nerves (median 2.6/3.2mm) and the vagus (median 2.0mm(2)) in all patients excluding the vagus in those with persistent AD (median 4.0mm(2)). The peripheral nerves did not change significantly (P>0.05).
Ultrasonographic detection of cervical spinal nerve enlargement supports the diagnosis of GBS in the early phase. Its regression may be a good parameter for the clinical restitution over time. Vagus enlargement may be a risk marker for development of AD.
Ultrasound is a reliable diagnostic follow-up tool in early GBS.

2015Sep
Expert Opin Drug Saf
Expert Opin Drug Saf 2015 Sep 28;14(9):1387-94. Epub 2015 Jul 28.
a 1 The Zabludowicz Center for Autoimmune Diseases, Chaim Sheba Medical Center , Tel-Hashomer 52621, Israel +972 3 5302652 ; +972 3 5352855 ; shoenfel@post.tau.ac.il.

With the safety of human papilloma virus vaccine (HPVv) being questioned, this article aims to assess the risks and benefits of the commercially available HPVv. Within the last decade, two vaccines (Gardasil and Cervarix) have been put on the market to prevent infection with the most oncogenic HPV subtypes. Both vaccines contain aluminum adjuvants that are meant to cause a hyper stimulated immune response to prevent HPV infection. Read More


The purpose of this paper is to consider the safety of these two vaccines based on the data from the U.S. Vaccine Adverse Event Reporting System (VAERS) and case reports.
The current HPVv are both effective and generally safe. However, it should be noted that autoimmune side effects have been reported in several studies. Further research should be done to understand the relationship between HPVv and autoimmunity.

Posterior reversible encephalopathy syndrome (PRES) is a rare occurrence in patients with Guillain-Barré syndrome (GBS). Two patients with neuroradiological evidence of PRES without central nervous system (CNS) symptoms were recently reported. We present the case of a GBS patient with minimal CNS symptoms and magnetic resonance imaging findings consistent with PRES. Read More

PRES in GBS might be an underestimated condition. It should be suspected in GBS patients in the presence of even mild CNS symptoms. A timely PRES diagnosis along with early correction of autonomic system dysfunction in GBS patients is recommended to prevent possible dangerous CNS complications.

2015Jul
Clin Neurophysiol
Clin Neurophysiol 2015 Jul 17. Epub 2015 Jul 17.
Neurology, Yong Loo Lin School of Medicine, National University Singapore, 5 Lower Kent Ridge Road, Singapore 119074, Singapore.
2015Aug
J. Clin. Virol.
J Clin Virol 2015 Aug 23;69:156-64. Epub 2015 Jun 23.
Internal Medicine Department, Grenoble University Hospital, France.
2015Jul
PLoS ONE
PLoS One 2015 23;10(7):e0131374. Epub 2015 Jul 23.
Department of Neurology, Shanghai Pudong Hospital, Fudan University Pudong Medical Center, 2800 Gongwei Road, Shanghai 201399, China.
2015Jul
PLoS ONE
PLoS One 2015 22;10(7):e0133520. Epub 2015 Jul 22.
Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China.

The proportion of different subtypes of Guillain-Barré syndrome (GBS) and their prognosis varied significantly among different regions. This study attempts to investigate the clinical subtypes and outcome of GBS in southwest China. Patients with GBS admitted to The First Affiliated Hospital of Chongqing Medical University from January 2006 to March 2013 were included in our study. Read More

Patients were classified into acute inflammatory demyelinating polyneuropathy (AIDP) group, acute motor axonal neuropathy (AMAN) group, Miller-Fisher syndrome (MFS) group, cranial nerve variants(CNV), Bickerstaff's brainstem encephalitis overlaps with GBS (BBE-GBS) group and unclassifiable group based on clinical features and electrophysiological findings. Hughes function grade score (HFGS) was used to assess the prognosis at 3 and 6 months. The prognosis of different subtypes and outcome predictors were analyzed. The most common subtype of GBS was AIDP (57%), followed by AMAN (22%) and MFS (7%). The prognosis of AMAN and BBE-GBS is similar at 3 month(P = 0.0704)and 6 month (P = 0.1614) follow-up. The prognosis of AMAN group was poorer than that of AIDP group at 3 month and 6 month follow-up (P<0.001). Outcome of MFS group and that of CNV group at 6 months were both good(Hughes≤1). Hughes≥3(P<0.0001,OR = 6.650,95%CI = 2.865 to 15.023))and dysautonomia (P = 0.043,OR = 2.820,95%CI = 1.031 to 7.715)) were associated with poor outcome at 6 month follow-up. AIDP is the most common subtype of GBS. Prognosis of AMAN group and BBE-GBS group is poorer than that of AIDP group at 3 month and 6 month follow-up. Hughes≥3 at nadir and dysautonomia are predictors of poor prognosis at 6 month follow-up.

2015Jul
Case Rep Neurol Med
Case Rep Neurol Med 2015 23;2015:981439. Epub 2015 Jun 23.
Servicio de Neurologia, Hospital Universitario "Dr. José E. González" y Facultad de Medicina, Universidad Autónoma de Nuevo León, Madero y Gonzalitos, S/N, 64460 Monterrey, NL, Mexico.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) which commonly affects the peripheral nervous system. A 38-year-old female with a history of asthma presented with a 2-week history of bilateral lower extremity paresthesias that progressed to symmetric ascending paralysis. Nerve conduction studies could not rule out Guillain-Barre syndrome (GBS) and plasmapheresis was considered. Read More

Her blood work revealed marked eosinophilia (>50%), she had purpuric lesions in her legs, and a head magnetic resonance image showed evidence of pansinusitis. Coupled with a history of asthma we suspected EGPA-associated neuropathy and started steroid treatment. The patient showed rapid and significant improvement. ANCAs were later reported positive. ANCA-associated vasculitides present most often as mononeuritis multiplex, but they can mimic GBS and should always be considered in the differential diagnosis, since the treatment strategies for these conditions are radically different.

2015Aug
J. Neuroimmunol.
J Neuroimmunol 2015 Aug 20;285:53-6. Epub 2015 May 20.
Royal Brisbane and Women's Hospital, Department of Neurology, Butterfield Street, Herston QLD 4029, Australia; University of Queensland, Centre for Clinical Research, Herston Campus, Herston, QLD 4029, Australia.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired inflammatory neuropathy, which has similar clinical and pathological features to Guillain-Barré Syndrome (GBS), but differs in time course. We investigated the frequency of genes encoding Killer immunoglobulin-like receptors and their HLA ligands in subjects with CIDP, in subjects with GBS and in healthy controls. There were no differences in KIR gene frequency among the 3 groups. Read More

The gene frequencies for HLA-B Bw4-I were significantly greater in CIDP than HC, but did not differ from GBS. The frequency of the combination of 3DL1/HLA-B Bw4I was greater in CIDP than HC, but did not differ from that of GBS. These data raise the possibility of NK cell function being an important factor in the pathogenesis of CIDP.

2015Jul
PLoS ONE
PLoS One 2015 21;10(7):e0131730. Epub 2015 Jul 21.
State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Communicable Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing, China, 102206; Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases, Hangzhou, China, 310003.
2015Jun
Indian J Anaesth
Indian J Anaesth 2015 Jun;59(6):391-2
Department of Neuroanaesthesia, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.