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Specialty: Neurosurgery
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Total 602 results found since Jan 2013.
How is the blood-nerve barrier involved in the pathogenetic mechanisms of multifocal motor neuropathy?
Unknown humoral factors and some autoantibodies in sera from multifocal motor neuropathy (MMN) patients cause disruption of the blood–nerve barrier (BNB). MMN is a pure motor neuropathy characterised by asymmetric distal dominant weakness, slowly progressive course and multifocal conduction blocks in nerve conduction studies.1 MMN is included in the list of the differential diagnosis of motor neurone disease because of the presence of atrophy and fasciculation in the affected limbs. Patients with MMN frequently have elevated levels of immunoglobulin (Ig)M antibodies against ganglioside GM1 in sera.2 High-dose intrave...
Source: Journal of Neurology, Neurosurgery and Psychiatry - April 10, 2014 Category: Neurosurgery Authors: Kusunoki, S. Tags: Immunology (including allergy), Brain stem / cerebellum, Cranial nerves, Infection (neurology), Multiple sclerosis, Neuromuscular disease, Peripheral nerve disease, Ophthalmology, Drugs: musculoskeletal and joint diseases Editorial commentaries Source Type: research
A roadmap for genetic testing in ALS
Up-to-date guidelines help neurologists and ALS patients determine when genetic testing might be appropriate and how best to interpret results Chiò and colleagues present a new set of clinical suggestions for the genetic counselling and testing of patients diagnosed with amyotrophic lateral sclerosis (ALS).1 The paper provides sage, straightforward advice for neurologists faced with questions from patients or their relatives about their risk of developing this fatal neurodegenerative disease and the role of genetic testing in quantifying that risk. Key among their recommendations is that mutational screening be exte...
Source: Journal of Neurology, Neurosurgery and Psychiatry - April 10, 2014 Category: Neurosurgery Authors: Traynor, B. J. Tags: Immunology (including allergy), Dementia, Drugs: CNS (not psychiatric), Motor neurone disease, Neuromuscular disease, Spinal cord, Memory disorders (psychiatry) Editorial commentaries Source Type: research
Myasthenia gravis and pregnancy: pressing time for best practice guidelines
Simple consensus guidelines should lead to uniform, consistent management of myasthenia gravis in pregnancy As is well known, the placenta is an immune privileged site, adapted to protect the developing fetus from potentially harmful immune effects from the host. In myasthenia gravis (MG), an organ-specific autoimmune disorder of neuromuscular transmission, antibodies specific for the acetylcholine receptor (AChR), principally of the immunoglobulin (Ig)G subclass,1 cause muscle weakness. Despite its immune protective role, the placenta allows these specific IgG antibodies to cross into the developing fetal circulation, aff...
Source: Journal of Neurology, Neurosurgery and Psychiatry - April 10, 2014 Category: Neurosurgery Authors: Maddison, P. Tags: Immunology (including allergy), Drugs: CNS (not psychiatric), Neuromuscular disease, Musculoskeletal syndromes Editorial commentaries Source Type: research
Sera from patients with multifocal motor neuropathy disrupt the blood-nerve barrier
Conclusions
The sera from MMN patients may disrupt the BNB function via the autocrine secretion of VEGF in PnMECs, or the exposure to autoantibodies against PnMECs that are contained in the MMN sera. Autoantibodies against PnMECs in MMN sera may activate the BNB by upregulating the VCAM-1 expression, thereby allowing for the entry of a large number of circulating inflammatory cells into the peripheral nervous system.
Source: Journal of Neurology, Neurosurgery and Psychiatry - April 10, 2014 Category: Neurosurgery Authors: Shimizu, F., Omoto, M., Sano, Y., Mastui, N., Miyashiro, A., Tasaki, A., Maeda, T., Koga, M., Kaji, R., Kanda, T. Tags: Immunology (including allergy), Motor neurone disease, Neuromuscular disease, Spinal cord Source Type: research
Myasthenia in pregnancy: best practice guidelines from a UK multispecialty working group
A national UK workshop to discuss practical clinical management issues related to pregnancy in women with myasthenia gravis was held in May 2011. The purpose was to develop recommendations to guide general neurologists and obstetricians and facilitate best practice before, during and after pregnancy. The main conclusions were (1) planning should be instituted well in advance of any potential pregnancy to allow time for myasthenic status and drug optimisation; (2) multidisciplinary liaison through the involvement of relevant specialists should occur throughout pregnancy, during delivery and in the neonatal period; (3) provi...
Source: Journal of Neurology, Neurosurgery and Psychiatry - April 10, 2014 Category: Neurosurgery Authors: Norwood, F., Dhanjal, M., Hill, M., James, N., Jungbluth, H., Kyle, P., O'Sullivan, G., Palace, J., Robb, S., Williamson, C., Hilton-Jones, D., Nelson-Piercy, C. Tags: Immunology (including allergy), Neuromuscular disease Source Type: research
Reduced grey matter perfusion without volume loss in early relapsing-remitting multiple sclerosis
Conclusions
The decrease in GM perfusion in the absence of volume loss is consistent with neuronal metabolic dysfunction in early RRMS. Future studies in larger cohorts and longitudinal follow-up are needed to investigate the functional and prognostic significance of the early GM perfusion deficits observed.
Source: Journal of Neurology, Neurosurgery and Psychiatry - April 10, 2014 Category: Neurosurgery Authors: Debernard, L., Melzer, T. R., Van Stockum, S., Graham, C., Wheeler-Kingshott, C. A., Dalrymple-Alford, J. C., Miller, D. H., Mason, D. F. Tags: Immunology (including allergy), Multiple sclerosis Source Type: research
The clinical relevance of voltage gated potassium channel (VGKC)-complex antibodies: the story is still unfolding
An increasingly broad spectrum of neurological disorders have been associated with voltage gated potassium channel (VGKC)-complex antibodies, including peripheral nerve hyperexcitability, Morvan's syndrome, limbic encephalitis, epilepsy and, more recently, idiopathic pain and psychiatric disorders. Over the past several years, there have been great leaps in our understanding of VGKC autoimmunity, including identification of specific antigenic targets in VGKC associated proteins.1 2 Several specific targets of VGKC-complex associated antibodies have now been identified, including the cell surface antigens leucine-rich gliom...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 6, 2014 Category: Neurosurgery Authors: Park, S. B. Tags: Immunology (including allergy), Epilepsy and seizures, Infection (neurology), Neurooncology, Pain (neurology), Spinal cord, CNS cancer Editorial commentaries Source Type: research
Autologous haematopoietic stem cell transplantation: a viable treatment option for CIDP
Conclusions
Our results though hampered by the limited number of patients and the lack of a control group suggest AHSCT to be efficacious in therapy-refractory CIDP, with a manageable complication profile. Confirmation of these results is necessary through randomised controlled trials.
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 6, 2014 Category: Neurosurgery Authors: Press, R., Askmark, H., Svenningsson, A., Andersen, O., Axelson, H. W., Stromberg, U., Wahlin, A., Isaksson, C., Johansson, J.-E. J., Hagglund, H. Tags: Immunology (including allergy), Neuromuscular disease, Peripheral nerve disease Neuro-inflammation Source Type: research
Clinical relevance of positive voltage-gated potassium channel (VGKC)-complex antibodies: experience from a tertiary referral centre
Conclusions
As antibody testing becomes more widely available, and many samples are referred from patients with less clear-cut diagnoses, it is important to assess the utility of the results. VGKC-complex antibodies in the range of 100–400 pM (0.1–0.4 nM) were considered clinically relevant in rare conditions with peripheral nerve hyperexcitability and appeared to associate with tumours (12.5%). By contrast high-positive (>400 pM; >0.4 nM) levels were considered definitely (38%) or possibly (49%) clinically relevant, but not all patients had a ‘classical’ limbic encephaliti...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 6, 2014 Category: Neurosurgery Authors: Paterson, R. W., Zandi, M. S., Armstrong, R., Vincent, A., Schott, J. M. Tags: Immunology (including allergy), Drugs: CNS (not psychiatric), Infection (neurology), Neuromuscular disease, Peripheral nerve disease, Spinal cord, Musculoskeletal syndromes Neuro-inflammation Source Type: research
Subcutaneous immunoglobulin therapy for inflammatory neuropathy: current evidence base and future prospects
Intravenous immunoglobulin therapy is of proven effect in chronic inflammatory neuropathies, including chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). In more recent years, there have been a number of anecdotal case reports and small series, followed by a few trials of variable design, of subcutaneous immunoglobulin therapy in these neuropathies. To date, limited evidence suggests that the subcutaneous route may be a more clinically effective, better-tolerated, at least cost-equivalent and a more patient-friendly option than the still more used intravenous alternative. Long-t...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 6, 2014 Category: Neurosurgery Authors: Rajabally, Y. A. Tags: Immunology (including allergy), Neuromuscular disease, Peripheral nerve disease Source Type: research
Immune-mediated neuropathies following stem cell transplantation
Conclusions
IMN occurs rarely in patients after SCT. Two possible mechanisms include (1) an immune reconstitution syndrome, supported by stereotypical neuropathy types (radiculoplexus and polyradiculoneuropathies), monophasic course and temporal association with SCT and (2) a paraneoplastic phenomenon, supported by frequent early malignancy recurrence following IMN.
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 6, 2014 Category: Neurosurgery Authors: Karam, C., Mauermann, M. L., Johnston, P. B., Lahoria, R., Engelstad, J. K., Dyck, P. J. B. Tags: Immunology (including allergy), Multiple sclerosis, Neuromuscular disease, Peripheral nerve disease Source Type: research
Efficacy of subcutaneous interferon {beta}-1a on MRI outcomes in a randomised controlled trial of patients with clinically isolated syndromes
Conclusions
Both subcutaneous IFN β-1a 44 µg regimens improved MRI outcomes versus placebo, with the three times a week regimen having a more pronounced effect than once a week dosing.
Trial registration
clinicaltrial.gov identifier, NCT00404352.
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 6, 2014 Category: Neurosurgery Authors: De Stefano, N., Comi, G., Kappos, L., Freedman, M. S., Polman, C. H., Uitdehaag, B. M. J., Hennessy, B., Casset-Semanaz, F., Lehr, L., Stubinski, B., Jack, D. L., Barkhof, F. Tags: Open access, Immunology (including allergy), Multiple sclerosis, Neuroimaging, Radiology, Radiology (diagnostics) Source Type: research
Plasma phosphorylated TDP-43 levels are elevated in patients with frontotemporal dementia carrying a C9orf72 repeat expansion or a GRN mutation
Conclusions
Our study shows that plasma pTDP-43 levels may be increased in some genetic forms of FTD known to be associated with TDP-43 proteinopathies.
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 6, 2014 Category: Neurosurgery Authors: Suarez-Calvet, M., Dols-Icardo, O., Llado, A., Sanchez-Valle, R., Hernandez, I., Amer, G., Anton-Aguirre, S., Alcolea, D., Fortea, J., Ferrer, I., van der Zee, J., Dillen, L., Van Broeckhoven, C., Molinuevo, J. L., Blesa, R., Clarimon, J., Lleo, A. Tags: Immunology (including allergy), Dementia, Memory disorders (psychiatry) Neurodegeneration Source Type: research
What is the prototype of familial amyloid polyneuropathy?
Familial amyloid polyneuropathy (FAP) is a hereditary form of amyloidosis most commonly caused by mutations in transthyretin (TTR) and was initially reported from endemic foci in Portugal, Japan and Sweden. However, development in gene diagnostic techniques revealed that FAP patients are more widely distributed and have varied phenotypes even in the same mutation.1 2 For example, Japanese patients who have early onset FAP with amyloidogenic TTR Val30Met mutation (FAP amyloidogenic transthyretin (ATTR) Val30Met) in endemic foci in Kumamoto and Nagano prefectures exhibit clinicopathological features distinct from those of la...
Source: Journal of Neurology, Neurosurgery and Psychiatry - June 10, 2014 Category: Neurosurgery Authors: Koike, H., Sobue, G. Tags: Genetics, Immunology (including allergy), Neuromuscular disease, Peripheral nerve disease Editorial commentaries Source Type: research
Antibodies to GM1: galactocerebroside complexes in multifocal motor neuropathy: it takes two to tango
In the mid-1980s, Pestronk reported the first cases of multifocal motor neuropathy to be associated with serum anti-monosialoganglioside (GM1) IgM antibodies.1 The potential importance of an easily measurable antibody as a biomarker for this diagnostically challenging but treatable neurological disorder was rapidly recognised. Subsequently, an extensive literature has emerged on the usefulness of anti-GM1 antibody testing in clinical practice and on its relationship to multifocal motor neuropathy (MMN) pathogenesis. The conventional ELISA sensitivity is currently considered to be around 50%; perhaps as a result of this rat...
Source: Journal of Neurology, Neurosurgery and Psychiatry - June 10, 2014 Category: Neurosurgery Authors: Willison, H. J., Galban-Horcajo, F., Halstead, S. K. Tags: Immunology (including allergy) Editorial commentaries Source Type: research
