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Autoimmune choreas
Chorea, a movement disorder characterised by a continuous flow of unpredictable muscle contractions, has a myriad of genetic and non-genetic causes. Although autoimmune processes are rare aetiology of chorea, they are relevant both for researchers and clinicians. The aim of this article is to provide a review of the epidemiology, clinical and laboratory features, pathogenesis and management of the most common autoimmune causes of chorea. Emphasis is given particularly to Sydenham's chorea, systemic lupus erythematosus, primary antiphospolipid antibody syndrome, paraneoplastic chorea and anti-N-methyl-d-aspartate receptor encephalitis.
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 4, 2017 Category: Neurosurgery Authors: Cardoso, F. Tags: Immunology (including allergy), Infection (neurology), Connective tissue disease, Systemic lupus erythematosus Movement disorders Source Type: research

Neurodegeneration in multiple sclerosis and neuromyelitis optica
Multiple sclerosis (MS) and neuromyelitis optica (NMO) are autoimmune demyelinating diseases of the central nervous system (CNS), having distinct immunological and pathological features. They have two pathogenic components, ‘inflammation’ and ‘neurodegeneration’, with different degrees of severity and pathogenetic mechanisms. The target antigen of autoimmunity in NMO is the water channel aquaporin-4 (AQP4), and antibodies directed against this antigen result in astrocyte damage. MS is a disease primarily affecting myelin and oligodendrocytes, but thus far, no MS-specific autoantigen has been identif...
Source: Journal of Neurology, Neurosurgery and Psychiatry - January 12, 2017 Category: Neurosurgery Authors: Kawachi, I., Lassmann, H. Tags: JNNP Patients' choice, Immunology (including allergy), Cranial nerves, Drugs: CNS (not psychiatric), Multiple sclerosis, Ophthalmology Source Type: research

Management of myasthenia gravis compared to national guidelines
Conclusion Management of MG patients at UHB in general comply with national guidelines. However, variations in practice were noted for once or alternate day steroid usage and also regarding reduction or withdrawal of Pyridostigmine.
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Perera, P. S., Tiet, M. Y., Jacob, S. Tags: Immunology (including allergy), Neuromuscular disease, Musculoskeletal syndromes ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Case report: pandysautonomia
We present the case of a 25 year old previously healthy female who became unwell with abdominal pain and vomiting a day after taking part in a half marathon and was found to have a pseudo-obstruction requiring laparotomy. Post surgery she developed a dry mouth and urinary retention and was consistently tachycardic with labile blood pressure. She developed one episode of parotitis which resolved with antibiotics. Family history included relatives with Addison's disease, hypothyroidism and Raynaud's phenomenon. Neurological examination was normal. There were no other findings to suggest systemic autoimmune disease. Vasculiti...
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Al-Ansari, A., Rickards, C., Thomas, L., Bain, S. Tags: Genetics, Immunology (including allergy), HIV/AIDS, Pain (neurology), Hypertension, Ophthalmology, Connective tissue disease, Musculoskeletal syndromes ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Case presentation: the dancing man from sierra leone
Discussion Making this diagnosis is challenging, as brain imaging often shows non specific changes and blood results are usually normal. CSF may reveal raised proteins and WBCs. A high index of suspicion is required to arrange a brain biopsy as this confirms the diagnosis.
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Al-Rifai, M. S., Salek-Haddadi, A. Tags: Immunology (including allergy), Neuroimaging, Stroke, Radiology, Drugs: musculoskeletal and joint diseases, Vascularitis, Surgical diagnostic tests ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Neuromyelitis optica in hiv: a case report and literature review
We present a case and review the literature. A 49 year old lady of African descent with known HIV diagnosed in 2004 – compliant with her antiretroviral medication, Atripla – was admitted with a 3 week history of thoracic back pain, spreading paraesthesia and weakness involving both legs, and constipation. Examination revealed a spastic paraparesis, a sensory level at T3/4 on the right and reduced vibration sense to the iliac crests bilaterally. MRI spine revealed increased signal within the cord from T3–7 in keeping with a longitudinally extensive transverse myelitis. CSF was acellular with normal protein...
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Appleton, J. P., Kenton, A. Tags: Neurogastroenterology, Immunology (including allergy), HIV/AIDS, Cranial nerves, Multiple sclerosis, Pain (neurology), Spinal cord, Ophthalmology ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Salt intake in multiple sclerosis: friend or foe?
High sodium intake has been recently noted as a putative environmental factor linked to multiple sclerosis. In their JNNP publication, Nourbakhsh and colleagues1 report a multicentric study of patients with paediatric multiple sclerosis where no association was detected between sodium intake and time to relapse. High dietary intake of salt is associated with elevated blood pressure, a major risk factor for cardiovascular disease.2 In 2010, 1.65 million annual deaths were attributed to sodium intake above the reference level (2 g/day).2 Nevertheless, the average consumption oscillates between 4 and 6 g/day.2 Beyon...
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Farez, M. F. Tags: Immunology (including allergy), Multiple sclerosis, Hypertension, Connective tissue disease, Musculoskeletal syndromes Editorial commentaries Source Type: research

New insights on neuromyelitis optica
Neuromyelitis-optica is a relapsing inflammatory autoimmune condition characterized mainly by acute myelitis and optic neuritis although other criteria exist. IgG against AQP-4 receptor on the astrocyte foot processes is 76% sensitive and 100% Specific for Neuromyelitis-optica. Immunosuppression with Azathioprine is 50% effective but never reported to worsen Neuromyelitis-optica. A 52-year-old-female with history of blindness secondary to bilateral optic neuritis and Myasthenia gravis, in remission, underwent extensive work up for relapsing remitting brain and cervical spine demyelinating lesions and frequent muscle spasms...
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Hussein, O., Cajavilca, C., Aachi, R., Minagar, A. Tags: Immunology (including allergy), Cranial nerves, Infection (neurology), Multiple sclerosis, Neuromuscular disease, Peripheral nerve disease, Spinal cord, Ophthalmology ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Rituximab use in a patient with anti-ri positive opsoclonus myoclonus syndrome
We describe a patient with OMS, who had a previous (and completely treated) diagnosis of Breast cancer more than 10 years ago, who presented with features of dizziness, opsoclonus, generalised myoclonus and truncal ataxia. Investigations revealed positive anti-Ri antibodies, but comprehensive screen for an underlying malignancy was negative. Patient was initially treated with intravenous immunoglobulins (IVIg) with good response. However, despite this patient had a further relapse which required hospitalisation with ventilator support and another course of IVIg. A second relapse was treated with pulsed IV cyclophosphamide ...
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Sadalage, G., Bowen, J., Nightingale, S., Jacob, S. Tags: Immunology (including allergy), Cranial nerves, Drugs: CNS (not psychiatric), Ophthalmology, Adult intensive care, Drugs: musculoskeletal and joint diseases ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Vsn16r - a novel treatment for spasticity in experimental multiple sclerosis
This study identifies a novel target for control of spasticity and suggests that VSN16R may be a useful novel therapeutic, which offers tolerability advantages over existing treatments. This may facilitate adoption of earlier treatment following to development of spasticity in MS. A phase II clinical trial (NCT02542787) is currently in progress.
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Williams, T., Pryce, G., Giovannoni, G., Selwood, D., Baker, D. Tags: Immunology (including allergy), Multiple sclerosis, Drugs: musculoskeletal and joint diseases ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Thyroid autoimmunity following alemtuzumab treatment for ms
We present long-term outcomes of the most common AID (thyroid), in order to inform pre-treatment counselling and subsequent disease management. Methods 87 patients treated with alemtuzumab were followed within an active surveillance program and detailed data collected prospectively. Standard clinical indices, thyroid diagnosis, thyroid function tests, thyroid autoantibodies and treatment were recorded. Results Mean follow-up was 7.4 years. 31 (36%,24F:7M) developed thyroid AID. Mean interval from first treatment was 3.0 years (range 0.8–8.7); peak incidence in years 2–3. 12 (38.7%) patients developed hyp...
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Healy, S., Nasser, T., Willis, M., Muller, I., Harding, K., Pickersgill, T., Wardle, M., Dayan, C., Robertson, N. Tags: Immunology (including allergy) ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Tubular aggregates and cylindrical spirals in autoimmune limb-girdle myasthenia
Limb-girdle myasthenia is a rare form of autoimmune myasthenia which presents without the typical ocular or bulbar symptoms. Patients present with proximal muscle weakness and are often misdiagnosed as a myopathy and tests for myasthenia may not always be performed. This 43-year-old lady presented with "legs giving way" while climbing stairs or bending down. She was initially investigated by the rheumatologists who noted a marginally increased creatine kinase level (263). A muscle biopsy which showed tubular aggregates on gomori trichrome staining and cylindrical spirals on electron microscopy. She was referred for a neuro...
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Sadalage, G., Jacob, S. Tags: Genetics, Immunology (including allergy), Muscle disease, Neuromuscular disease, Radiology, Musculoskeletal syndromes, Surgical diagnostic tests ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

What influences false positive achr antibody results?
This study looked into the samples tested during this period to identify any false positive rates. For the purpose of the study, false positive was defined as lack of response to autoimmune myasthenia treatments (and the absence of other clinical or neurophysiological features of MG), or tests requested in error when there was no clinical suspicion of myasthenia. 696 samples were tested for AChR from 582 patients. 280 patients (48.1%) were reported to have positive AChR antibodies. 27 of these were considered to be false positive. We analysed the clinical details of these patients and have identified factors like other aut...
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Sadalage, G., Torane, P., Karim, A., Jacob, S. Tags: Immunology (including allergy), Drugs: CNS (not psychiatric), Neuromuscular disease ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Necrotising autoimmune myopathy (NAM): antibodies seem to be specific markers in aiding diagnosis
Watanabe et al1 report on prospectively studied patients with necrotising autoimmune myopathy (NAM), diagnosed among 460 individuals with inflammatory myopathies (IM), over a 4-year period. This is the largest ever NAM cohort diagnosed with histological criteria and exclusion of genetically-defined muscular dystrophies. Among 387 patients (after excluding 16% with IBM), 30% had NAM with antibodies either against SRP (18%) or HMGCR (12%); another 13% had overlap myositis (OM), also a necrotising but perifascicular myositis, with anti-Jo-1 antibodies. Considering that antibodies are presently detected in 60–80% of NAM ...
Source: Journal of Neurology, Neurosurgery and Psychiatry - September 12, 2016 Category: Neurosurgery Authors: Dalakas, M. C. Tags: Genetics, Immunology (including allergy), Muscle disease, Neuromuscular disease, Radiology, Musculoskeletal syndromes, Surgical diagnostic tests Editorial commentaries Source Type: research

Clinical features and prognosis in anti-SRP and anti-HMGCR necrotising myopathy
Conclusions Anti-SRP antibodies are associated with severe neurological symptoms, more so than are anti-HMGCR antibodies. Although these autoantibodies are independent serological markers associated with IMNM, patients bearing either share common characteristics.
Source: Journal of Neurology, Neurosurgery and Psychiatry - September 12, 2016 Category: Neurosurgery Authors: Watanabe, Y., Uruha, A., Suzuki, S., Nakahara, J., Hamanaka, K., Takayama, K., Suzuki, N., Nishino, I. Tags: Immunology (including allergy), Drugs: CNS (not psychiatric), Muscle disease, Neuromuscular disease, Musculoskeletal syndromes Source Type: research

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