1/9. Laminar cortical necrosis in central nervous system lupus: sequential changes in MR images.A 44-year-old woman with systemic lupus erythematosus developed central nervous system lupus presenting with headache, fever, cloudiness of consciousness, and psychotic symptoms. T1-weighted and proton MR images showed laminar high intensity lesions in the parietal and temporal cortex bilaterally. T2-weighted images of the lesions showed low signal intensity. Treatment with corticosteroids alleviated the clinical symptoms within 7 months. The low T2 and high T1 signal abnormalities disappeared in a year and in 5 years, respectively. The laminar cortical lesions on MR images were suggested to represent cortical necrosis associated with central nervous system lupus.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
2/9. Molecular tracking of antigen-specific T cell clones in neurological immune-mediated disorders.T cells recognizing self or microbial antigens may trigger or reactivate immune-mediated diseases. Monitoring the frequency of specific T cell clonotypes to assess a possible link with the course of disease has been a difficult task with currently available technology. Our goal was to track individual candidate pathogenic T cell clones, selected on the basis of previous extensive studies from patients with immune-mediated disorders of the CNS, including multiple sclerosis, HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) and chronic lyme neuroborreliosis. We developed and applied a highly specific and sensitive technique to track single CD4( ) and CD8( ) T cell clones through the detection and quantification of T cell receptor (TCR) alpha or beta chain complementarity-determining region 3 transcripts by real-time reverse transcriptase (RT)-PCR. We examined the frequency of the candidate pathogenic T cell clones in the peripheral blood and CSF during the course of neurological disease. Using this approach, we detected variations of clonal frequencies that appeared to be related to clinical course, significant enrichment in the CSF, or both. By integrating clonotype tracking with direct visualization of antigen-specific staining, we showed that a single T cell clone contributed substantially to the overall recognition of the viral peptide/MHC complex in a patient with HAM/TSP. T cell clonotype tracking is a powerful new technology enabling further elucidation of the dynamics of expansion of autoreactive or pathogen-specific T cells that mediate pathological or protective immune responses in neurological disorders.- - - - - - - - - - ranking = 562.31647777732keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
3/9. Thyrotoxic autoimmune encephalopathy: a repeat positron emission tomography study.Thyroid related autoantibodies have been related to the development of encephalopathy, known as Hashimoto's encephalopathy. However, their relation with the encephalopathy occurring in patients with Graves' disease has not been well established. The case is reported of a 51 year old woman presenting with subacute progressive dementia with evidence of hyperthyroidism. She had Graves' disease associated with high titres of thyroid related autoantibodies. Her encephalopathy was not improved by antithyroid drugs, but promptly responded to corticosteroid treatment, and stabilised with a gradual reduction of thyroid related autoantibody titres. brain positron emission tomography initially showed a diffuse and multifocal cerebral hypometabolism with subsequent normalisation on her clinical recovery, which was consistent with the acute and reversible cerebral inflammation probably mediated by autoimmune mechanisms.- - - - - - - - - - ranking = 0.5keywords = ms (Clic here for more details about this article) |
4/9. The spectrum of autoimmune autonomic neuropathies.We analyzed the clinical characteristics of 18 patients (13 female, 5 male) who had autoimmune autonomic neuropathy (AAN) and ganglionic acetylcholine receptor (AChR) autoantibodies. Mean age was 61.4 years (standard deviation, 12.0 years). Ten patients had subacute symptom onset, six with an antecedent event. Eight patients had chronic AAN, characterized by insidious symptom onset, without antecedent event, and gradual progression. A majority of patients with high antibody values (>1.00 nmol/L) had a combination of sicca complex (marked dry eyes and dry mouth), abnormal pupillary light response, upper gastrointestinal symptoms, and neurogenic bladder. Chronic AAN segregated into two subgroups. One subgroup (N = 4) had low antibody titer (0.09 /- 0.01 nmol/L) and a paucity of cholinergic symptoms. It was indistinguishable from pure autonomic failure. The other subgroup (N = 4) had high antibody titer (11.6 /- 2.08 nmol/L), sicca complex, abnormal pupils, and neurogenic bladder; three had severe upper gastrointestinal dysfunction. Higher antibody titers correlated with greater autonomic dysfunction and more frequent cholinergic dysautonomia. These observations expand the clinical spectrum of AAN to include chronic cases, some being indistinguishable from pure autonomic failure, and support the concept that ganglionic AChR antibodies are important diagnostically and pathophysiologically in acquired dysautonomia.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
5/9. Neuropathology and binding studies in anti-amphiphysin-associated stiff-person syndrome.The authors report a 71-year-old woman with amphiphysin-associated paraneoplastic stiff-person syndrome, opsoclonus, and encephalopathy. The patient's symptoms temporarily responded to plasmapheresis in parallel with a decline of serum anti-amphiphysin antibody titers. Later, the encephalopathy progressed rapidly and the patient died. Binding studies and the detection of autoantibodies in the patient's CNS as well as the treatment response suggest a pathogenic role of the anti-amphiphysin antibodies.- - - - - - - - - - ranking = 0.5keywords = ms (Clic here for more details about this article) |
6/9. Autoimmune optic neuropathy with anticardiolipin antibody mimicking multiple sclerosis in a child.PURPOSE: To demonstrate that autoimmune optic neuropathy (AON) may occur in the pediatric population. DESIGN: Interventional case report. methods: A 4-year-old developed four episodes of bilateral optic neuritis with mild concurrent weakness, ataxia, or dizziness; one episode of unilateral optic neuritis; and one episode of weakness over a period of 4 years. RESULTS: Autoimmune optic neuropathy was diagnosed because of the presence of anticardiolipin antibody and an abnormal skin biopsy with thrombin and immunoreactant deposition. She was treated with corticosteroids, aspirin, and gammaglobulin. This diminished the frequency and intensity of her attacks. CONCLUSION: This case represents the diagnosis and treatment of AON in a child.- - - - - - - - - - ranking = 2249.2659111093keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
7/9. Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody.OBJECTIVES: To describe the clinical features and coexisting serum autoantibodies in seven patients with encephalitis associated with autoantibodies to alpha-dendrotoxin-sensitive voltage-gated potassium channels (VGKCs), and to compare this disorder with other autoimmune encephalopathies. methods: Clinical information was obtained from a retrospective review of medical records and telephone interviews. All autoantibody testing was performed in a single laboratory. RESULTS: The seven patients were examined for subacute cognitive and behavioral changes. seizures, usually temporal-onset complex partial type, were documented in six patients, and all seven patients had EEG abnormalities. None had symptoms or signs of neuromuscular hyperexcitability. One described hypersalivation. Four patients had additional autoantibody markers of neurologic autoimmunity (muscle acetylcholine receptor, striational, P/Q-type calcium channel, or GAD65), and two had thyroperoxidase antibodies. Two patients had a history of cancer: one had active prostate adenocarcinoma, and the second had a remote history of tongue carcinoma. Cranial MRI demonstrated mesial temporal lobe abnormalities in all patients. One patient improved spontaneously, and six were treated with IV methylprednisolone. Three improved remarkably with treatment. At follow-up evaluation, one had no cognitive deficits, four had mild persistent short-term memory dysfunction, and two had persistent disabling behavioral deficits. CONCLUSIONS: Voltage-gated potassium channel antibodies are a valuable serologic marker of a potentially reversible autoimmune encephalopathy. The neurologic manifestations of this disorder are indistinguishable from paraneoplastic limbic encephalitis but are distinct from Morvan syndrome and Hashimoto encephalopathy.- - - - - - - - - - ranking = 0.5keywords = ms (Clic here for more details about this article) |
8/9. A dystonic syndrome associated with anti-basal ganglia antibodies.Anti-basal ganglia antibodies (ABGA) have been associated with movement disorders (usually tics and chorea) and psychiatric disturbance in children. This report describes five adult and adolescent patients (one male, four females; mean age of onset, 16 years (range, 13-35)) who presented subacutely with a clinical syndrome dominated by dystonia and had ABGA binding to antigens of similar molecular weights to those seen in Sydenham's chorea. Three patients had a clear history of respiratory infection before the onset of their symptoms. Three patients received immunosuppressive treatment, with three showing a notable reduction in symptoms. It is hypothesised that dystonia in adults or adolescents may be part of the clinical spectrum of the post-infectious syndrome associated with ABGA.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
9/9. Juvenile psoriatic arthritis and acquired sensorineural hearing loss in a teenager: is there an association?Autoimmune inner ear disease is a cause of sensorineural hearing loss, first described in 1979 by McCabe. The occurrence during rheumatic diseases is already documented in adults, but to our knowledge, this evidence is still lacking in children. A 13-yr-old girl affected by juvenile psoriatic arthritis, treated with etanercept, developed a bilateral and asymmetric sensorineural deafness. The patient significantly improved after steroid administration. Once ruled out the principal causes of sensorineural hearing loss, we also considered the hypothesis of an anti-TNF side effect. However, the clinical presentation, the efficacy on steroid treatment and the presence of inner ear auto-antibodies prompt us to consider autoimmune-SNHL as the most plausible diagnosis. The young age of our patient seems to suggest a genetic susceptibility to autoimmunity and supports the concept of associated autoimmune diseases.- - - - - - - - - - ranking = 0.5keywords = ms (Clic here for more details about this article) |