Recurrent partial seizures with ictal yawning as atypical presentation of Hashimoto's encephalopathy (steroid-responsive encephalopathy associated with autoimmune thyroiditis).

Hashimoto's encephalopathy (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a rare condition whose pathogenesis is unknown, though autoimmune-mediated mechanisms are thought to be involved. The prevalent neurological manifestations of this disorder are epileptic seizures and psychocognitive disorders associated with EEG alterations. High anti-thyroid antibody titers (particularly in cerebrospinal fluid) and the effectiveness of steroid therapy are usually considered to be crucial elements in the diagnostic process. We describe a 19-year-old female patient who had been referred to the psychiatric unit because of behavioral disorders characterized predominantly by delirium with sexual content. She developed recurrent focal seizures characterized by atypical ictal semiology (repetitive forceful yawning) and a rare EEG pattern (recurrent seizures arising from the left temporal region without evident "encephalopathic" activity). The presence of anti-thyroperoxidase antibodies in her cerebrospinal fluid and a good response to steroids confirmed the diagnosis of HE. The atypical presentation in the case we describe appears to widen the electroclinical spectrum of HE and highlights its importance for differential diagnosis purposes in the neuropsychiatric setting.

Autoimmunity in multiple sclerosis: study of a wide spectrum of autoantibodies.

The aim of this study was to assess the frequency of organ- and nonorgan-specific autoantibodies in MS patients and evaluate whether the presence of autoantibodies is an indicator of disease activity and/or a prognosis factor. One hundred and five definite MS patients in different stages and with different course and 75 blood donors were tested for the autoantibodies TgA, TMA/TPO-A, PCA, ANA, aCl, SMA, AMA and ANCA. All patients were screened for the LAC. Autoantibodies to at least one autoantigen were found in 66.6% MS patients and in 13.3% controls (P < 0.001). The frequency of TgA, TMA/TPO-A, ANA, aCl and SMA was statistically higher in patients than in controls. Circulating ANCAs were found in seven MS, a never reported finding. An early onset of MS (< 20 years) was associated with a lower autoantibody frequency (P < 0.01) Primary and secondary progressive MS had a higher antibody frequency than relapsing-remitting (P < 0.05) or benign (P < 0.001) MS. Up to 86% of patients were autoantibody-positive during the acute stage, but only 30% of them remained positive during the remission stage (P < 0.001). A generalised immune dysregulation occurs in MS patients, mostly during the acute stages and in the progressive courses, involving activation of both autoreactive Th1-cells (mainly linked to CNS lesions) and B-cells via Th2 cells.

[Antithyroid antibodies and endemic goiter]. / Autoanticorpi antitiroidei e gozzo endemico.

Relationship between thyroid autoantibodies and endemic goiter have been studied in 164 subjects from three different areas of endemic goiter: 91 patients and 31 healthy controls from Central Sardinia, 23 patients from Northern Latium, and 19 patients from Southern Latium. In subjects with endemic goiter from Sardinia higher levels of thyroid autoantibodies were present as compared to the healthy controls; microsomal fraction autoantibodies titer was higher than antithyroglobulin autoantibodies. In subjects from the two other endemic goiter areas the antimicrosomal and antithyroglobulin autoantibodies were absent, with the exception of one patient with basedow's goiter. It is suggested that some of the areas classified as positive for endemic goiter are indeed characterized by an extensive genetic predisposition to lymphocytic chronic thyroiditis complicated by nodular goiter.

Autoantibodies to insulin do appear in non-diabetic patients with autoimmune disorders: comparison with anti-immunoglobulin antibodies and other autoimmune phenomena.

Insulin- and anti-immunoglobulin-antibodies have been recently reported in pre-diabetic subjects: the former has been proposed as a predictive marker of Type I diabetes in non-diabetic-subjects. To evaluate the diabetes-related specificity of these antibodies, the presence of insulin autoantibodies, using a recently developed and highly sensitive competitive radioimmune assay, and of anti-immunoglobulin antibodies together with that of immune complexes and of other autoantibodies has been investigated in patients with organ- or non-organ-specific autoimmune diseases. One hundred and eleven serum samples were assayed from patients with Graves' disease, primary hypothyroidism, chronic autoimmune thyroiditis, Addison's disease, chronic autoimmune hepatitis, pernicious anemia, lupus erythematosus, and rheumatoid arthritis, together with 45 serum samples from normal subjects. From patients with autoimmune diseases, 32.4% of all sera revealed values of insulin autoantibodies above the limit of positivity (p less than 0.001); anti-immunoglobulin antibodies were present in 4.1% of patients (NS); immune complexes were found in 19.5% (NS) of all patients, but in 38% of patients with Graves' disease and chronic hepatitis (p less than 0.02). There was a trend for multiple autoantibody positivity to be associated with high levels of insulin autoantibodies (p less than 0.05). Thus, whereas contrary to expectation anti-immunoglobulin antibodies are not associated with non-diabetes-related autoimmune diseases, increased humoral immunoresponsiveness to endogenous insulin appears to be related to autoimmunity in general rather than restricted to Type I diabetes.

Gastric parietal cell antibodies: demonstration by immunofluorescence of their reactivity with surface of the gastric parietal cells.

Viable, intact gastric cells were obtained by pronase digestion of inverted rat stomach. The cell suspensions contained two main distinct cell population, i.e. 'large' cells (mean diameter 16 microns) and 'small' cells (mean diameter 8.5 microns). By indirect immunofluorescence on smears of dispersed rat gastric cells, the large cells were identified as parietal cells, since all the sera containing parietal cell antibodies (PCA) were seen to react with the cytoplasm of these cells, leaving the cytoplasm of the small cells completely unstained. Thirty-one PCA-positive sera and forty-one PCA-negative sera were tested for gastric cell surface-reactive antibodies by an indirect immunofluorescence technique on suspensions of viable gastric cells. All the PCA-containing sera yielded a membrane immunofluorescence confined to the large cells, while none of the PCA-negative sera induced this fluorescent pattern. The surface reaction persisted unmodified when F(ab')2 fragments processed from IgG PCA-positive sera and FITC-conjugated pepsin fragments of rabbit IgG directed against the F(ab')2 fragments of human IgG were employed for the membrane fluorescence studies. The absorption of PCA-positive sera with viable, intact gastric cells led to the disappearance of both the surface immunofluorescence of the viable large cells and the cytoplasmic fluorescence of the rat parietal cells. These results demonstrate that PCA invariably react with an antigen represented on the surface of parietal cells, and that this antigen is immunologically identical to the intracytoplasmic 'microsomal' antigen.

The lymphocyte stimulation test: a sensitive technique for the detection of circulating platelet antibodies.

Platelet autoantibodies are unable to react in standard immunological reactions and this makes it difficult to detect their presence in the serum. The present work shows that peripheral blood lymphocytes from normal donors are stimulated in vitro by autologous platelets sensitized with sera from patients with different types of chronic thrombocytopenia. Comparison of the lymphocyte stimulation test with the platelet factor 3 (PF3) availability assay and the serotonin (5HT) release test demonstrated that the former method was the more sensitive one. Some sera from patients with SLE and thrombocytopenia also induced both a slight lymphocyte stimulation in the absence of platelets and a complement-dependent release of serotonin, probably due to the presence of immune complexes.

Platelet antibodies in different forms of chronic thrombocytopenia.

Three techniques have been employed for the in vitro detection of circulating platelet antibodies in thrombocytopenic patients affected by 'idiopathic' form or by lupus erythematosus (SLE), the complement fixation test, the platelet factor 3 availability test and the serotonin release test. 29 of the 35 sera tested (82.8%) gave positive results for antiplatelet activity. In particular the serotonin release test allows to distinguish 4 groups of patients: a first group affected by idiopathic form; two groups with autoimmune thrombocytopenia and various degrees of serotonin release, and finally a fourth group which comprises subjects affected by SLE, with circulating immunocomplexes.

Mucus antibodies in pulmonary tuberculosis and chronic obstructive lung disease.

An indirect immunofluorescence technique and an indirect immunoperoxidase technique were used on cryostat sections of human group-O submaxillary salivary gland and rat stomach. Circulating antibodies reacting with mucus antigen(s) were found in sera from 52-7% of patients with active pulmonary tuberculosis and in 21-8% of patients with chronic obstructive lung disease. Among hospital patients with other diseases and healthy controls, mucus antibodies were found in 7-2 and 5.4% respectively. The mucus antibodies were not absorbed by an excess of red blood-cells derived from group AD+ healthy subjects or from the rat donor of the stomach, while the fluorescence and the immunoperoxidase reactions were almost completely abolished after the absorption of positive sera with human dried bronchial secretion. It is postulated that mucus antibody may be a new and important serological marker of disorders accompanied by mucus accumulation in the lung and possibly other organs and/or by severe changes of the anatomical structures which act as a barrier to the reabsorption of abnormal amounts of mucus.