Transient global amnesia

Background Hashimoto encephalopathy has been described as a syndrome of encephalopathy and high serum antithyroid antibody concentrations that is responsive to glucocorticoid therapy, but these could be chance associations. Objective To study a patient with Hashimoto encephalopathy and to review the literature to determine whether Hashimoto encephalopathy is an identifiable syndrome. Data Sources and Extraction We searched the MEDLINE database to June 2002 for "Hashimoto" or "autoimmune thyroiditis" and "encephalopathy" and examined all identified articles and articles referenced therein, including all languages. We included all patients with noninfectious encephalopathy (clouding of consciousness and impaired cognitive function) and high serum antithyroid antibody concentrations. We excluded patients if they did not meet these inclusion criteria or if their symptoms could be explained by another neurologic disorder. We recorded clinical features and the results of imaging, electroencephalographic, thyroid function, and cerebrospinal fluid studies. Data Synthesis We identified 85 patients (69 women and 16 men; mean age, 44 years) with encephalopathy and high serum antithyroid antibody concentrations. Among these patients, 23 (27%) had strokelike signs, 56 (66%) had seizures, 32 (38%) had psychosis, 66 (78%) had a high cerebrospinal fluid protein concentration, and 80 (98%) of 82 had abnormal electroencephalographic findings. Thyroid function varied from overt hypothyroidism to overt hyperthyroidism; the most common abnormality was subclinical hypothyroidism (30 patients [35%]). Among patients treated with glucocorticoids, 66 (96%) improved. Conclusions The combination of encephalopathy, high serum antithyroid antibody concentrations, and responsiveness to glucocorticoid therapy seems unlikely to be due to chance. However, there is no evidence of a pathogenic role for the antibodies, which are probably markers of some other autoimmune disorder affecting the brain.

https://jamanetwork.com/journals/jamaneurology/articlepdf/783630/NNR20012.pdf

Hashimoto encephalopathy: syndrome or myth?

http://dosequis.colorado.edu/Courses/BrainWeb/papers/MG1.pdf

Myasthenia gravis: a comprehensive review of immune dysregulation and etiological mechanisms.

The Measurement and Appraisal of Adult Intelligence

Objective: To describe 16 patients with a coincidence of 2 rare diseases: aquaporin-4 antibody (AQP4-Ab)–mediated neuromyelitis optica spectrum disorder (AQP4-NMOSD) and acetylcholine receptor antibody (AChR-Ab)–mediated myasthenia gravis (AChR-MG). Methods: The clinical details and antibody results of 16 patients with AChR-MG and AQP4-NMOSD were analyzed retrospectively. Results: All had early-onset AChR-MG, the majority with mild generalized disease, and a high proportion achieved remission. Fifteen were female; 11 were Caucasian. In 14/16, the MG preceded NMOSD (median interval: 16 years) and 11 of these had had a thymectomy although 1 only after NMOSD onset. In 4/5 patients tested, AQP4-Abs were detectable between 4 and 16 years prior to disease onset, including 2 patients with detectable AQP4-Abs prior to thymectomy. AChR-Abs decreased and the AQP4-Ab levels increased over time in concordance with the relevant disease. AChR-Abs were detectable at NMOSD onset in the one sample available from 1 of the 2 patients with NMOSD before MG. Conclusions: Although both conditions are rare, the association of MG and NMOSD occurs much more frequently than by chance and the MG appears to follow a benign course. AChR-Abs or AQP4-Abs may be present years before onset of the relevant disease and the antibody titers against AQP4 and AChR tend to change in opposite directions. Although most cases had MG prior to NMOSD onset, and had undergone thymectomy, NMOSD can occur first and in patients who have not had their thymus removed.

Myasthenia gravis and neuromyelitis optica spectrum disorder: a multicenter study of 16 patients.

The prevalence of myasthenia gravis (MG) in the counties of Hordaland and Sogn & Fjordane on January 1, 1984 was 9.6 per 100,000 inhabitants. Other autoimmune diseases were found in 11 out of 48 MG patients. The occurrence of autoimmune thyroiditis (5 patients, 10.4%) and systemic lupus erythematosus (4 patients, 8.3%) in the MG patients was clearly higher than that reported in the general population. Rheumatoid arthritis was found in 2 patients (4.2%). The autoimmune diseases were mainly recorded among the nonthymectomized MG patients. In addition to those with definite diseases of autoimmune nature, 3 other MG patients had thyroid antibodies and 1 had antinuclear factor without clinical evidence of autoimmune disease. Seven MG patients (14.6%) had unspecific arthralgia during active periods of MG. Two MG patients had ankylosing spondylitis.

Associated disorders in myasthenia gravis: autoimmune diseases and their relation to thymectomy.

Serum concentrations of C4, IgG, IgA, and IgM were followed in 8 selected patients with myasthenia gravis (MG) during a 5-day course of plasma exchange (PE), using donor plasma as a replacement solution. C3 activation products (C3b, iC3b and C3c) and the terminal SC5b-9 complement complex were measured in 4 of the patients. All patients improved during the treatment, including 2 patients without detectable antibodies to AChR in serum. The main findings of the study were marked complement activation and an approximately 50% fall in the serum concentrations of IgM and C4 during PE, independent of the concentrations in the donor plasma. The concentrations of IgG and IgA did not change significantly. The fall in C4 during PE is presumably caused by C4 consumption. We postulate that the fall in IgM is an effect of a complement-induced vasodilatation and that PE-induced complement consumption may influence the effect of PE in patients with MG.

Plasma exchange in myasthenia gravis: changes in serum complement and immunoglobulins.

— Plasma exchange (PE) was performed in 6 patients with myasthenia gravis (MG). Acetylcholine receptor (AChR) antibodies and other MG-correlated antibodies were quantified each day before, during and after PE. The antibody concentrations decreased during PE with a consisting increase between two exchanges. Repeated PE induced a transient decline in the AChR antibody concentration. The clinical improvement was maximal during the week after PE and correlated in time with increasing concentrations of AChR antibodies.

Plasma exchange in myasthenia gravis: effect on anti‐AChR antibodies and other autoantibodies

Seventeen patients suffering from myasthenia gravis were treated with plasma exchange. Serious complications were not encountered. The effect usually appeared on the second or the third day of treatment. In 12 patients with a total of 31 plasma exchange courses, the treatment resulted in a shift in Osserman group. Six of these had long-standing remissions. Three patients responded moderately and two patients did not respond to plasma exchange.

Plasma exchange in myasthenia gravis: clinical effect.

Psychophysiologic response patterns were studied in female migraine patients during headache-free periods (n = 16) and in controls (n = 8). Cardiovascular variables studied were heart rate (HR) and temporal artery pulse wave amplitudes (PWA). Phasic responses to 80-dB, 1000-Hz tones, each lasting 2 sec and sounded at irregular intervals, were studied within a habituation laboratory design. In addition, electromyographic (EMG) levels from the frontal muscles and hand temperature were measured. HR responses were less pronounced and PWA reduction more pronounced in migraine patients than in controls. There were no differences between migraine patients and controls in habituation pattern. Migraine patients showed lower hand temperature and higher EMG levels than controls. Migraine patients were treated with eight temperature biofeedback sessions and were evaluated clinically and psychophysiologically during and 2 months after treatment. Clinical follow-up evaluation was done 2 years after treatment. An averag...

S. Thorlacius

Papers

Associated disorders in myasthenia gravis: autoimmune diseases and their relation to thymectomy.

Plasma exchange in myasthenia gravis: changes in serum complement and immunoglobulins.

Plasma exchange in myasthenia gravis: effect on anti‐AChR antibodies and other autoantibodies

Plasma exchange in myasthenia gravis: clinical effect.

Psychophysiologic response patterns in migraine before and after temperature biofeedback. Prediction of treatment outcome.