PubMed · 2908451
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T Tamargo Menéndez, H Fernández Suárez. [Autoimmune sensorineural hypoacusis].. https://pubmed.ncbi.nlm.nih.gov/2908451/
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HISTORY AND CLINICAL FINDINGS: A 58-year-old woman, known for 10 years to have Hashimoto's thyroiditis, was admitted from another hospital where, after an initial period of unconsciousness, she had developed progressive severe dementia, abnormal arousal and generalized myoclonia. Jakob-Creutzfeldt disease (JCD) was suspected. INVESTIGATIONS: The electroencephalogram (EEG) showed marked slowing of the basic activity and episodes of triphasic waves. The titres of thyroid antibodies (TPO 764 kU/l, TgAk 398 kU/l) and of the antinuclear antibodies (ANA 1:1280) were raised, as was the erythrocyte sedimentation rate (80/120 mm and the cerebrospinal fluid albumin concentration (1 g/l). TREATMENT AND COURSE: The history and findings suggested autoimmune encephalitis (AIE) and treatment with prednisolone, 2 mg/kg body weight daily, was initiated, achieving lasting improvement of arousal within two days. 6 weeks later the EEG merely showed mild alteration of basic activity. The thyroid antibody titres were now within normal limits and the signs of inflammation were regressing. CONCLUSION: In case of rapidly progressive dementia autoimmune antibodies should be looked for in the differential diagnosis, because autoimmune disease may be the treatable cause.
Intravenous immunoglobulin (IVIg) has been widely used in neurological diseases during the last decade. The current indications of IVIg in neurological diseases are reviewed and discussed on the basis of the available experimental data and clinical trials. Compared to other immunomodulating treatments used in neurological diseases, IVIg has only few side effects with a small risk of transmission of infectious agents. Good clinical evidence for the effectiveness is available for Guillain-Barré-Syndrome, chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy. In conditions like myasthenia gravis and myositis favourable effects of IVIg were reported, but future studies have to be awaited. For all other neurological conditions where IVIg has been administered, there is currently no support for the use of IVIg other than in controlled trials. In conclusion, IVIg is a promising immunomodulary therapy that has been shown to be effective in some neurological autoimmune diseases. Routine use in neurological practice should be restricted to diseases for which a positive effect has been proven in controlled trials. For all other conditions no definite recommendations can presently be made.