C. Wilhelmgossling et al., AUTOIMMUNE ENCEPHALOPATHY ASSOCIATED WITH HASHIMOTOS-THYROIDITIS AS ACAUSE OF PROGRESSIVE DEMENTIA, Deutsche Medizinische Wochenschrift, 123(10), 1998, pp. 279-284
History and clinical findings: A 58-year-old woman, known for 10 years
to have Hashimoto's thyroiditis, was admitted from another hospital w
here, after an initial period of unconsciousness, she had developed pr
ogressive severe dementia, abnormal arousal and generalized myoclonia.
Jakob-Creutzfeldt disease (JCD) was suspected. Investigations: The el
ectroencephalogram (EEG) showed marked slowing of the basic activity a
nd 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 an
d the cerebrospinal fluid albumin concentration (1 g/l). Treatment and
course: The history and findings suggested autoimmune encephalities (
AIE) and treatment with prednisolone, 2 mg/kg body weight daily, was i
nitiated, achieving lasting improvement of arousal within two days. 6
weeks later the EEG merely showed mild alteration of basic activity. T
he thyroid antibody titres were now within normal limits and the signs
of inflammation were regressing. Conclusion: In case of rapidly progr
essive dementia autoimmune antibodies should be looked for in the diff
erential diagnosis, because autoimmune disease may be the treatable ca
use.