Hashimoto's myoclonic encephalopathy: An underdiagnosed treatable condition?
Article first published online: 4 NOV 2004
Copyright © 1996 Movement Disorder Society
Volume 11, Issue 5, pages 555–562, September 1996
How to Cite
Ghika-Schmid, F., Ghika, J., Regli, F., Dworak, N., Bogousslavsky, J., Städler, C., Portmann, L. and Despland, P. A. (1996), Hashimoto's myoclonic encephalopathy: An underdiagnosed treatable condition?. Mov. Disord., 11: 555–562. doi: 10.1002/mds.870110511
- Issue published online: 4 NOV 2004
- Article first published online: 4 NOV 2004
- Manuscript Accepted: 12 JAN 1996
- Hashimoto's myoclonic encephalopathy;
- Subacute diffuse encephalopathy
We report two patients with subacute diffuse encephalopathy characterized by confusion, myoclonic encephalopathy, and mild akineto-rigid extrapyramidal signs in one case and by apathy, memory deficit, and partial complex seizures in the other. Hashimoto's thyroiditis with high titers of antithyroglobulin antibodies was diagnosed in both patients, who were unresponsive to anticonvulsant medication, but showed rapid neurological improvement following steroid treatment. On neuropsychological examination, predominant frontotemporal dysfunction was noted. Electroencephalographic activity was remarkable for its rhythmical delta activity, unresponsive to, or even paradoxically increased by, anticonvulsant treatment. On magnetic resonance imaging, atrophy with temporal predominance was found. These observations support the idea that this potentially treatable dementia and movement disorder should be classified as a separate clinical entity.