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Hashimoto's myoclonic encephalopathy: An underdiagnosed treatable condition?
Author(s) -
GhikaSchmid F.,
Ghika J.,
Regli F.,
Dworak N.,
Bogousslavsky J.,
Städler C.,
Portmann L.,
Despland P. A.
Publication year - 1996
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.870110511
Subject(s) - myoclonus , encephalopathy , medicine , apathy , thyroiditis , dementia , extrapyramidal symptoms , pediatrics , psychology , pathology , anesthesia , psychiatry , disease , antipsychotic , schizophrenia (object oriented programming)
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.