Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition?

Details

Serval ID
serval:BIB_B7E03965DC7F
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition?
Journal
Movement Disorders
Author(s)
Ghika-Schmid F., Ghika J., Regli F., Dworak N., Bogousslavsky J., Städler C., Portmann L., Despland P.A.
ISSN
0885-3185 (Print)
ISSN-L
0885-3185
Publication state
Published
Issued date
1996
Volume
11
Number
5
Pages
555-562
Language
english
Notes
Publication types: Case Reports ; Journal Article ; Review
Publication Status: ppublish
Abstract
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 anti-thyroglobulin 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.
Keywords
Adult, Aged, Anti-Inflammatory Agents/administration & dosage, Anti-Inflammatory Agents/therapeutic use, Anticonvulsants/administration & dosage, Anticonvulsants/therapeutic use, Brain/physiopathology, Clonazepam/administration & dosage, Clonazepam/therapeutic use, Cognition Disorders/diagnosis, Electroencephalography, Extrapyramidal Tracts/physiopathology, Female, Frontal Lobe/physiopathology, Humans, Methylprednisolone/administration & dosage, Methylprednisolone/therapeutic use, Myoclonus/drug therapy, Myoclonus/physiopathology, Neuropsychological Tests, Prednisone/administration & dosage, Prednisone/therapeutic use, Temporal Lobe/physiopathology, Thyroiditis, Autoimmune/diagnosis, Thyroiditis, Autoimmune/drug therapy, Thyrotropin/secretion
Pubmed
Web of science
Create date
25/01/2008 11:40
Last modification date
20/08/2019 15:25
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