Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition?
Identifieur interne : 004C00 ( Ncbi/Checkpoint ); précédent : 004B99; suivant : 004C01Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition?
Auteurs : F. Ghika-Schmid [Suisse] ; J. Ghika ; F. Regli ; N. Dworak ; J. Bogousslavsky ; C. St Dler ; L. Portmann ; P A DesplandSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 1996.
English descriptors
- KwdEn :
- 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), Thyroiditis, Autoimmune (physiopathology), Thyrotropin (secretion).
- MESH :
- chemical , administration & dosage : Anti-Inflammatory Agents, Anticonvulsants, Clonazepam, Methylprednisolone, Prednisone.
- chemical , secretion : Thyrotropin.
- chemical , therapeutic use : Anti-Inflammatory Agents, Anticonvulsants, Clonazepam, Methylprednisolone, Prednisone.
- diagnosis : Cognition Disorders, Thyroiditis, Autoimmune.
- drug therapy : Myoclonus, Thyroiditis, Autoimmune.
- physiopathology : Brain, Extrapyramidal Tracts, Frontal Lobe, Myoclonus, Temporal Lobe, Thyroiditis, Autoimmune.
- Adult, Aged, Electroencephalography, Female, Humans, Neuropsychological Tests.
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.
DOI: 10.1002/mds.870110511
PubMed: 8866497
Affiliations:
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Regli</name></author><author><name sortKey="Dworak, N" sort="Dworak, N" uniqKey="Dworak N" first="N" last="Dworak">N. Dworak</name></author><author><name sortKey="Bogousslavsky, J" sort="Bogousslavsky, J" uniqKey="Bogousslavsky J" first="J" last="Bogousslavsky">J. Bogousslavsky</name></author><author><name sortKey="St Dler, C" sort="St Dler, C" uniqKey="St Dler C" first="C" last="St Dler">C. St Dler</name></author><author><name sortKey="Portmann, L" sort="Portmann, L" uniqKey="Portmann L" first="L" last="Portmann">L. Portmann</name></author><author><name sortKey="Despland, P A" sort="Despland, P A" uniqKey="Despland P" first="P A" last="Despland">P A Despland</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1996">1996</date><idno type="RBID">pubmed:8866497</idno><idno type="pmid">8866497</idno><idno type="doi">10.1002/mds.870110511</idno><idno type="wicri:Area/PubMed/Corpus">004782</idno><idno type="wicri:Area/PubMed/Curation">004782</idno><idno type="wicri:Area/PubMed/Checkpoint">004819</idno><idno type="wicri:Area/Ncbi/Merge">004C00</idno><idno type="wicri:Area/Ncbi/Curation">004C00</idno><idno type="wicri:Area/Ncbi/Checkpoint">004C00</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition?</title><author><name sortKey="Ghika Schmid, F" sort="Ghika Schmid, F" uniqKey="Ghika Schmid F" first="F" last="Ghika-Schmid">F. Ghika-Schmid</name><affiliation wicri:level="3"><nlm:affiliation>Service de Neurologie, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.</nlm:affiliation><country xml:lang="fr">Suisse</country><wicri:regionArea>Service de Neurologie, Centre Hospitalier Universitaire Vaudois, Lausanne</wicri:regionArea><placeName><settlement type="city">Lausanne</settlement><region nuts="3" type="region">Canton de Vaud</region></placeName></affiliation></author><author><name sortKey="Ghika, J" sort="Ghika, J" uniqKey="Ghika J" first="J" last="Ghika">J. Ghika</name></author><author><name sortKey="Regli, F" sort="Regli, F" uniqKey="Regli F" first="F" last="Regli">F. Regli</name></author><author><name sortKey="Dworak, N" sort="Dworak, N" uniqKey="Dworak N" first="N" last="Dworak">N. Dworak</name></author><author><name sortKey="Bogousslavsky, J" sort="Bogousslavsky, J" uniqKey="Bogousslavsky J" first="J" last="Bogousslavsky">J. Bogousslavsky</name></author><author><name sortKey="St Dler, C" sort="St Dler, C" uniqKey="St Dler C" first="C" last="St Dler">C. St Dler</name></author><author><name sortKey="Portmann, L" sort="Portmann, L" uniqKey="Portmann L" first="L" last="Portmann">L. Portmann</name></author><author><name sortKey="Despland, P A" sort="Despland, P A" uniqKey="Despland P" first="P A" last="Despland">P A Despland</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="ISSN">0885-3185</idno><imprint><date when="1996" type="published">1996</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Aged</term><term>Anti-Inflammatory Agents (administration & dosage)</term><term>Anti-Inflammatory Agents (therapeutic use)</term><term>Anticonvulsants (administration & dosage)</term><term>Anticonvulsants (therapeutic use)</term><term>Brain (physiopathology)</term><term>Clonazepam (administration & dosage)</term><term>Clonazepam (therapeutic use)</term><term>Cognition Disorders (diagnosis)</term><term>Electroencephalography</term><term>Extrapyramidal Tracts (physiopathology)</term><term>Female</term><term>Frontal Lobe (physiopathology)</term><term>Humans</term><term>Methylprednisolone (administration & dosage)</term><term>Methylprednisolone (therapeutic use)</term><term>Myoclonus (drug therapy)</term><term>Myoclonus (physiopathology)</term><term>Neuropsychological Tests</term><term>Prednisone (administration & dosage)</term><term>Prednisone (therapeutic use)</term><term>Temporal Lobe (physiopathology)</term><term>Thyroiditis, Autoimmune (diagnosis)</term><term>Thyroiditis, Autoimmune (drug therapy)</term><term>Thyroiditis, Autoimmune (physiopathology)</term><term>Thyrotropin (secretion)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="administration & dosage" xml:lang="en"><term>Anti-Inflammatory Agents</term><term>Anticonvulsants</term><term>Clonazepam</term><term>Methylprednisolone</term><term>Prednisone</term></keywords><keywords scheme="MESH" type="chemical" qualifier="secretion" xml:lang="en"><term>Thyrotropin</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Anti-Inflammatory Agents</term><term>Anticonvulsants</term><term>Clonazepam</term><term>Methylprednisolone</term><term>Prednisone</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Cognition Disorders</term><term>Thyroiditis, Autoimmune</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Myoclonus</term><term>Thyroiditis, Autoimmune</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Brain</term><term>Extrapyramidal Tracts</term><term>Frontal Lobe</term><term>Myoclonus</term><term>Temporal Lobe</term><term>Thyroiditis, Autoimmune</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>Electroencephalography</term><term>Female</term><term>Humans</term><term>Neuropsychological Tests</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">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.</div></front></TEI><affiliations><list><country><li>Suisse</li></country><region><li>Canton de Vaud</li></region><settlement><li>Lausanne</li></settlement></list><tree><noCountry><name sortKey="Bogousslavsky, J" sort="Bogousslavsky, J" uniqKey="Bogousslavsky J" first="J" last="Bogousslavsky">J. Bogousslavsky</name><name sortKey="Despland, P A" sort="Despland, P A" uniqKey="Despland P" first="P A" last="Despland">P A Despland</name><name sortKey="Dworak, N" sort="Dworak, N" uniqKey="Dworak N" first="N" last="Dworak">N. Dworak</name><name sortKey="Ghika, J" sort="Ghika, J" uniqKey="Ghika J" first="J" last="Ghika">J. Ghika</name><name sortKey="Portmann, L" sort="Portmann, L" uniqKey="Portmann L" first="L" last="Portmann">L. Portmann</name><name sortKey="Regli, F" sort="Regli, F" uniqKey="Regli F" first="F" last="Regli">F. Regli</name><name sortKey="St Dler, C" sort="St Dler, C" uniqKey="St Dler C" first="C" last="St Dler">C. St Dler</name></noCountry><country name="Suisse"><region name="Canton de Vaud"><name sortKey="Ghika Schmid, F" sort="Ghika Schmid, F" uniqKey="Ghika Schmid F" first="F" last="Ghika-Schmid">F. Ghika-Schmid</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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