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Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto’s encephalopathy
BACKGROUND: Creutzfeldt-Jakob disease and Hashimoto’s encephalopathy often show similar clinical presentation. Among Creutzfeldt-Jakob disease mimics, Hashimoto’s encephalopathy is particularly important as it is treatable with corticosteroids. Thus, in cases of middle-aged woman diagnosed with prob...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4172833/ https://www.ncbi.nlm.nih.gov/pubmed/25196053 http://dx.doi.org/10.1186/s12883-014-0179-y |
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author | Jang, Jae-Won Park, So Young Park, Young Ho Kim, Jung E Kim, SangYun |
author_facet | Jang, Jae-Won Park, So Young Park, Young Ho Kim, Jung E Kim, SangYun |
author_sort | Jang, Jae-Won |
collection | PubMed |
description | BACKGROUND: Creutzfeldt-Jakob disease and Hashimoto’s encephalopathy often show similar clinical presentation. Among Creutzfeldt-Jakob disease mimics, Hashimoto’s encephalopathy is particularly important as it is treatable with corticosteroids. Thus, in cases of middle-aged woman diagnosed with probable Creutzfeldt-Jakob disease and who exhibit high titers of antithyroid antibodies, corticosteroid pulse therapy is typically performed with expectations of near complete recovery from Hashimoto’s encephalopathy. Herein, we provide the first case report that exhibited a negative effect of corticosteroid pulse therapy for a patient with Creutzfeldt-Jakob disease with features of Hashimoto’s encephalopathy. CASE PRESENTATION: We report a case of 59-year-old Asian woman with blurred vision, dysarthria, myoclonus, and rapidly progressive dementia. Cerebrospinal fluid showed 14-3-3 protein positive. Electroencephalogram showed periodic sharp waves (1.5 Hz) at the bilateral frontal or occipital areas. Magnetic resonance imaging showed high signal intensities at the bilateral cerebral cortex, caudate nucleus, and putamen. The patient was diagnosed with probable Creutzfeldt-Jakob disease. However, serum analysis showed a high titer of antithyroid antibodies. We started corticosteroid pulse therapy with subsequent aggravation of seizure activity including generalized myoclonus, epilepsia parialis continua, and ballistic dyskinesia, which was effectively treated with clonazepam. CONCLUSION: We provide evidence of a case of Creutzfeldt-Jakob disease that exhibited clinical deterioration after corticosteroid therapy. Although histopathological confirmation with brain biopsy is not easily available in Creutzfeldt-Jakob disease patients, selective initiation of corticosteroid pulse therapy should be considered in cases of uncertain diagnosis for differentiation with Hashimoto’s encephalopathy. |
format | Online Article Text |
id | pubmed-4172833 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-41728332014-09-25 Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto’s encephalopathy Jang, Jae-Won Park, So Young Park, Young Ho Kim, Jung E Kim, SangYun BMC Neurol Case Report BACKGROUND: Creutzfeldt-Jakob disease and Hashimoto’s encephalopathy often show similar clinical presentation. Among Creutzfeldt-Jakob disease mimics, Hashimoto’s encephalopathy is particularly important as it is treatable with corticosteroids. Thus, in cases of middle-aged woman diagnosed with probable Creutzfeldt-Jakob disease and who exhibit high titers of antithyroid antibodies, corticosteroid pulse therapy is typically performed with expectations of near complete recovery from Hashimoto’s encephalopathy. Herein, we provide the first case report that exhibited a negative effect of corticosteroid pulse therapy for a patient with Creutzfeldt-Jakob disease with features of Hashimoto’s encephalopathy. CASE PRESENTATION: We report a case of 59-year-old Asian woman with blurred vision, dysarthria, myoclonus, and rapidly progressive dementia. Cerebrospinal fluid showed 14-3-3 protein positive. Electroencephalogram showed periodic sharp waves (1.5 Hz) at the bilateral frontal or occipital areas. Magnetic resonance imaging showed high signal intensities at the bilateral cerebral cortex, caudate nucleus, and putamen. The patient was diagnosed with probable Creutzfeldt-Jakob disease. However, serum analysis showed a high titer of antithyroid antibodies. We started corticosteroid pulse therapy with subsequent aggravation of seizure activity including generalized myoclonus, epilepsia parialis continua, and ballistic dyskinesia, which was effectively treated with clonazepam. CONCLUSION: We provide evidence of a case of Creutzfeldt-Jakob disease that exhibited clinical deterioration after corticosteroid therapy. Although histopathological confirmation with brain biopsy is not easily available in Creutzfeldt-Jakob disease patients, selective initiation of corticosteroid pulse therapy should be considered in cases of uncertain diagnosis for differentiation with Hashimoto’s encephalopathy. BioMed Central 2014-09-08 /pmc/articles/PMC4172833/ /pubmed/25196053 http://dx.doi.org/10.1186/s12883-014-0179-y Text en © Jang et al. licensee BioMed Central Ltd. 2014 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Jang, Jae-Won Park, So Young Park, Young Ho Kim, Jung E Kim, SangYun Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto’s encephalopathy |
title | Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto’s encephalopathy |
title_full | Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto’s encephalopathy |
title_fullStr | Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto’s encephalopathy |
title_full_unstemmed | Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto’s encephalopathy |
title_short | Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto’s encephalopathy |
title_sort | symptomatic aggravation after corticosteroid pulse therapy in definite sporadic creutzfeldt-jakob disease with the feature of hashimoto’s encephalopathy |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4172833/ https://www.ncbi.nlm.nih.gov/pubmed/25196053 http://dx.doi.org/10.1186/s12883-014-0179-y |
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