Emergency (****); * (*): *-* This open-access article distributed under the terms of the Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0). Copyright © 2014 Shahid Beheshti University of Medical Sciences. All rights reserved. Downloaded from: www.jemerg.com 144 Emergency 2014; 2 (3): 144-146 CASE REPORT Hashimoto Encephalopathy in Case of Progressive Cognitive Impairment; a Case Report Abbas Tafakhori1, Bahaadin Siroos1*, Mojdeh Ghabaii1, Mohammad Hossein Harirchain1, Masih Tajdini1, Sushil Kumar Garg2 1. Department of Neurology, Iranian Center of Neurological Research, Tehran University of Medical Sciences, Tehran, Iran 2. Department of Surgery, University of Minnesota, Minneapolis, USA Abstract Hashimoto's encephalopathy (HE) is a rare condition characterized by atypical psychiatric and heterogeneous neu- rological manifestations such as acute cerebral ischemia, seizure, tremors, myoclonus, psychosis, depression, cogni- tive disorders, and fluctuating loss of consciousness. Here, a case of 28 year-old man was reported who referred to the emergency department (ED) with different acute neurologic disorders and final diagnose of HE. Key words: Encephalopathy; unconsciousness; cognition disorders; immunoglobulins, thyroid-stimulating; neuro- logic Manifestations Cite this article as: Tafakhori A, Siroos B, Ghabaii M, Harirchain MH, Tajdini M, Kumar Garg S. Hashimoto encephalopathy in case of progressive cognitive impairment; a case report. Emergency. 2014;2(3):144-6. Introduction: 1 ashimoto's encephalopathy (HE) is a rare condi- tion characterized by atypical psychiatric and heterogeneous neurological manifestations such as acute cerebral ischemia, seizure, tremors, myoclonus, psychosis, depression, cognitive disorders, and fluctuat- ing loss of consciousness (1-3). Despite a wealth of studies identifying the etiology of HE, its exact patho- genesis is not still completely understood (4, 5). Due to non-specific findings it is often considered to be a diag- nosis of exclusion. Currently, HE is considered to be a treatable dementia but there is no consensus on the duration or drug of choice for treatment. Here, a case of 28 year-old man was reported who referred to the emergency department (ED) with different acute neuro- logic disorders and final diagnose of HE. Case report: A previously healthy 28 year-old man was referred to the ED with a history of ophthalmoplegia and ataxia followed by progressive cognitive impairment. Two months prior to this episode, mild neck pain and sore throat were the only findings in his past medical history. He had no histo- ry of alcohol consumption, substance abuse, medication use, congenital disease, syncope, ischemic or hemorrhag- ic cerebrovascular attract, seizure, trauma, or any other known medical problems. The patients’ on-arrival vital signs were as follow: systolic blood pressure (SBP): 120 *Corresponding Author: Bahaaddin siroos, M.D; Department of Neurology, Iranian Center of Neurological Research, Tehran University of Medical Sciences, Tehran Iran. Phone/Fax: +982161192424/+982166581558. Email: bsiroos@razi.tums.ac.ir Received: July 2014; Accepted: July 2014 mmHg, pulse rate (PR): 90/minute, respiratory rate (RR): 14/minute, oral temperature: 37.5◦C, oxygen satu- ration 96% with nasal cannula and 100% oxygen, and Glasgow coma scale (GCS) 15/15. Physical examination revealed jerky movements in limbs, normal size and re- active pupils, fluctuating disorientation, severe cerebral ataxia, bilateral sixth nerve and upward gaze palsy, and increased deep tendon reflexes. A mini mental state ex- amination resulted in 10 of 30 points. The patients’ head and neck examination, lung and heart sounds, four limbs pulses, and capillary refile were normal. After assess- ment of airway, breathing, and circulation (ABC) patients were checked in terms of coma cocktail. Blood sugar was measured 100 mg/dl with glucometer. Electrocardiog- raphy (ECG) revealed normal sinus rhythm with normal axis. Brain computed tomography had not any abnormal findings, but bilateral hyper-signal white matter lesions were detected on fluid attenuation inversion recovery (FLAIR) and T2 weighted magnetic resonance imaging (MRI) (Figure 1). Cerebrospinal fluid (CSF) analysis re- sults were as follows: protein: 170 mg/dl, glucose: 67 mg/dl, cell count: 80/mm3 with lymphocyte dominancy. All of laboratory parameters as cell blood count, coagula- tion profile, kidney and liver function tests, venous blood gas parameters, and electrolyte were in normal range. Neurology consult was taken and patient admitted in neurology ward for further evaluations. Electroenceph- alography showed abnormal nonspecific sharp and spike waves in a slow background. Neural antibodies such as anti–N-methyl-D-aspartate receptor (NMDAR) were neg- ative. Thyroid statues demonstrated subclinical hypothy- roidism. Anti-thyroglobulin and anti-peroxidase antibod- H mailto:bsiroos@razi.tums.ac.ir This open-access article distributed under the terms of the Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0). Copyright © 2014 Shahid Beheshti University of Medical Sciences. All rights reserved. Downloaded from: www.jemerg.com 145 Tafakhori et al ies were 220 IU/ml and 740 IU/ml, respectively, which were significantly higher than normal ranges. All other assessments were normal. Taking the above data into consideration and excluding other differential diagnosis, HE was presumed. The patient underwent ten days of Methyl Prednisolone pulse regime (one gram per day) with tapering Prednisolone (120 mg orally per day) for two weeks. All signs and symptoms especially cognition and ophthalmoplegia dramatically improved and white matter lesions disappeared after two weeks of treatment (Figure 2). Discussion: Heterogeneous clinical manifestations and absence of non-specific tests have made the HE diagnosis as a chal- lenging problem for physicians. Susan lee et al. in a re- view concluded that psychiatrists should be aware of this often unrecognized entity to ensure accurate diag- nosis and timely treatment (6). HE has different MRI manifestations from normal appearance to demye- lination, ischemic lesions, edema, and atrophy (7). Usu- ally, HE is diagnosed by high levels of anti-TPO antibod- ies, normal T4, and thyroid stimulating hormone (TSH) titers in the presence of above mentioned heterogene- ous clinical manifestations (8). Recent studies have suggested cerebrospinal fluid titer of anti-thyroid anti- bodies as a pathognomonic test for HE (1, 9). According to the published case series, steroid is the only accepted treatment for this disease and among 81 adults and children, about 50% of patients recovered completely and the other cases relapsed or improved with residual deficits (10, 11). Previous studies have recommended three to five days corticosteroids (12). The present study is the first one that give the patient 10 days of Methyl Prednisolone. The patient responded completely to the corticosteroid. It seems that a favorable progno- sis may depend upon rapid recognition of HE and ag- gressive steroid treatment. However, further studies are needed to evaluate the advantage of this type of treatment for disease. Conclusion: In the case of unknown psychiatric and neurologic man- ifestations, measuring serum level of thyroid hormones and CSF titer of anti-thyroid antibodies could be helpful in limitation of differential diagnosis and timely initia- tion of proper treatment. Acknowledgments: None Conflict of interest: Authors declared no conflict of interest. Funding support: None Authors’ contributions: All authors passed four criteria for authorship contribu- tion based on recommendations of the International Committee of Medical Journal Editors. References: 1. de Holanda NCP, de Lima DD, Cavalcanti TB, Lucena CS, Bandeira F. Hashimoto's encephalopathy: systematic review of the literature and an additional case. J Neuropsychiatry Clin Neurosci. 2011;23(4):384-90. 2. 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