Article

Hashimoto's encephalopathy mimicking spinocerebellar ataxia

Department of Neurology, Xuanwu Hospital, Capital Medical University, 45 Changchun Street, 100053, Beijing, Poeple's Republic of China.
Journal of Neurology (Impact Factor: 3.84). 03/2011; 258(9):1705-7. DOI: 10.1007/s00415-011-5976-2
Source: PubMed
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    ABSTRACT: Hashimoto's Encephalopathy (HE) is a very rare condition characterized by psychosis, seizures, cognitive fluctuations, and myoclonus. In a few published cases, plasma exchange has been used due to the theoretical removal of antithyroid peroxidase antibodies (anti-TPO), one of the postulated causes of the condition. We report a case of HE treated by plasma exchange where no clinical or neurophysiologic improvement was observed despite documented reduction of the anti-TPO antibody to levels below the limits of laboratory detection. We discuss these findings in the context of the known literature for this disease process. J. Clin. Apheresis, 2014. © 2014 Wiley Periodicals, Inc.
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    ABSTRACT: OBJECTIVE: To share our experience on clinical presentation and management of patients diagnosed with Hashimoto's Encephalopathy (HE) at Vanderbilt Medical Center between 1999 and 2012. BACKGROUND: HE is a rare disorder characterized by encephalopathy and central nervous system (CNS) dysfunction, elevated antithyroid antibodies, the absence of infection or structural abnormalities in the CNS, and a response to treatment with steroids. The relationship between thyroid antibodies and encephalopathy has remained unresolved. DESIGN/METHODS: Retrospective chart review. RESULTS: We identified 13 patients who met the criteria for the diagnosis of HE. The median age was 49years (range, 2-66) and all except one were women. Encephalopathy in the form of altered mental status, stroke-like symptoms or seizures, with prompt resolution of symptoms upon receiving steroids, was the commonest presentation, seen in 7 patients. The second commonest presentation was subacute progressive decrease in cognitive function, which reversed within days to weeks after steroid therapy, seen in 4 patients. Electroencephalogram (EEG) was available in 12 patients and was abnormal in 8, showing nonspecific cerebral dysfunction in all 8 and epileptiform activity in 3. Treatment consisted of steroids in the acute phase for 12 of 13 patients with rapid improvement in symptoms. Maintenance therapy was rituximab in 7 patients, intravenous immunoglobulin (IVIg) in 7, azathioprine in 4, mycophenolate mofetil in 3, and methotrexate in 1 (some patients received sequential therapy with different agents). There was complete or near complete resolution of symptoms in 12 of the 13 patients. CONCLUSIONS: We present a cohort of patients in whom CNS dysfunction was associated with elevated antithyroid antibodies and reversal of disease followed immunomodulatory therapies.
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    ABSTRACT: Hashimoto's encephalopathy (HE) is a syndrome of altered mental status, hallucinations, delusional thinking, and often, epileptic seizures. It is diagnosed by the clinical syndrome, the presence of elevated titers of antithyroid antibodies, the lack of another diagnosis based on clinical evaluation, and the response to corticosteroid and other immunosuppressant treatment. This review discusses the symptoms, pathophysiology, and treatment of HE. The disorder is important to recognize because aggressive treatment may bring about a favorable clinical outcome. The disorder has a relatively benign prognosis, compared with many of the entities for which it can be mistaken.
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