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: 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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    ABSTRACT: Background: Hashimoto's encephalopathy (HE) presents with a variety of neurologic and neuropsychiatric features. In this study, we investigated the clinical and immunological profiles of the cerebellar ataxic form of HE. Methods: The clinical features, treatments, laboratory features, brain imaging, and serum anti-NH(2)-terminal of α-enolase autoantibodies (anti-NAE Abs), a useful diagnostic marker for HE, were investigated in 13 patients who presented with sporadic adult-onset cerebellar ataxia and fulfilled the HE diagnostic criteria (antithyroid Abs and responsiveness to immunotherapy). Results: All of the patients presented with truncal ataxia, but nystagmus was uncommon (17%). Eight patients had an insidious onset that mimicked spinocerebellar degeneration (SCD), but brain imaging showed little or no cerebellar atrophy in all of the patients. Those patients with serum anti-NAE Abs (n = 8) did not have nystagmus and tended to respond better to immunotherapy than the anti-NAE Ab-negative patients. Conclusion: The present study suggests that insidious adult-onset and truncal ataxia are common in the cerebellar ataxic form of HE, which mimics SCD, but that nystagmus and severe cerebellar atrophy are uncommon. Antithyroid and anti-NAE Abs may be useful for diagnosing cerebellar ataxic HE.
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