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: 3.38). 03/2011; 258(9):1705-7. DOI: 10.1007/s00415-011-5976-2
Source: PubMed
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    • "It has since been recognized that the presentation of HE is extremely varied, ranging from a fulminant encephalopathy to a chronic dementia [5]. Reported presentations of HE include amnestic syndrome [6], seizures including status epilepticus [7], focal neurological deficits such as ataxia [8] [9] [10] [11] and myoclonus [12] [13], and psychiatric manifestations including depression [14], mania [15], psychosis and hallucinations [16] [17] [18]. "
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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.
    Full-text · Article · Jun 2013 · Journal of the neurological sciences
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    ABSTRACT: Hashimoto's encephalopathy was first described by Lord Brain in 1966. Since, other designations have been proposed and the existence of the disease itself has been debated. However, the number of reported cases in the literature is still increasing and physicians are sometimes confronted with patients with neuropsychiatric manifestations and positive thyroid antibodies. This article is an update based upon a search through Medline database that identified 316 references published between 1961 and 2011. Hashimoto's encephalopathy is a rare condition for which there is a need for both diagnostic criteria and therapeutic consensus.
    No preview · Article · Apr 2012 · La Revue de M├ędecine Interne
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    ABSTRACT: Herein, we summarize articles in the field of neuro-otology published in the Journal of Neurology over the last year. Topics included acute and chronic vertigo as well as auditory and ocular motor disorders. Characteristic lesion locations in Pusher syndrome are reported and the usefulness of bedside ocular motor tests in vertebrobasilar stroke is revisited. Probing the vestibular system and its value in predicting the outcome in vegetative state is discussed. Several articles address new diagnostic and therapeutic approaches in different disorders associated with chronic vestibular, auditory or gait deficits. In a series of case reports, we focus on different eye movement disorders in the vertical plane, which are often difficult to assess.
    Full-text · Article · Sep 2012 · Journal of Neurology
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