Autoimmune Dementia: Clinical Course and Predictors of Immunotherapy Response

Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.
Mayo Clinic Proceedings (Impact Factor: 6.26). 10/2010; 85(10):881-97. DOI: 10.4065/mcp.2010.0326
Source: PubMed


To define the diagnostic characteristics and predictors of treatment response in patients with suspected autoimmune dementia.
Between January 1, 2002, and January 1, 2009, 72 consecutive patients received immunotherapy for suspected autoimmune dementia. Their baseline clinical, radiologic, and serologic characteristics were reviewed and compared between patients who were responsive to immunotherapy and those who were not. Patients were classified as responders if the treating physician had reported improvement after immunotherapy (documented in 80% by the Kokmen Short Test of Mental Status, neuropsychological testing, or both).
Initial immunotherapeutic regimens included methylprednisolone in 56 patients (78%), prednisone in 12 patients (17%), dexamethasone in 2 patients (3%), intravenous immune globulin in 1 patient (1%), and plasma exchange in 1 patient (1%). Forty-six patients (64%) improved, most in the first week of treatment. Thirty-five percent of these immunotherapy responders were initially diagnosed as having a neurodegenerative or prion disorder. Pretreatment and posttreatment neuropsychological score comparisons revealed improvement in almost all cognitive domains, most notably learning and memory. Radiologic or electroencephalographic improvements were reported in 22 (56%) of 39 patients. Immunotherapy responsiveness was predicted by a subacute onset (P<.001), fluctuating course (P<.001), tremor (P=.007), shorter delay to treatment (P=.005), seropositivity for a cation channel complex autoantibody (P=.01; neuronal voltage-gated potassium channel more than calcium channel or neuronal acetylcholine receptor), and elevated cerebrospinal fluid protein (>100 mg/dL) or pleocytosis (P=.02). Of 26 immunotherapy-responsive patients followed up for more than 1 year, 20 (77%) relapsed after discontinuing immunotherapy.
Identification of clinical and serologic clues to an autoimmune dementia allows early initiation of immunotherapy, and maintenance if needed, thus favoring an optimal outcome.

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Available from: Jeffrey W Britton, Oct 13, 2015
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    • "Although the role of thyroid autoantibodies is unclear, HE is a type of autoimmune encephalopathy. The majority of patients respond to treatment with steroids, while certain patients undergo spontaneous remission without steroid administration (9) and other patients fail to improve with steroid treatment (10). Although HE is a rare disease, the condition typically represents autoimmune encephalopathy. "
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    ABSTRACT: Hashimoto's encephalopathy (HE) is an acute encephalopathy associated with Hashimoto's thyroiditis. The majority of reported cases have been associated with hypothyroidism, while cases with hyperthyroidism are rare. The current study reported on a 56-year old female patient with HE, who was found to have progressively aggregated dysarthria, gait disturbance, somniloquy and delirium. Thyroid function tests revealed that the patient had hyperthyroidism, with high levels of anti-thyroid antibodies. Following treatment with corticosteroids, the neurological/psychiatric symptoms of the patient were relieved quickly. The one-year follow-up investigation indicated that there was no recurrence of the disease, demonstrating that the treatment administered for this rare case was effective.
    Experimental and therapeutic medicine 08/2014; 8(2):515-518. DOI:10.3892/etm.2014.1761 · 1.27 Impact Factor
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    • "However, the diagnosis is largely a clinical one, and the role of thyroid autoimmunity in this syndrome's pathogenesis remains unclear. Fluctuations in the TPO-Ab titre have not been associated with clinical changes in the encephalopathy [5,20], and a reliable association between the response to immune-based therapy and TPO-Ab has not been found [5,8,21,22]. The clinical context is key in making a diagnosis of SREAT, though the presentation can be variable and patients with SREAT are frequently misdiagnosed at presentation with alternative diagnoses including viral encephalitis and degenerative dementia [21]. "
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    ABSTRACT: Background Autoimmunity is considered an uncommon but under-recognised cause of cognitive decline. Methods Serum samples from 3,253 randomly selected subjects enrolled in the Hunter Community Study, aged 55-85 years, were assayed for thyrotropin stimulatory hormone, anti-thyroid peroxidase antibodies (TPO-Ab), anti-nuclear antibodies (ANA) and extractable nuclear antigens (ENA). Cognitive function was assessed using the Audio Recorded Cognitive Screen (ARCS) tool. Results TPO-Ab were found in 8.4% and ANA in 27.9% of the study population, of whom 3% had positive ENA findings. No relationship was found between the ARCS score and either TPO-Ab (coefficient = 0.133; 95% CI −0.20, 0.82, p = 0.616), ANA at a low (coefficient = 1.01; 95% CI −2.58, 0.55, p = 0.203) or a high titre (coefficient = −0.65; 95% CI −2.59, 1.28, p = 0.508), or ENA antibodies (coefficient = 5.12; 95% CI −0.53, 10.77; p = 0.076). Conclusions Autoantibody findings are common in an aging population and are not associated with cognitive decline.
    05/2014; 4(2):140-6. DOI:10.1159/000362716
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    • "However, no connection between multidrug hypersensitivity and antithyroid autoantibodies has been reported to date. These case reports are intended to raise the level of awareness on immunebased epilepsy and/or dementia, especially since these entities may respond to immunomodulatory therapies [8] [14]. Both of our reported patients showed improvement following treatment by steroids or plasma exchange. "
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    ABSTRACT: Hashimoto's encephalopathy is defined by the coexistence of encephalopathy and antithyroid antibodies. We report two cases of adult-onset temporal lobe epilepsy with subacute cognitive decline, high titers of antithyroid antibodies, multi-antiepileptic drug hypersensitivity, and good response to immunomodulatory treatment. The relevance of multidrug hypersensitivity in the setting of adult-onset epilepsy and the importance of searching for autoimmune causes for epilepsy are discussed.
    Epilepsy and Behavior Case Reports 12/2013; 1(1):132–135. DOI:10.1016/j.ebcr.2013.07.004
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