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Link: Hashimoto’s Encephalopathy: Systematic Review of the Literature and an Additional Case

Hashimoto’s encephalopathy, first described in 1966, is still problematic in terms of its pathophysiology, diagnosis, and treatment. The syndrome is more common in women, and is associated with autoimmune antithyroid antibodies. Presentation varies considerably; there may be episodes of cerebral ischemia, seizure, or psychosis, or there may be depression, cognitive decline, and periods of fluctuating consciousness. Because the symptoms respond so well to immunosuppressive treatment, prompt diagnosis and management are important. Here, the authors present a representative case report, along with a comprehensive review of current literature.

 

The full text can be read at http://neuro.psychiatryonline.org/doi/10.1176/jnp.23.4.jnp384#.

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.

Link: What’s in a NAIM? Hashimoto Encephalopathy, Steroid-Responsive Encephalopathy Associated With Autoimmune Thyroiditis, or Nonvasculitic Autoimmune Meningoencephalitis?

Hashimoto encephalopathy was first described by Lord Brain in 1966.1 Multiple case reports followed and the term Hashimoto encephalopathy became generally accepted for the syndrome of encephalopathy in association with Hashimoto thyroiditis. Although Brain’s original patient with Hashimoto encephalopathy did not improve with corticosteroid treatment, most of the patients described later showed marked improvement with some form of immunosuppression. This has led to the renaming of Hashimoto encephalopathy by some authors as “steroid-responsive encephalopathy associated with autoimmune thyroiditis” (SREAT)2 or “nonvasculitic autoimmune meningoencephalitis” (NAIM), a heterogeneous group that would include autoimmune disorders such as Sjögren syndrome and systemic lupus erythematosus–associated meningoencephalitis as well as Hashimoto encephalopathy. These disorders are unified by the feature of steroid responsiveness.

 

The partial article can be found at http://archneur.jamanetwork.com/article.aspx?articleid=790394.

Copyright © February 2006, Archives of Neurology

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.

Link: Recurrent status epilepticus associated with Hashimoto’s encephalopathy

Hashimoto’s encephalopathy (HE) is an infrequent disease with no well-known physiopathology. Status epilepticus is rarely reported in association with HE. We describe the 7-year evolution of a young woman who presented with recurrent status epilepticus as the main complication of HE. This evolution was especially marked by the occurrence of steroid-refractory symptoms and a poor outcome with persistent cognitive and behavioral consequences. We point out that the frontal lobes are especially implicated in these symptoms. This patient highlights the risk of multiple relapses and the need for a long follow-up period. We describe her clinical and paraclinical features, compare this patient to similar case reports, and comment on her outcome.

The full text can be found at http://www.sciencedirect.com/science/article/pii/S2213323213000297.

 

Copyright © Epilepsy & Behavior Case Reports, Elsevier 2013 | by H. Visée,C. Mabiglia,V. Vanderaspoilden,M.-D. Gazagnes,G. Glibert

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.

Link: Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis Presenting with Diffusion MR Imaging Changes

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) presents with focal or diffuse nonenhancing MR imaging abnormalities in 50% of patients with SREAT during subacute exacerbation. Vasculitic changes in biopsy studies as well as the elevation of antithyroid antibodies and CSF protein suggests an inflammatory cause. We report the case of a patient with SREAT with changes on diffusion-weighted MR imaging, which improved with corticosteroid therapy and plasmapheresis, supporting the theory of inflammatory changes in exacerbation of presumptive SREAT.

The full text of this study can be found at http://www.ajnr.org/content/29/8/1550.full.

Copyright © American Society of Neuroradiology | February 17, 2008
by C. Grommesa, C. Griffina, K.A. Downesb and A.J. Lernera

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.