Hashimoto’s Encephalopathy

Hashimoto’s Encephalopathy is a rare autoimmune disease associated with Hashimoto’s thyroiditis. It was first described in 1966. It is sometimes referred to as a neuroendocrine disorder, although the condition’s relationship to the endocrine system is widely disputed. – Wikipedia

 

• Video: RHR: Low Dose Naltrexone (LDN) as a Treatment for Autoimmune Disease
  An excellent and informative video on LDN therapy in autoimmune diseases. 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 […]
• Link: Private Medical Services by UK Doctors
  While we do not advocate using online pharmacies that are not montiored by reputable GPs, some patients may find this link informative and help form an understanding of what autoimmune conditions LDN (low dose naltrexone) may help treat. Please always review your current medications with your GP before starting LDN therapy.   https://www.prescribe4me.co.uk/questionnaires/ldn.aspx This website […]
• Link: Hashimoto's encephalopathy : epidemiology, pathogenesis and management.
  Hashimoto’s encephalopathy is a term used to describe an encephalopathy of presumed autoimmune origin characterised by high titres of antithyroid peroxidase antibodies. In a similar fashion to autoimmune thyroid disease, Hashimoto’s encephalopathy is more common in women than in men. It has been reported in paediatric, adult and elderly populations throughout the world. The clinical […]
• Blog: #ZebraFightTonight against #HashimotosEncephalopathy
  

  On the 26th of April, local Oregon news station, KMTR interviewed husband and wife, Tim and Kelly McCabe. Kelly is battling a rare autoimmune disease known as Hashimoto’s Encephalopathy (also know as Steroid Responsive Autoimmune Encephalopathy Associated with Thyroiditis or SREAT). Recently a member of the online support group, Understanding Hashimoto’s Encephalopathy – A Support Forum, Tabitha Andrews-Orth, meet up with Kelly for the first time in person.

• Link: 'What can happen if you’re not treated is a coma, or ultimately death'
  SPRINGFIELD, Ore. – Two women suffering from a rare autoimmune disease said they were denied treatment at RiverBend Hospital. The two women suffer from a disease called Hashimoto’s Encephalitis. It’s a disorder that causes inflammation of the brain. A specialist recommended both patients receive a specific treatment at RiverBend Hospital, but the hospital allegedly denied […]
• Link: SAT-064: Autoimmune Thyroid Disease Associated with Steroid-Responsive Encephalopathy: A Case Report on Hashimoto′s Encephalopathy
  Hashimoto’s encephalopathy (HE) is a rare and underdiagnosed clinical entity presenting as a neuropsychiatric syndrome, associated with serologic evidence of antithyroid antibodies after other causes of encephalopathy are excluded. The main hallmark of HE is its good prognosis and responsiveness to steroids and immunosupressants. We report a case of a patient with elevated thyroid antibodies […]
• Link: Hashimoto's encephalopathy by Joana Ramalho, MD, and Mauricio Castillo, MD
  This link posted by T.A.O. talks about Hashimoto’s Encephalopathy. Abstract We report a case of Hashimoto’s encephalopathy with atypical and partially reversible MRI findings. T2-weighted MRI images revealed bilaterally symmetric areas of increased signal in the mesial temporal lobes and basal ganglia. Despite clinical and imaging improvement after steroid therapy, some memory deficits and MRI […]
• Link: Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition?
  We report two patients with subacute diffuse encephalopathy characterized by confusion, myoclonic encephalopathy, and mild akineto-rigid extrapyramidal signs in one case and by apathy, memory deficit, and partial complex seizures in the other.   Unfortunately this is an abstract only but useful information for those suffering myoclonic jerks and Hashimoto’s Encephalopathy. The abstract can be […]
• Link: Autoantibodies to neuronal surface antigens in thyroid antibody-positive and -negative limbic encephalitis
  Background : Thyroid antibodies (Thy-Abs) are frequently detected in various autoimmune disorders in coexistence with other systemic autoantibodies. In association with an encephalopathy, they are often taken as evidence of Hashimoto’s encephalitis (HE). However, the presence of Thy-Abs in a cohort of limbic encephalitis (LE) patients and their association with anti-neuronal autoimmunity has not been […]
• Link: Hashimotos Encephalopathy – A Reversible Cause of Dementia
  A form of potentially reversible Autoimmune Dementia, Steroid Response Encephalopathy Associated With Autoimmune Thyroiditis (SREAT), also known as Hashimoto Encephalopathy (HE) is characterised by confusion with or without myoclonus, seizures, hyperreflexia, and psychosis. Presentation may be an insidious development of cognitive impairment or recurrent acute episodes of focal neurological deficit with confusion (can present acutely with […]
• Link: Hashimoto Encephalopathy: A Rare Intricate Syndrome
  Recently, several patients have been reported with various signs of encephalopathy and high thyroid antibody levels together with good responsiveness to glucocorticoid therapy. Despite the various clinical presentations, these cases have been termed “Hashimoto encephalopathy” (HE). Although all of the pathogenic components have not yet been clearly elucidated, it is believed that brain vasculitis and […]
• Link: Re: TPO v. microsomal abs in Hashimoto's Encephalopathy
  This link will take you to a Listserv specifically for neurologists and clinicians discussing a range of neurological and diagnostic testing information. This tread leads to a discussion on Hashimoto’s Encephalopathy between clinicians. Re: TPO v. microsomal abs in Hashimoto’s Encephalopathy This website is not a substitute for independent professional advice. Nothing contained in this […]
• Howard S. Kirshner, M.D. - Professor of Neurology and Vice-Chairman
  Dr. Kirshner earned his B.A. from Williams College in 1968, graduating summa cum laude. He received his medical degree from Harvard Medical School in 1972, cum laude, and served as an intern in Medicine at Massachusetts General Hospital. Dr. Kirshner spent two years as a staff associate in the Laboratory of Perinatal Physiology at the […]
• Link: Hashimoto's encephalopathy: Report of three cases
  Both severe thyrotoxicosis and hypothyroidism may affect brain function and cause a change in consciousness, as seen with a thyroid storm or myxedema coma. However, encephalopathy may also develop in patients with autoimmune thyroid diseases independent of actual thyroid function level, and this is known as Hashimoto’s encephalopathy. Although most patients are found to have […]
• Link: Hashimoto's Encephalopathy Update
  First described in 1966, Hashimoto’s encephalopathy (HE) is still considered a controversial disorder, likely under-recognized, with diagnosis primarily based on exclusion. That is, if no other causes for the neurological symptoms are found and high titers of thyroid antibodies are present, a diagnosis of HE is made.   This article comes from the blog The […]
• 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 […]
• 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 […]
• Link: Cognitive and affective dysfunctions in autoimmune thyroiditis
  Hashimoto’s thyroiditis (HT) is the most frequent cause of hypothyroidism in areas with sufficient iodine intake. While the impact of thyroid function on mood and cognition is well known, only in the recent years, an increasing number of studies report on the association of HT with cognitive and affective disturbances also in the euthyroid state. […]
• Link: The biomarker and treatment in Hashimoto's encephalopahty
  All that can be found is the abstract from a Japanese study on Hashimoto’s Encephalopathy. Hashimoto’s encephalopathy (HE) is a treatable disease based on autoimmune mechanisms associated with Hashimoto’s thyroiditis. We recently discovered the serum autoantibodies (Abs) against the NH2-terminal of alpha-enolase (NAE) as a diagnostic biomarker for HE.   The abstract can be found […]
• Video: Epilepsy Autoimmune Evaluation
  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 […]
• Link: Hashimoto’s encephalopathy: A frequently missed diagnosis
  Hashimoto’s encephalopathy is described as a syndrome of encephalopathy and high serum antithyroid antibodies that is responsive to glucocorticoid therapy. We report a patient with overt hypothyroidism who presented with stroke-like episodes and a status epilepticus which responded dramatically to intraveous methylprednisolone given for a severe bronchospasm following aspiration. The high antithyroid antibody titers and […]
• Link: The use of plasma exchange in Hashimoto's encephalopathy: A case report and review of the literature.
  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.   The abstract can be found at unboundmedicine.com; however, the full […]
• Link: Pediatric Hashimoto's encephalopathy with peripheral nervous system involvement
  Hashimoto encephalopathy is a syndrome of encephalopathy associated with elevated concentration of circulating serum anti-thyroid antibodies usually responsive to steroid therapy. We report a 13-year-old girl with Hashimoto encephalopa- thy and peripheral nervous system involvement. The child had experienced high-grade pyrexia, global headache and sleeplessness. After admission she had an ileus with a distended urinary […]
• Link: Catatonia: A Duel of Hashimoto’s Encephalopathy and Anti NMDA Receptor Encephalitis (P5.166)
  OBJECTIVE To present a unique case of diagnostic conundrum in a catatonic patient positive for both anti NMDA receptor and anti TPO antibodies with a normal 24 hour EEG, brain MRI and CSF.BACKGROUND:Catatonia, although typically associated with schizophrenia and other affective disorders, can be an uncommon presenting feature of two rare neurologic disorders: Hashimoto’s encephalopathy […]
• Link: Severe complication of catatonia in a young patient with Hashimoto's encephalopathy comorbid with Cornelia de Lange syndrome
  Dear Editor, Cornelia de Lange syndrome (CdLs) is a rare genetic disease diagnosed as a cluster of symptoms; however, there are only a few reports mentioning the immunologic problems in patients with CdLs. Hashimoto’s encephalopathy (HE) is an autoimmune encephalitis, which manifests as neurocognitive impairment and elevated antithyroid antibody (ATA) titers. Childhood HE manifests especially […]
• Link: Hashimoto's Encephalopathy - A Brief Review
  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, […]
• Link: Hashimoto’s encephalopathy mimicking presenile dementia
  Abstract Hashimoto’s encephalopathy (HE), which carries kaleidoscopic clinical presentations, is easily misdiagnosed in clinical practice. Early diagnosis and prompt initiation of steroid therapy are associated with good prognosis. We describe a 50-year-old female patient who had subclinical hypothyroidism and who presented herself with gradual cognitive impairment, accompanied with auditory hallucination and delusion. Increased anti-thyroid antibodies […]
• Link: Anti-NMDAR encephalitis misdiagnosed as Hashimoto's encephalopathy
  Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a well-defined autoimmune disorder. Hashimoto’s encephalopathy (HE) is a still controversial entity, lacking definite diagnostic criteria. This study discusses a paediatric case of Autoimmune Encephalopathy with presentation similar to Hashimoto’s Encephalitis. The abstract only can be found at http://www.sciencedirect.com/science/article/pii/S1090379813001256. This article is only available by subscription or purchase.   Copyright © 2013 […]
• 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 […]
• Link: Types Of Autoimmune Encephalitis
  Autoimmune Encephalitis may be triggered by infection in which case the term “Post-infectious Encephalitis” is used. ADEM( Acute Disseminated Encephalomyelitis ) is a Post-infectious Encephalitis. The illness usually follows in the wake of a mild viral infection (such as those that cause rashes in childhood) or immunisations. Typically there is a delay of days to two […]
• Link: Antithyroid antibodies in the CSF: Their role in the pathogenesis of Hashimoto’s encephalopathy
   Abstract Antithyroid antibodies and circulating immune complexes (CIC) were found in the CSF of six patients with Hashimoto’s encephalopathy (HE) but not in the CSF of 21 controls. The synthesis of autoantibodies and CIC was intrathecal and their titers were independent of the patients’ clinical status or therapy. Their presence in the CSF of patients […]
• Link: Complex partial status epilepticus as a manifestation of Hashimoto's encephalopathy
  Epileptic seizures are a frequent manifestation of Hashimoto’s encephalopathy. However, status epilepticus associated with Hashimoto’s encephalopathy are not well characterized in medical literature. We described here a 16-year-old girl who presented with complex partial status epilepticus associated with elevated anti-thyroid antibodies. Ictal EEG showed lateralized high amplitude rhythmic delta waves over the right hemisphere and […]
• 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.

• Link: A Case of Refractory Hashimoto’s Encephalopathy Demonstrating Improvement with Plasmapheresis (P5.227)
  Objective To describe an unusual case of Hashimoto’s encephalopathy (HE) associated with significant imaging findings that responded to plasmapheresis. Background Hashimoto’s thyroiditis was first described by Hakaru Hashimoto in 1912 but it was Lord Brain who first noted the association between thyroiditis and an unusual encephalopathy in 1966 that he called Hashimoto’s encephalopathy. As it […]
• Link: Hashimoto's Encephalopathy Complicated by Cerebellar Ataxia: A Case Report
  

  To present a case of Hashimoto’s Encephalopathy (HE) with long-term neurological sequelae.BACKGROUND: Hashimoto’s Encephalopathy, also known as steroid responsive encephalopathy secondary to autoimmune thyroiditis (SREAT), is a rare condition that typically affects middle aged females with known anti-TPO antibodies and a history of Hashimoto’s thyroiditis. The diagnosis is often overlooked because there is no diagnostic biomarker, and patients can present with several non-specific neurological symptoms that are often incorrectly attributed to other disease processes. Neurological outcomes are rarely reported. It is important to recognize and distinguish HE from other encephalopathies as it is a distinct, treatable entity, which is almost always responsive to steroid therapy.

• Link: A follow-up 18F-FDG brain PET study in a case of Hashimoto's encephalopathy causing drug-resistant status epilepticus treated with plasmapheresis
  

  Hashimoto’s encephalopathy (HE) is a rare neuropsychiatric syndrome associated with antithyroid antibodies. It may have an acute onset (episodes of cerebral ischemia, seizure, and psychosis) or it may present as an indolent form (depression, cognitive decline, myoclonus, tremors, and fluctuations in level of consciousness). We here describe a case of encephalopathy presenting as non-convulsive status epilepticus associated with Hashimoto’s thyroiditis (HT), unresponsive to corticosteroid therapy, with improvement after plasma exchange treatment.

• Link: Hashimoto's Encephalitis: Unusual Cause of Reversible Dementia
  Abstract: Hashimoto’s encephalopathy (HE) is a poorly understood and often misdiagnosed rare autoimmune disease with varied neurological and psychiatric features. The low prevalence and varied clinical features coupled with unclear pathogenesis and histopathologic characteristics have caused still doubts in any particular diagnostic criteria. Therefore, more case studies are needed to characterize the clinical, laboratory and […]
• Link: Two patients with Hashimoto's encephalopathy and uncontrolled diabetes successfully treated with levetiracetam
  Abstract: Hashimoto’s encephalopathy (HE) is a rare syndrome of progressive or relapsing–remitting encephalopathy associated with elevated serum anti-thyroid antibody concentrations. It is thought to be an autoimmune process that generally responds well to high-dose corticosteroids and other immunomodulatory therapies. However, some patients are unresponsive to steroids or are unable to receive immune therapy. A viable […]
• Link: Clinical and neuropathological findings in Hashimoto’s encephalopathy: a case report
  

  Hashimoto’s encephalopathy (HE) is a controversial autoimmune condition of the central nervous system (CNS) characterized by focal and/or diffuse symptoms concomitant with raised levels of anti-thyroid antibodies, in particular anti-thyroperoxidase (TPO) and/or anti-thyroglobulin (TG) antibodies [1]. Hereby, we provide the full clinical and neuropathological description of an HE patient with a rapid and fatal course.

• PDF Link: Hashimoto’s Encephalopathy Presenting as Acute Psychosis
  Abstract: Hashimoto’s encephalopathy is a relapsing encephalopathy occurring in association with Hashimoto’s thyroiditis, with high titers of anti-thyroid antibodies. Clinically the patients may presents with acute or subacute encephalopathy, seizure, myoclonus, and tremulousness, stroke like episode, amnesia or dementia. Here we are reporting a case of hashimoto’s encephalopathy who presented with features of acute psychosis. […]
• PDF Link: Autoimmune encephalitis - History & current knowledge
  This PDF link will take you to a PDF published by PANDAS Network and has a wealth of information about auto-immune diseases.   Introduction The acute fulminating epidemic form of encephalitis lethargica (von Economo, EL) seems to have disappeared, since there have been no further reported epidemics after 1916-1927. During that period of time, both […]
• Link: Hashimoto's Encephalitis by by Dr. Sarosh Irani, John Radcliffe Hospital, Oxford
  

  Hashimoto’s Encephalopathy (HE) was first described in 1966. It is a rare condition, which is probably of autoimmune origin. Autoimmunity describes disorders in which the immune system mistakenly attacks and destroys the body’s tissues. HE is usually defined by the presence of high levels of thyroid antibodies in the blood. HE has been reported in all age groups but typically affects females of around 50 years of age. – The Encephalitis Society

• Link: Pathophysiological decrease in the regional cerebral blood flow in Hashimoto's encephalopathy: a multiple-case SPECT study.
  

  Seven untreated patients with HE and 10 age-matched healthy controls underwent brain single photon emission computed tomography (SPECT) with N-isopropyl-p-[(123)I]iodoamphetamine. All patients had anti-NH2-terminal of α-enolase autoantibodies (Abs), which served as a useful diagnostic marker for HE, in addition to anti-thyroid Abs in their sera and responded to corticosteroid therapy. The obtained SPECT images were compared between the patients and the controls using 3D-SSP analysis.

• PDF Link: Autoantibodies Associated with Diseases of the CNS
  Linked is a lengthy PDF article regarding multiple auto-immune diseases affecting the Central Nervous System. Several CNS disorders associated with specifi c antibodies to ion channels, receptors, and other synaptic proteins have been recognised over the past 10 years, and can be often successfully treated with immunotherapies. Antibodies to components of voltage-gated potassium channel complexes […]
• Link: Autoimmune Encephalopathy
  This MedScape article in “seminars in neurology”, written by Eoin P. Flanagan, M.B.B.Ch., Richard J. Caselli, M.D. for the Department of Neurology, Mayo Clinic, Rochester, Minnesota; and Department of Neurology, Mayo Clinic, Scottsdale, Arizona., and discusses non-paraneoplastic encephalopathy. It focuses on the global effects of auto-immune encephalopathy laboratory testing, imaging tools, anti-body testing, ruling out […]
• Link: Encefalopatía de Hashimoto
  The article discusses two forms of presentation, “vasculitic” which is remitting/relapsing and “progressive” which is characterized by confussion, psychosis, and potentially coma. The original article, published in Spanish, is located at http://www.imedicinas.com/pfw_files/cma/ArticulosR/Neurologia/2002/10/109100206280632.pdf. The full text can be translated at https://translate.google.com/. The full text of this article is found at imedicinas.com and was published in 2002. […]
• Link: Steroid-responsive Encephalopathy in Autoimmune Thyroiditis: Clinical Spectrum and MRI Observations in Three Cases
  This study looks at three cases of Hashimoto’s Encephalopathy, discussing MRI and EEG observations in each case. To read the full text, proceed to http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2077662/. The full text of this article is found at PMC and was published in 2011. Copyright © Annals of Indian Academy of Neurology   Is there a tag missing? Suggest […]
• Link: Hashimoto’s Encephalopathy Cases: Chinese Experience
  This study discusses Hashimoto’s Encephalopathy and it’s presentation of multiple neurologic symptoms and high anti-body titer. It specifically focuses on the Chinese population but may be of some interest for the purpose of personal research.   The full text of this article is found at BMC Neurology. Copyright © 2012 Tang et al.; licensee BioMed […]
• Link: Hashimoto's Encephalopathy Presenting with Progressive Cerebellar Ataxia
  

  Hashimoto’s encephalopathy is an autoimmune encephalopathy that came to be regarded as a new clinical entity distinct from myxo‐oedema encephalopathy, associated with Hashimoto’s thyroiditis. – US National Library of Medicine National Institutes of Health