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Hashimoto encephalopathy, also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis, has been defined by sub-acute onset encephalopathy, with elevated thyroid antibodies, and immunotherapy responsiveness, in the absence of specific neural autoantibodies. We aimed to retrospectively review 144 cases referred with suspected Hashimoto encephalopathy over a 13-year period, and to determine the clinical utility of thyroid antibodies in the course of evaluation of those patients. One hundred and forty-four patients (all thyroid antibody positive) were included; 72% were women. Median age of symptom onset was 44.5 years (range, 10–87). After evaluation of Mayo Clinic, 39 patients (27%) were diagnosed with an autoimmune CNS disorder [autoimmune encephalopathy (36), dementia (2) or epilepsy (1)]. Three of those 39 patients had neural-IgGs detected (high glutamic acid decarboxylase-65, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor-receptor and neural-restricted unclassified antibody), and 36 were seronegative. Diagnoses among the remaining 105 patients (73%) were functional neurological disorder (n = 20), neurodegenerative disorder (n = 18), subjective cognitive complaints (n = 14), chronic pain syndrome (n = 12), primary psychiatric (n = 11), sleep disorder (n = 10), genetic/developmental (n = 8), non-autoimmune seizure disorders (n = 2) and other (n = 10). More patients with autoimmune CNS disorders presented with sub-acute symptom onset (P, Valencia-Sanchez et al. reviewed the spectrum of cases referred to their Autoimmune Neurology Clinic with suspected Hashimoto encephalopathy, and found that almost three-quarters had an alternative non-autoimmune CNS diagnosis. They conclude that thyroid antibodies are of little utility in the evaluation of autoimmune encephalopathy in contemporary clinical practice., Graphical Abstract Graphical Abstract

Autoimmune encephalopathy is one of the treatable causes of rapidly progressive dementia; however, it is often underdiagnosed. Autoantibodies against voltage-gated calcium channel (VGCC) have been linked to several neurological disorders, including Lambert-Eaton syndrome, but VGCC antibody-associated encephalopathy is uncommon. Herein, we present a case of a 74-year-old woman with prominent neuropsychiatric symptoms followed by rapid cognitive decline. Extensive initial studies were nondiagnostic. Subsequently, serum N-type VGCC antibody was positive. After treatment with intravenous immunoglobulin, the patient's cognition and neuropsychiatric symptoms significantly improved.

Hashimoto's encephalopathy (HE) is a rare, clinically heterogeneous condition associated with positive thyroid autoantibodies. It is increasingly recognized as an important and treatable cause of autoimmune encephalopathy. Thyroid-associated antibodies such as thyroperoxidase (TPO) antibody, thyroglobulin (TG) antibody, and thyrotropin receptor (TR) antibody were found in HE patients with seizure disorders. Although antithyroid antibodies are required for the diagnosis of HE, their role in the pathogenesis of HE remains uncertain. Instead of playing a key role in the pathophysiology processes of HE, it is suggested that thyroid-associated antibodies are hallmarks of HE. Seizure disorders were found in approximately two-thirds of HE patients, and common anticonvulsant therapy alone is usually ineffective. Some patients did not respond to any antiepileptic drugs. The use of immunotherapy can effectively control seizure disorders. Electroencephalography and imaging findings are not specific to HE patients and can also be seen in other causes of encephalopathies. However, the prognosis in the majority of patients with HE was usually good if it is diagnosed and treated correctly.

Over the past two decades, the discovery of various antibodies related to autoimmune encephalitis has given us a new insight into pathogenic mechanisms and treatment of these syndromes. That is important since many patients with autoimmune encephalopathy are children and young adults. These patients often respond favorably to immunotherapy. Delay in diagnosis and treatment has been associated with a worse prognosis. In this chapter, we review the epidemiology, pathophysiology, clinical characteristics, diagnosis, and treatment of different syndromes associated with autoimmune encephalitis.

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory central nervous system syndrome, and encephalitis associated with anti-N-methyl-d-aspartate receptor (NMDAR) antibodies is an autoimmune encephalopathy. A patient with both diseases, separately or simultaneously, is rare as a clinical phenomenon, but cannot be ignored. We report the clinical characteristics and imaging features of a special case with anti-NMDAR encephalitis followed by NMOSD. We subsequently reviewed the English language literature about demyelinating disorders with anti-NMDAR encephalitis. Details of the 34 patients identified are summarized and compared. There may be a connection between anti-NMDAR encephalitis and NMOSD.

The understanding of the etio-pathogenesis of autoimmune encephalopathy syndromes has grown significantly in recent years. These are potentially reversible conditions, with variable clinical presentation and potential dramatic response to immunotherapy.In this article we review various diagnostic techniques and therapeutic options for management of autoimmune encephalopathy. We also review medication adverse effects and monitoring strategies. Expert commentary: Early diagnosis and immunomodulatory treatment remains the cornerstone of management, to halt the underlying neuro-inflammatory process and prevent permanent neuronal injury. The availability of serological testing and various imaging modalities has further improved detection of these immune-mediated neurological disorders. Understanding the mechanisms and potential adverse effects of immunomodulatory therapies will help physicians to choose the most favorable therapeutic strategy for each patient.

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.

Encephalitis is an acute inflammatory condition of the brain caused by infective or autoimmune mechanisms. It typically involves headache, fever, confusion, seizures, and sensory or motor disturbance. Recently there have been increased understandings of the role of neuronal cell antibodies in presentations of encephalitis that have psychiatric features only.

Rad opisuje prikaz slučaja bolesnice sa sindromom SREAT i kliničkom slikom mioklosu-distonije sindroma. Dodatno je uključen i presjek dotadašnje literature prikaza slučajeva sa SREAT sindromom.

Over the last 15 years it has become clear that rare but highly recognizable diseases of the central nervous system (CNS), including newly identified forms of limbic encephalitis and other encephalopathies, are likely to be mediated by antibodies (Abs) to CNS proteins. The Abs are directed against membrane receptors and ion channel-associated proteins that are expressed on the surface of neurons in the CNS, such as N-methyl D-aspartate receptors and leucine-rich, glioma inactivated 1 protein and contactin-associated protein like 2, that are associated with voltage-gated potassium channels. The diseases are not invariably cancer-related and are therefore different from the classical paraneoplastic neurological diseases that are associated with, but not caused by, Abs to intracellular proteins. Most importantly, the new antibody-associated diseases almost invariably respond to immunotherapies with considerable and sometimes complete recovery, and there is convincing evidence of their pathogenicity in the relatively limited studies performed so far. Treatments include first-line steroids, intravenous immunoglobulins, and plasma exchange, and second-line rituximab and cyclophosphamide, followed in many cases by steroid-sparing agents in the long-term. This review focuses mainly on N-methyl D-aspartate receptor- and voltage-gated potassium channel complex-related Abs in adults, the clinical phenotypes, and treatment responses. Pediatric cases are referred to but not reviewed in detail. As there have been very few prospective studies, the conclusions regarding immunotherapies are based on retrospective studies. Electronic supplementary material The online version of this article (doi:10.1007/s13311-015-0410-6) contains supplementary material, which is available to authorized users.