What is Hashimoto's encephalopathy?
Hashimoto's encephalopathy. Hashimoto's encephalopathy, also known as steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a neurological condition characterized by encephalopathy, thyroid autoimmunity, and good clinical response to steroids.
Is Hashimoto’s an autoimmune disease?
Some researchers speculate that the autoimmune process may involve inflammation and damage to blood vessels within the brain (autoimmune cerebral vasculitis). However, more research is necessary to determine the exact, underlying mechanisms that cause Hashimoto encephalopathy. Affected Populations
Is Hashimoto's a neuroendocrine disorder?
It is 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. It is recognized as a rare disease by the NIH Genetic and Rare Diseases Information Center.
What are the complications of Hashimoto's disease?
Complications. Hashimoto's disease can also cause sexual desire (libido) to decrease in both men and women and can lead to slowed mental functioning. Myxedema (miks-uh-DEE-muh). This rare, life-threatening condition can develop due to long-term hypothyroidism as a result of untreated Hashimoto's disease.
Can Hashimoto's cause brain damage?
Because there is no evidence that the antithyroid antibodies in Hashimoto encephalopathy contribute to brain damage, and because most of the affected individuals have a normal-functioning thyroid, some researchers believe these are coincidental findings.
Can Hashimoto's thyroiditis cause death?
Is Hashimoto's disease dangerous or fatal? If left untreated, hypothyroidism can lead to some serious complications and, in rare cases, death. These include: Heart problems, such as enlarged heart or heart failure.
Is Hashimoto's Encephalopathy the same as Hashimoto's disease?
Hashimoto encephalopathy (HE) is an uncommon syndrome associated with Hashimoto thyroiditis (HT). "Steroid-responsive encephalopathy associated with autoimmune thyroiditis" (SREAT) and a more general term, "nonvasculitic autoimmune meningoencephalitis," are also used to describe this condition.
What is end stage hashimotos?
Overt hypothyroidism or full-blown disease The end-stage of Hashimoto's is when your thyroid has become so damaged that you no longer have enough thyroid hormones and have to go on medication.
How is Hashimoto's encephalopathy treated?
Treatment. The primary treatment for Hashimoto's encephalopathy is oral corticosteroid drugs, usually prednisone, or intravenous (IV) Medrol (methylprednisolone). 3 Most people respond quickly and well to drug treatment, with their symptoms improving or even resolving within a few months.
Does Hashimoto's shorten life expectancy?
With effective treatment and routine follow-ups with a physician, those with Hashimoto's disease can lead a long, healthy life.
What are the symptoms of Hashimoto's encephalopathy?
Hashimoto encephalopathy (HE) affects the brain and how the brain works. Symptoms of HE may include behavior changes, confusion, cognitive difficulty, and seizures. Psychosis, including visual hallucinations and paranoid delusions, has also been reported. HE occurs mainly in adults and affects females more than males.
How fast does Hashimoto's progress?
It takes an average of 10 years to be diagnosed with Hashimoto's between the start of the autoimmune attack on the thyroid gland, and when the person is diagnosed.
How serious is Hashimoto's disease?
If Hashimoto's is left untreated, complications can be life-threatening. Because the hormones produced by the thyroid are so vital to the body's functions, untreated Hashimoto's can lead to serious and even life-threatening complications.
What other diseases can Hashimoto's cause?
Hashimoto's disease is an autoimmune disorder that can cause hypothyroidism, or underactive thyroid. Rarely, the disease can cause hyperthyroidism, or overactive thyroid.
Can I get disability for Hashimoto's?
Hashimoto's Disease is not included in the SSA's listing of conditions that qualify for Social Security disability benefits. Even though it is not considered a disability by the SSA, if you have Hashimoto's Disease and it affects your ability to work full time, you could qualify for Social Security disability benefits.
What can Hashimoto's lead to?
Hashimoto's thyroiditis can cause your thyroid to not make enough thyroid hormone. It is an autoimmune disease. It occurs when your body makes antibodies that attack the cells in your thyroid. Symptoms may include an enlarged thyroid gland (goiter), tiredness, weight gain, and muscle weakness.
How severe is Hashimoto's?
This rare, life-threatening condition can develop due to long-term, severe, untreated hypothyroidism. Its signs and symptoms include drowsiness followed by profound lethargy and unconsciousness. A myxedema coma may be triggered by exposure to cold, sedatives, infection or other stress on your body.
What happens if you don't treat Hashimoto's?
If left untreated, hypothyroidism caused by Hashimoto disease can lead to serious complications: Goiter, which can interfere with swallowing or breathing. Heart problems such as enlarged heart or heart failure. Mental health issues such as depression, decreased sexual desire, slowed mental functioning.
What does Hashimoto's disease do to a person?
Hashimoto's thyroiditis can cause your thyroid to not make enough thyroid hormone. It is an autoimmune disease. It occurs when your body makes antibodies that attack the cells in your thyroid. Symptoms may include an enlarged thyroid gland (goiter), tiredness, weight gain, and muscle weakness.
Can you live a normal life with Hashimoto's?
However, even though Hashimoto's disease and the hypothyroidism it causes can have widespread effects on your mind and body, it doesn't need to control your life. With good treatment, a healthy lifestyle, and a strong support system, you can still live a full and happy life even with chronic disease.
What is the autoimmune registry?
The Autoimmune Registry supports research for Hashimoto encephalopathy by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.
How to treat Hashimoto's encephalopathy?
Treatment of Hashimoto encephalopathy (HE) is focused on managing the symptoms. Treatment usually involves medications such as corticosteroids and treatment of thyroid abnormalities (if present). Most people with HE respond to steroid therapy. People who do not respond to steroids and/or cannot tolerate steroid treatment have been treated with other immunosuppressive medications or intravenous immunoglobulin therapy. [1] [4]
What is the diagnosis of Hashimoto encephalopathy?
Diagnosis of Hashimoto encephalopathy (HE) is based on the symptoms, clinical exam, and specific laboratory testing. Imaging tests such as an MRI or a CT scan may be helpful. Other more common conditions may need to be excluded before a diagnosis of HE can be made.
How many people have Hashimoto's?
There have been at least 200 cases of Hashimoto encephalopathy reported in the medical literature. It has been estimated that about 2 in 100,000 people has Hashimoto encephalopathy. [1] [4]
What are the symptoms of Hashimoto's disease?
Hashimoto encephalopathy (HE) affects the brain and how the brain works. Symptoms of HE may include behavior changes, confusion, cognitive difficulty, and seizures. Psychosis, including visual hallucinations and paranoid delusions, has also been reported. HE occurs mainly in adults and affects females more than males.
What is the HPO database?
People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources.
What is support and advocacy?
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services.
What is HE in medical terms?
Hashimoto's encephalopathy (HE) is a rare disease that involves impaired brain function (encephalopathy). The condition is named for its possible association with Hashimoto's thyroiditis, a common autoimmune thyroid disease, but the exact cause is not fully understood. 1 HE contributes to neurological issues such as seizures, confusion, or dementia.
What is the diagnosis of Creutzfeldt-Jakob disease?
4. Diagnosis consists of first ruling out other known causes of encephalopathy, then checking for the presence of specific antibodies (the immune system's "attack" cells) and thyroid dysfunction.
What is the treatment for Hashimoto's disease?
Treatment. The primary treatment for Hashimoto's encephalopathy is oral corticosteroid drugs, usually prednisone, or intravenous (IV) Medrol (methylprednisolone). 3 Most people respond quickly and well to drug treatment, with their symptoms improving or even resolving within a few months. The 2016 review found that 91% of participants responded ...
How does Hashimoto's affect the brain?
Hashimoto's encephalopathy affects how your brain functions, causing cognitive decline and altered consciousness. It usually progresses in one of three ways: 2. Relapsing and remitting: Episodes of acute symptoms come and go, often involving seizures and stroke-like attacks.
How old is Hashimoto's?
The average age of onset of symptoms of Hashimoto's encephalopathy is around 40 to 55 years old. Women are diagnosed with it about four times more often than men. 1 HE can also affect children, but that's believed to be rare.
How rare is Hashimoto's encephalopathy?
Hashimoto's encephalopathy is considered rare, affecting just two people out of every 100,000. However, it's likely that many more cases are undiagnosed or misdiagnosed since the condition isn't well-understood or highly recognized. 1. The average age of onset of symptoms of Hashimoto's encephalopathy is around 40 to 55 years old.
What is progressive cognitive decline?
Progressive: Cognitive decline gradually progresses into dementia, hallucinations, confusion, sleep-wake cycle impairment, or even a coma.
What is SREAT in medical terms?
Specialty. Neurology. Hashimoto's encephalopathy, also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis ( SREAT ), is a neurological condition characterized by encephalopathy, thyroid autoimmunity, and good clinical response to corticosteroids. It is associated with Hashimoto's thyroiditis, ...
What is relapsing encephalopathy?
Definition. A relapsing encephalopathy occurs in association with Hashimoto's thyroiditis, with high titers of antithyroid antibodies. Clinically, the condition may present one or more symptoms. Onset is often gradual and may go unnoticed by the patient and close associates to the patients.
How long does it take for Hashimoto's encephalopathy to show symptoms?
Signs and symptoms. The onset of symptoms tends to be fairly gradual and to occur over 1-12 years. Symptoms of Hashimoto's encephalopathy may include: Personality changes. Aggression. Delusional behavior. Concentration and memory problems. Coma. Disorientation.
How long does it take for steroid-responsive encephalopathy to resolve?
Initial treatment is usually with oral prednisone (50–150 mg/day) or high-dose intravenous methylprednisolone (1 g/day) for 3–7 days.
How many cases of Hashimoto's were published?
Up to 2005, almost 200 case reports of this disease were published. Between 1990 and 2000, 43 cases were published. Since that time, research has expanded and numerous cases are being reported by scientists around the world, suggesting that this rare condition is likely to have been significantly undiagnosed in the past. Over 100 scientific articles on Hashimoto's encephalopathy were published between 2000 and 2013.
What is the prevalence of a syphilis?
The prevalence has been estimated to be 2.1/100,000 with a male-to-female ratio of 1:4. The mean age of onset is 44 with 20% of cases presenting before the age of 18 years. Most reported cases occur during the patient's fifth decade of life.
How long does steroid treatment last?
Duration of treatment is usually between 2 and 25 years. Earlier reports suggested that 90% of cases stay in remission after discontinuation of treatment, but this is at odds with more recent studies, which suggest that relapse commonly occurs after initial high-dose steroid treatment.
What is the autoimmune registry?from rarediseases.info.nih.gov
The Autoimmune Registry supports research for Hashimoto encephalopathy by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.
What is an antithyroid antibody?from rarediseases.org
Antithyroid antibodies are ones that mistakenly target thyroid tissue. However, it is unclear whether these antibodies play any role in the development of Hashimoto encephalopathy or are a coincidental finding. The main signs and symptoms are related to the encephalopathy.
What is HE in medical terms?from uptodate.com
Hashimoto encephalopathy (HE) is an uncommon syndrome associated with Hashimoto thyroiditis (HT). "Steroid-responsive encephalopathy associated with autoimmune thyroiditis" (SREAT) and a more general term, "nonvasculitic autoimmune meningoencephalitis," are also used to describe this condition. Originally described in 1966, it remains ...
What is a HE?from uptodate.com
Hashimoto encephalopathy (HE) is an uncommon syndrome associated with Hashimoto thyroiditis (HT). "Steroid-responsive encephalopathy associated with autoimmune thyroiditis" (SREAT) and a more general term, "nonvasculitic autoimmune meningoencephalitis," are also used to describe this condition.
What is the enzyme that is expressed in most tissues of the body?from rarediseases.org
Alpha enolase is an enzyme expressed in most tissues of the body. The presence of two autoantibodies, anti-alpha enolase and antithyroid antibodies, supports the theory that there is an active autoimmune process affecting individuals with Hashimoto encephalopathy.
How many people are affected by Hashimoto encephalopathy?from rarediseases.org
Affected Populations. Hashimoto encephalopathy is a rare disease that affects women more often than men. The disorder is estimated to affect 2.1 per 100,000 individuals in the general population. It can affect children, but only approximately 60 affected children have been described in the medical literature.
What is Hashimoto encephalopathy?from rarediseases.org
General Discussion. Hashimoto encephalopathy is a rare disorder characterized by impaired brain function (encephalopathy). The exact cause is unknown, but it is believed to be an immune-mediated disorder or a disorder in which there is inflammation resulting from abnormal functioning of the immune system. Affected individuals have the presence of ...
Abstract
Hashimoto's encephalopathy is a rare disease with nonspecific symptoms, associated with elevated levels of anti‐TPO and/or anti‐TG. It can be potentially fatal. However, it is responsive to steroid and treated in due time, it can be fully reversible.
AUTHOR CONTRIBUTION
Ronja MB Lagström wrote the first draft of the article, collected comments from co‐authors, and prepared the final version. Natascha N Østerbye was responsible for the neuropsychology, prepared the table on neuropsychometrical data, and revised the draft.
REFERENCES
1. Laurent C, Capron J, Quillerou B, et al. Steroid‐responsive encephalopathy associated with autoimmune thyroiditis (SREAT): characteristics, treatment and outcome in 251 cases from the literature. Autoimmun Rev. 2016;15:1129‐1133. [ PubMed] [ Google Scholar]
Authors
Ronja Maria Birgitta Lagström 1 2 , Natascha Nellum Østerbye 1 3 , Otto Mølby Henriksen 4 , Peter Høgh 1 5
Affiliations
1 Department of Neurology Regional Dementia Research Center, Zealand University Hospital Roskilde Denmark.
How does Hashimoto's disease affect thyroid?
Hashimoto's disease typically progresses slowly over years and causes chronic thyroid damage, leading to a drop in thyroid hormone levels in your blood. The signs and symptoms are mainly those of an underactive thyroid gland (hypothyroidism). Signs and symptoms of hypothyroidism include: Fatigue and sluggishness. Increased sensitivity to cold.
What causes a virus to attack the thyroid gland?
Hashimoto's disease is an autoimmune disorder in which your immune system creates antibodies that damage your thyroid gland. Doctors don't know what causes your immune system to attack your thyroid gland. Some scientists think a virus or bacterium might trigger the response, while others believe a genetic flaw may be involved.
What is the most common cause of hypothyroidism?
The thyroid gland is part of your endocrine system, which produces hormones that coordinate many of your body's functions. Inflammation from Hashimoto's disease , also known as chronic lymphocytic thyroiditis, often leads to an underactive thyroid gland (hypothyroidism). Hashimoto's disease is the most common cause of hypothyroidism.
What are the symptoms of a coma?
Its signs and symptoms include drowsiness followed by profound lethargy and unconsciousness. A myxedema coma may be triggered by exposure to cold, sedatives, infection or other stress on your body. Myxedema requires immediate emergency medical treatment. Birth defects.
Where is the thyroid gland located?
Thyroid gland. Your thyroid gland is located at the base of your neck, just below the Adam's apple. Hashimoto's disease is a condition in which your immune system attacks your thyroid, a small gland at the base of your neck below your Adam's apple. The thyroid gland is part of your endocrine system, which produces hormones that coordinate many ...
What are the causes of Hashimoto's disease?
Radiation exposure. People exposed to excessive levels of environmental radiation are more prone to Hashimoto's disease.
When does Hashimoto's disease occur?
Hashimoto's disease can occur at any age but more commonly occurs during middle age. Heredity. You're at higher risk for Hashimoto's disease if others in your family have thyroid or other autoimmune diseases. Other autoimmune disease.
Hashimoto's Encephalopathy Symptoms
- Hashimoto's encephalopathy affects how your brain functions, causing cognitive decline and altered consciousness. It usually progresses in one of three ways:2 1. Relapsing and remitting: Episodes of acute symptoms come and go, often involving seizures and stroke-like attacks. 2. Progressive: Cognitive decline gradually progresses into dementia, hal...
Causes
- It's unknown what exactly causes Hashimoto's encephalopathy, but scientists think that like Hashimoto's thyroiditis, HE is an autoimmune condition, meaning that the immune system mistakenly targets your body's own tissues as if they were a virus or bacteria. In the case of HE, the target is the brain. In Hashimoto's thyroiditis, the immune system attacks the thyroid gland, c…
Diagnosis
- Currently, healthcare providers don't have a definitive test for Hashimoto's encephalopathy. Because its symptoms primarily affect your brain, HE is easy to misdiagnose or overlook. People are sometimes misdiagnosed with Creutzfeldt-Jakob disease, dementia, Alzheimer's disease, or stroke.4 Diagnosis consists of first ruling out other known causes of encephalopathy, then chec…
Treatment
- The primary treatment for Hashimoto's encephalopathy is oral corticosteroid drugs, usually prednisone, or intravenous (IV) Medrol (methylprednisolone).3Most people respond quickly and well to drug treatment, with their symptoms improving or even resolving within a few months. The 2016 review found that 91% of participants responded completely or by at least 50% to steroid tr…
Prognosis
- Like most autoimmune disorders, Hashimoto's encephalopathy is not considered curable, but rather treatable. The prognosis is generally good. After initial treatment, the disorder often lapses into remission. Some patients are able to discontinue drug therapy for a number of years. There is a risk of future relapse, but in the 2016 review, only 16% percent of patients had one or more rela…
Frequently Asked Questions
- Which type of healthcare providers treats Hashimoto encephalopathy?
Neurologists are often the best-equipped specialists to diagnose and oversee treatment for Hashimoto encephalopathy.12 Other healthcare providers who may be involved in diagnosis and management of this autoimmune condition include rheumatologists, psychiatrists, psychologist… - Is Hashimoto encephalopathy a form of dementia?
Dementia is a symptom of Hashimoto encephalopathy (HE). HE may cause rapidly progressive dementia or dementia of unknown origin, but the cognitive changes may be reversible with proper treatment.13
A Word from Verywell
- Hashimoto's encephalopathy can create a bit of a diagnostic challenge. Fortunately, most people respond well to treatment, even if they aren't diagnosed for several years. If you or someone close to you develops acute neurocognitive symptoms that your healthcare provider can't explain, be sure to mention any personal or family history of Hashimoto's disease or other thyroid condition…
Overview
Hashimoto's encephalopathy, also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a neurological condition characterized by encephalopathy, thyroid autoimmunity, and good clinical response to corticosteroids. It is associated with Hashimoto's thyroiditis, and was first described in 1966. It is sometimes referred to as a neuroendocrine dis…
Signs and symptoms
The onset of symptoms tends to be fairly gradual and to occur over 1-12 years.
Symptoms of Hashimoto's encephalopathy may include:
• Personality changes
• Aggression
• Delusional behavior
Pathogenesis
The mechanism of pathogenesis is not known, but is thought to be an autoimmune disorder, similar to Hashimoto's thyroiditis, as its name suggests.
Consistent with this hypothesis, autoantibodies to alpha-enolase have been found to be associated with Hashimoto's encephalopathy. Since enolase is the penultimate step in glycolysis, if it were inhibited (for example by being bound by autoantibodies), one would expect decreased energy p…
Diagnosis
• Increased liver enzyme levels (55% cases)
• Increased thyroid-stimulating hormone (55% cases)
• Increased erythrocyte sedimentation rate (25% cases)
Cerebrospinal fluid findings:
Treatment
Because most patients respond to corticosteroids or immunosuppressant treatment, this condition is now also referred to as steroid-responsive encephalopathy.
Initial treatment is usually with oral prednisone (50–150 mg/day) or high-dose intravenous methylprednisolone (1 g/day) for 3–7 days. Thyroid hormone treatment is also included if required.Failure of some patients to respond to this first-line treatment has produced a variety o…
Prognosis
Duration of treatment is usually between 2 and 25 years. Earlier reports suggested that 90% of cases stay in remission after discontinuation of treatment, but this is at odds with more recent studies, which suggest that relapse commonly occurs after initial high-dose steroid treatment. Left untreated, this condition can result in coma and death.
Epidemiology
The prevalence has been estimated to be 2.1/100,000 with a male-to-female ratio of 1:4. The mean age of onset is 44 with 20% of cases presenting before the age of 18 years. Most reported cases occur during the patient's fifth decade of life.
History
The first case of HE was described by Brain et al. in 1966. The patient was a 48-year-old man with hypothyroidism, multiple episodes of encephalopathy, stroke-like symptoms, and Hashimoto's thyroiditis confirmed by elevated antithyroid antibodies.