Definition
Hashimoto's encephalopathy is a rare disease involving brain damage. This disease affects 2 out of 100,000 people. It is named "Hashimoto's encephalopathy" because it is believed to be related to Hashimoto's thyroiditis, an autoimmune disease that affects the thyroid gland in the neck.
Hashimoto's encephalopathy affects the brain and its function. In Hashimoto's disease, the body's immune cells attack the thyroid gland cells that produce hormones. This disease is quite rare, with an estimated number of cases less than 1000 in the United States. Hashimoto's encephalopathy mostly occurs in adults and is more commonly found in women than in men.
Causes
The exact cause of Hashimoto's encephalopathy is not clearly understood. There is a hypothesis suggesting the presence of autoimmune vasculitis (inflammation of blood vessels due to the body's immune response) that attacks thyroid gland antigens and is found in the brain. Although there are reported cases of Hashimoto's encephalopathy, its cause remains entirely unknown.
Hashimoto's thyroiditis is a chronic inflammation of the thyroid gland because thyroid cells are destroyed by the body's immune cells. In encephalopathy, thyroid inflammation leads to brain damage, disrupting brain function. In 1991, there were five reported cases of encephalopathy and Hashimoto's thyroiditis. These patients experienced a decrease in consciousness and seizures. Laboratory results showed the presence of antibodies (proteins that protect against disease) against thyroid cells. Treatment was successful with steroids, drugs to reduce the inflammatory response.
Risk factor
Hashimoto's encephalopathy only occurs in 2 out of 100,000 people, indicating that this condition is very rare. Risk factors for this disease include autoimmune diseases such as Hashimoto's thyroiditis. This autoimmune disease causes inflammation of thyroid cells, leading to a decrease in thyroid hormone levels (hypothyroidism) in the body.
The average age of patients with Hashimoto's encephalopathy is around 40-55 years. Women are diagnosed with this disease four times more often than men.
Symptoms
Hashimoto's encephalopathy affects brain function, so it can cause a decline in cognitive function and consciousness. Symptoms may also vary from person to person. Symptoms of the disease can range from mild to severe, and some patients may have more symptoms than others.
The most common symptoms of Hashimoto's encephalopathy include:
- Headaches.
- Confusion.
- Stroke-like episodes.
- Neurological and cognitive disturbances.
- Ataxia (poor muscle control, a decrease in muscle coordination causing movement and balance disturbances).
- Seizures resistant to antiepileptic drugs.
In addition to the above symptoms, other symptoms may include:
- Behavioral changes and emotional instability.
- Speech problems.
- Decreased concentration and attention.
- Visual hallucinations and paranoid delusions.
- Excessive reflexes.
- Brain ischemia (decreased blood flow to specific parts of the brain).
- Decreased consciousness.
- Tremors (body parts trembling).
- Disorientation or inability to recognize the surrounding environment.
- Concentration and memory problems.
- Myoclonus, sudden muscle twitches or stiffness.
Diagnosis
The diagnosis of Hashimoto's encephalopathy is based on the signs and symptoms experienced and shown by the patient, as well as supported by the presence of antibodies against thyroid cells.
- Medical Interview: The doctor will ask for the patient's complete medical history.
- Physical Examination: Doctors conducting physical examinations may find signs of ataxia in gait, but may also find no symptoms at all.
- Supporting Examinations: Examinations that lead to Hashimoto's encephalopathy include laboratory tests and imaging.
Management
The management of encephalopathy is treatment with medications such as steroids. Steroids are usually given for a year to achieve good results. About 98% of patients treated with steroids improve without leaving residual symptoms.
Another treatment for Hashimoto's encephalopathy is intravenous immunoglobulin (IVIG), which involves antibodies from healthy individuals being injected into the blood to attack excess antibodies in the body. Additionally, plasma exchange can also be performed as advanced therapy. Plasma exchange (plasma exchange, PE) is performed by removing plasma from your body and replacing it with healthy plasma to reduce the number of antibodies against thyroid cells.
Complications
Like many other autoimmune diseases, therapy aims to alleviate the symptoms experienced by patients. The possibility of patient recovery is very good. After receiving initial therapy, this disease usually goes into remission or temporary recovery. Some patients may not even continue drug therapy for years. This disease has a risk of relapse or recurrence. However, this research shows that only 16% of patients may experience recurring symptoms. Typically, these patients are those who experience Hashimoto's encephalopathy until they reach a coma.
Prevention
Prevention is difficult because the incidence of Hashimoto's encephalopathy is very rare, even in patients with Hashimoto's thyroiditis. Patients with Hashimoto's thyroiditis are advised to regularly consult a doctor so that their disease can be well controlled and not cause brain disturbances.
When to see a doctor?
Hashimoto's disease can develop slowly over time. Hashimoto's thyroiditis can cause a decrease in hormone production which can lead to symptoms such as:
- Sensitivity to cold temperatures.
- Increasingly frequent drowsiness.
- Fatigue and slowness.
- Muscle weakness, pain, or stiffness.
- Dry skin.
- Joint pain and stiffness.
- Hair loss.
- Swelling of the thyroid gland in the neck, etc.
You are advised to visit a doctor if you experience any of the above symptoms, especially if the complaints are interfering with your activities. Hashimoto's encephalopathy can improve on its own, but knowledge of the disease type or possible other diseases can help in faster recovery.
Encephalopathy has other causes such as decreased blood flow to the brain, inflammation, infection, trauma, cancer, and so on. In complex cases, there are no specific diagnostic tools that can support the diagnosis of Hashimoto's encephalopathy.
In addition, you can visit a doctor if you need further assistance. You can also visit a doctor for consultation if you experience mood changes, have symptoms of depression, or have thoughts of ending your life.
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- dr Hanifa Rahma
Hashimoto encephalopathy - About the Disease. Genetic and Rare Diseases Information Center. (2022). Retrieved 2 October 2022, from https://rarediseases.info.nih.gov/diseases/8570/hashimoto-encephalopathy.
Hashimoto's disease - Symptoms and causes. Mayo Clinic. (2022). Retrieved 2 October 2022, from https://www.mayoclinic.org/diseases-conditions/hashimotos-disease/symptoms-causes/syc-20351855.
Kanakamedala, A., & Othman, B. (2022). Hashimoto Encephalopathy - EyeWiki. Eyewiki.aao.org. Retrieved 2 October 2022, from https://eyewiki.aao.org/Hashimoto_Encephalopathy.
Shomon, M., & Waldman, L. (2021). How Hashimoto's Encephalopathy Is Diagnosed. Verywell Health. Retrieved 2 October 2022, from https://www.verywellhealth.com/hashimotos-encephalopathy-overview-3231700.