Learn all about Hashimoto’s disease sometimes also referred to as a neuroendocrine disorder, although the condition’s relationship to the endocrine system is widely disputed. A relapsing encephalopathy occurring in association with Hashimoto’s thyroiditis, with high titers of anti-thyroid antibodies.is called hashimoto’s disease.
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. It is associated with Hashimoto’s thyroiditis. It was first described in 1966
The term Hashimoto’s encephalopathy is used to describe an encephalopathy of presumed autoimmune origin characterized by high titres of antithyroid peroxidase antibodies. 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 presentation may involve a relapsing and remitting course and include seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms and myoclonus. Thyroid function is usually clinically and biochemically normal. It is likely that antithyroid antibodies are not pathogenic, but titres can be a marker of treatment response. Pathological findings can suggest an inflammatory process, but features of a severe vasculitis are often absent.
Psychiatric disturbances with Hashimoto encephalopathy are extremely frequent and include disorganized behavior with poor self-care, psychosis (often with visual hallucinations), changes in mood or personality, and sleep dysfunction .Seizures are often associated with Hashimoto encephalopathy, but unique to this syndrome are fluctuating stroke-like episodes that span multiple different vascular territories.37 Other neurological symptoms such as myoclonus, tremor, ataxia, and headache have been reported in one-third of case
Hashimoto encephalopathy is almost uniformly responsive to a prolonged course of high-dose corticosteroids. On average, treatment continues 4 to 6 weeks before clinical recovery starts and corticosteroid taper is initiated. Multiple studies have described relapse of symptoms with early cessation of therapy, which highlights the need to continue therapy beyond simply the appearance of improvement.
What is Autoimmune Encephalitis?
Encephalitis (en-sef-uh-LIE-tis) is inflammation of the brain. There are several causes, but the most common is viral infection. Autoimmune encephalitis (AE) is a complex disease that often requires collaboration among multiple medical disciplines for effective diagnosis and treatment. Most AE patients can expect to see a team of doctors that may include neurologists, rheumatologists, psychiatrists, immunologists and others.
Anti-NMDA Receptor Encephalitis
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a newly recognised autoimmune condition. Unlike classic paraneoplastic limbic encephalopathies with onconeural antibodies directed to intracellular antigens, anti-NMDAR encephalitis harbors antibodies against neuronal extracellular membrane N-methyl-D-aspartate receptor subunit 1 (NR1) of NMDA receptor, and may not be accompanied with tumors.
It has been demonstrated that anti-NR1 antibodies selectively bind, cross-link, and internalize surface NMDA receptors, and lead to decreased postsynaptic NMDA receptor-mediated currents in a reversible and antibody titer-dependent manner. Although recent studies showed few patients with non-tumor-associated anti-NMDAR encephalitis have evidence of elevated anti-thyroid peroxidase (anti-TPO) antibodies there is lack of anti-NMDAR and anti-TPO antibodies combined follow-up in details in current literatures. The majority of patients with anti-NMDAR encephalitis have a prodromal flu-like illness.
In line with this, numerous pathogens have been implicated and identified on serum studies, including mycoplasma pneumoniae influenza virus A, influenza virus B, Chlamydia pneumoniae, Bordetella pertussis and parapertussis To our knowledge, this represents the first anti-NMDAR encephalitis case associated with serum Epstein-Barr virus viral capsid antigen IgM (EBV-VCA-IgM). Although anti-NMDA receptor encephalitis was initially associated with ovarian teratomas, larger studies have revealed that nearly half of patients do not have an identifiable tumor.
Patients usually fare well with intervention and treatment early in the course of the disease, including tumor resection if applicable; with immunotherapy, 75% of patients have full or substantial recovery. Most patients continue to have psychiatric abnormalities after neurological recovery, including attention deficits and behavioral disinhibition. Return to baseline behavioral status is usually slow, and often requires months for symptom resolution.
Autoimmune Encephalitis Symptoms
Some of the common symptoms of autoimmune encephalitis include
- Loss of balance.
- Numbness or weakness in a part of the body.
- Blurred or slow speech or inability to speak.
- Distorted vision.
- Involuntary movements.
- Memory disturbance.
- Cognitive impairment.
There is decreased level of consciousness where the patient can become unresponsive, catatonic or may even slip into a coma.
- Autoimmune encephalitis patients may experiences symptoms of partial or complete appetite loss for prolonged periods of time.
- Extreme anxiety.
- The drink and food items taste inedible or can trigger nausea.
- There is excessive eating and the patient does not feel sated.
- Loss of inhibition.
- Autoimmune encephalitis symptoms may also include inability to sleep (insomnia).
- Pressured, rapid or involuntary speech.
- Paranoid thoughts.
- Hallucinations (auditory/visual).
Autoimmune Encephalitis Treatment
Autoimmune Encephalitis treatment leads to the best outcomes. A number of options are available to treat AE. These therapies are broken down into what are considered “first line” and “second line” treatment options. One or more “first line” treatments may be prescribed by your physician as soon as a patient is diagnosed with AE. The four most common “first line” treatments include the following:
- Removal of a teratoma (if present) that could be triggering the autoimmune response
- Use of anti-inflammatory drugs (i.e. steroids)
- Use of plasmapheresis to remove harmful antibodies from blood
- Treatment with intravenous immunoglobulin (IVIG) which is believed to occupy the binding sites where harmful antibodies attach to brain cells.