Steroid-responsive encephalopathy associated with autoimmune thyroiditis
=Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis =
Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT), also known as Hashimoto's Encephalopathy, is a rare neurological disorder characterized by an association with autoimmune thyroid disease. This condition is notable for its responsiveness to corticosteroid treatment.
Clinical Presentation[edit | edit source]
Patients with SREAT may present with a variety of neurological symptoms, which can include:
- Cognitive impairment
- Seizures
- Myoclonus
- Ataxia
- Psychiatric symptoms such as depression or psychosis
The clinical presentation can vary significantly between individuals, making diagnosis challenging.
Pathophysiology[edit | edit source]
The exact pathophysiology of SREAT is not fully understood. It is believed to be an autoimmune process, where the body's immune system mistakenly attacks the central nervous system. This is often associated with the presence of anti-thyroid antibodies, such as anti-thyroperoxidase (anti-TPO) and anti-thyroglobulin antibodies.
Diagnosis[edit | edit source]
Diagnosis of SREAT is primarily clinical, supported by laboratory and imaging findings. Key diagnostic criteria include:
- Presence of anti-thyroid antibodies
- Exclusion of other causes of encephalopathy
- Positive response to corticosteroid therapy
Additional diagnostic tools may include:
- Electroencephalogram (EEG) showing non-specific abnormalities
- Magnetic Resonance Imaging (MRI) of the brain, which may be normal or show non-specific changes
- Cerebrospinal fluid (CSF) analysis, which may show elevated protein levels
Treatment[edit | edit source]
The mainstay of treatment for SREAT is corticosteroids, such as prednisone. Patients typically show a rapid improvement in symptoms following the initiation of steroid therapy. In cases where steroids are not effective or cannot be used, other immunosuppressive therapies may be considered.
Prognosis[edit | edit source]
The prognosis for patients with SREAT is generally favorable, especially with early diagnosis and treatment. Most patients respond well to corticosteroids, although some may experience relapses or require long-term immunosuppressive therapy.
Epidemiology[edit | edit source]
SREAT is a rare condition, with a higher prevalence in females and typically presenting in middle age. The exact incidence is unknown due to its rarity and the potential for underdiagnosis.
See Also[edit | edit source]
External Links[edit | edit source]
NIH genetic and rare disease info[edit source]
Steroid-responsive encephalopathy associated with autoimmune thyroiditis is a rare disease.
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Contributors: Prab R. Tumpati, MD
