- Last edited on July 18, 2023
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cl:0-autoimmune-encephalitis:hashimotos [on August 26, 2019] |
cl:0-autoimmune-encephalitis:hashimotos [on July 18, 2023] psychdb [Investigations] |
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- | ====== Hashimoto's Encephalitis ====== | + | ====== Hashimoto's Encephalitis (Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis [SREAT]) ====== |
{{INLINETOC}} | {{INLINETOC}} | ||
===== Primer ===== | ===== Primer ===== | ||
- | **Hashimoto's Encephalopathy (HE)** (also known as Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis [SREAT]), is a rare, clinically heterogeneous neurological disorder associated with [[cl:thyroid-disorders:hashimotos|Hashimoto's disease]] and positive thyroid autoantibodies. It is increasingly recognized as an important and treatable cause of autoimmune encephalitis, though remains somewhat controversial as a diagnosis. | + | **Hashimoto's Encephalopathy (HE)** (also known as Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis [SREAT]), is a rare, clinically heterogeneous neurological disorder associated with [[cl:thyroid-disorders:hashimotos|Hashimoto's disease]] and positive thyroid autoantibodies. It is is a form of [[cl:0-autoimmune-encephalitis:home|autoimmune encephalitis]]. It is increasingly recognized as an important and treatable cause of autoimmune encephalitis, though remains somewhat controversial as a diagnosis. |
== Epidemiology == | == Epidemiology == | ||
- | HE predominantly affects women in a wide age range, from 10 to 80 years of age. | + | * HE predominantly affects women in a wide age range, from 10 to 80 years of age. |
== Comorbidity == | == Comorbidity == | ||
- | Clinical or subclinical thyroid disease, usually hypothyroidism, occurs in over half of the cases. | + | * Clinical or subclinical thyroid disease, usually [[cl:thyroid-disorders:hypothyroidism|hypothyroidism]], occurs in over half of the cases. |
===== Diagnostic Criteria ===== | ===== Diagnostic Criteria ===== | ||
+ | <alert icon="fa fa-arrow-circle-right fa-lg fa-fw" type="success">See also main article: **[[cl:0-autoimmune-encephalitis:home]]**</alert> | ||
<WRAP group> | <WRAP group> | ||
<WRAP half column> | <WRAP half column> | ||
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- **Presence of serum thyroid antibodies** (thyroid peroxidase, thyroglobulin) | - **Presence of serum thyroid antibodies** (thyroid peroxidase, thyroglobulin) | ||
- **Absence of well characterized neuronal antibodies in serum and CSF** | - **Absence of well characterized neuronal antibodies in serum and CSF** | ||
- | - **Reasonable exclusion of alternative causes** ([[cl:0-autoimmune-encephalitis#differential-diagnosis|see Differential Diagnosis]]) | + | - **Reasonable exclusion of alternative causes** ([[cl:0-autoimmune-encephalitis:home#differential-diagnosis|see Differential Diagnosis]]) |
</WRAP> | </WRAP> | ||
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The clinical presentation of HE can include seizures, myoclonus, hallucinations, and stroke-like episodes with normal or non-specific CSF and brain MRI abnormalities. | The clinical presentation of HE can include seizures, myoclonus, hallucinations, and stroke-like episodes with normal or non-specific CSF and brain MRI abnormalities. | ||
</callout> | </callout> | ||
+ | <callout type="question" title="Controversial Diagnosis" icon="true"> | ||
+ | Hashimoto’s encephalopathy should be diagnosed only when a thorough clinical assessment has been performed, including comprehensive testing for well characterized neuronal antibodies, and ruling out other potential causes of encephalopathy or encephalitis. In essence, it should be a diagnosis of exclusion.</callout> | ||
</WRAP> | </WRAP> | ||
</WRAP> | </WRAP> | ||
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<WRAP group> | <WRAP group> | ||
<WRAP half column> | <WRAP half column> | ||
- | anti-TPO, anti-thyroglobulin, and anti-dsDNA can be elevated in HE. | + | * anti-TPO (thyroid peroxidase antibody), anti-thyroglobulin, and anti-dsDNA can be elevated in HE |
+ | * Thyroid peroxidase antibody is elevated in over 90% of cases of Hashimoto’s thyroiditis. | ||
+ | * Although the test has a false positive rate of up to 20%, a positive result may indicate SREAT, and should prompt a thorough search for neurological symptoms that might support a diagnosis of encephalopathy. | ||
</WRAP> | </WRAP> | ||
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</WRAP> | </WRAP> | ||
</WRAP> | </WRAP> | ||
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===== Treatment ===== | ===== Treatment ===== | ||
Patients with a non-specific encephalopathy with subclinical or overt thyroid disease, anti-thyroid antibodies, and no other explanation for the symptoms should be considered for a trial of steroids. Most patients treated with corticosteroids with or without levothyroxine will have clinical improvement.[([[https://www.ncbi.nlm.nih.gov/pubmed/12580699/|Chong, J. Y., Rowland, L. P., & Utiger, R. D. (2003). Hashimoto encephalopathy: syndrome or myth? Archives of neurology, 2, 164–171.]])] | Patients with a non-specific encephalopathy with subclinical or overt thyroid disease, anti-thyroid antibodies, and no other explanation for the symptoms should be considered for a trial of steroids. Most patients treated with corticosteroids with or without levothyroxine will have clinical improvement.[([[https://www.ncbi.nlm.nih.gov/pubmed/12580699/|Chong, J. Y., Rowland, L. P., & Utiger, R. D. (2003). Hashimoto encephalopathy: syndrome or myth? Archives of neurology, 2, 164–171.]])] | ||
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* [[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2018852/|Gómez-Bernal, G. J., Reboreda, A., Romero, F., Bernal, M. M., & Gómez, F. (2007). A case of Hashimoto's encephalopathy manifesting as psychosis. Primary care companion to the Journal of clinical psychiatry, 9(4), 318.]] | * [[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2018852/|Gómez-Bernal, G. J., Reboreda, A., Romero, F., Bernal, M. M., & Gómez, F. (2007). A case of Hashimoto's encephalopathy manifesting as psychosis. Primary care companion to the Journal of clinical psychiatry, 9(4), 318.]] | ||
* [[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3693614/|Payer, J., Petrovic, T., Lisy, L., & Langer, P. (2012). Hashimoto encephalopathy: a rare intricate syndrome. International Journal of Endocrinology and Metabolism, 10(2), 506.]] | * [[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3693614/|Payer, J., Petrovic, T., Lisy, L., & Langer, P. (2012). Hashimoto encephalopathy: a rare intricate syndrome. International Journal of Endocrinology and Metabolism, 10(2), 506.]] | ||
+ | * [[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3714092/|Maroz, N., Bernhardt, N., & Chow, R. D. (2012). Autoimmune encephalopathy associated with thyroid autoantibodies as the cause of reversible cognitive impairment. Journal of community hospital internal medicine perspectives, 2(1), 11453.]] | ||
+ | * [[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4209691/|Anand, K. S., Garg, J., Verma, R., & Chakraborty, A. (2014). Hashimoto's encephalitis: Unusual cause of reversible dementia. Journal of family medicine and primary care, 3(3), 284.]] | ||
<WRAP group> | <WRAP group> |