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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]) ​======
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 ===== 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>​
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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]])
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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.
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 +<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>​
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-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 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.
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-<callout type="​warning"​ title="​Antibodies Are Non-Specific!"​ icon="​true">​+<callout type="​warning"​ title="​Thyroid ​Antibodies Are Non-Specific!"​ icon="​true">​
 Thyroid antibodies are not specific for Hashimoto’s encephalopathy. They can be present in up to 13% of healthy individuals,​ and up to 27% in white women older than 60 years. It can also be present in other autoimmune encephalitis disorders. Thyroid antibodies are not specific for Hashimoto’s encephalopathy. They can be present in up to 13% of healthy individuals,​ and up to 27% in white women older than 60 years. It can also be present in other autoimmune encephalitis disorders.
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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.]]
  
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