- Credits - Section Writer: Dr. Om J Lakhani - Section Editor: Dr. Om J Lakhani Support us: 1. Support us by purchasing our book - Click here for more details: [[Volume 1- THE BEST OF NOTES IN ENDOCRINOLOGY BOOK SERIES]] 2. [Support you by Becoming a YouTube member (Click here)](https://www.youtube.com/channel/UC6zQSf7dLDqfQOeM4mNUBTQ/join) - Video lecture - <iframe width="560" height="315" src="https://www.youtube.com/embed/Y5K9ICXJIh4" title="YouTube video player" frameborder="0" allow="accelerometer; autoplay; clipboard-write; encrypted-media; gyroscope; picture-in-picture" allowfullscreen></iframe> <br> - Q. What is [[Hashimoto's encephalopathy]]? <br> - It is a form of Non-infective encephalopathy associated with Autoimmune thyroid disorder with positive [[Anti TPO antibody]] - It has excellent response to glucocorticoids - It is a diagnosis of exclusion <br> - Q. Is the term "encephalopathy" same as "encephalitis"? <br> - "Encephalopathy" means "brain disorder" and it is a broad term - "Encephalitis" means "brain inflammation" and it deals with inflammatory disorder of the brain and it is a more specific term <br> - Q. Who was the first to describe [[Hashimoto's encephalopathy]]? <br> - Dr Russell Brain <br> - Q. What is the timing in terms of presentation? <br> - It is subacute in terms of onset <br> - Q. What is the most typical clinical presentation of HE? <br> - Subacute confusion with - Seizures - Myoclonus - Pyschosis - With altered consciousness <br> - Q. What is the epidemiology of this condition? <br> - Age of onset is on an average 51 years - However, it has been reported via a wide age variation - It is more common in females <br> - Q. What are the possible etiologies of Hashimoto’s encephalopathy? <br> - Autoimmune encephalomyelitis - Some people think [[Hashimoto's encephalopathy]] is a subset or type of ADEM- Acute disseminated encephalomyelities - Autoimmune cerebral vasculitis - Some people think [[Hashimoto's encephalopathy]] is a subform of NAIM- which is [[Non-vasculitic inflammatory meningoencephalitis]] <br> - Q. Is it because of the thyroid dysfunction? <br> - The neurological features of this condition are INDEPENDENT of the thyroid disorder - The symptoms are NOT due to the thyroid hormone itself and are a result of an associated autoimmune phenomenon <br> - Q. What is the typical clinical presentation of this syndrome? <br> - It presents as acute or subacute confusion with altered levels of consciousness <br> - Q. How does it progress? <br> - It progresses in two ways - a) It may progress as stroke-like multiple episodes of recurrent focal neurological deficits with variable degree of cognitive dysfunction - b) It may have a diffuse slow progression with deteriorating cognitive function characterized by dementia, hallucination, somnolence etc <br> - Q. Can seizures be seen in this condition? <br> - Yes - 2/3rd of patients have seizures are the time of initial presentation - it can be both focal as well as generalized seizures <br> - Q. What are other neurological features? <br> - 1. Psychosis and hallucinations - 2. Hyperreflexia and pyramidal tract signs - 3. Myoclonus, tremors etc <br> - Q. Can pyschiatric symptoms be seen in this condition? <br> - Yes - In fact, 30% of patients have some form of psychosis or psychiatric symptoms <br> - Q. Which antibody is specific against leading to [[Hashimoto's encephalopathy]] (HE)? <br> - Anti NAE antibody (N-amino terminal of alpha enolase) <br> - Q. Can it be associated with hypothyrodism? <br> - Yes <br> - Q. Is hypothyroidism always present with [[Hashimoto's encephalopathy]]? <br> - No - Thyroid dysfunction runs an independent course - [[Hashimoto's encephalopathy]] may be associated with - Hypothyroidism - Euthyroidism - Hyperthyroidism - Subclinical thyroid disorders <br> - Q. Can [[Hashimoto's encephalopathy]] be associates with [[Graves' disease]]? <br> - Yes - But it is very rare - <20 cases have been reported in literature <br> - Q. Which is key differentiating feature of HE vs neuropsychiatric manifestation due to hypothyroidism? <br> - Symptoms of hypothyroidism improve with treatment with LT4 whereas HE they do not improve with LT4 treatment <br> - Q. Is Anti TPO positive in HE? <br> - It is found in 100% of cases - Anti TPO antibody is also found in CSF and CSF anti TPO antibody may be more sensitive and specific for [[Hashimoto's encephalopathy]] <br> - Q. What level of Anti-TPO is associated with [[Hashimoto's encephalopathy]]? <br> - As such there is no correlation between Anti TPO antibody levels and [[Hashimoto's encephalopathy]] - However median values reported in literature are 900 IU/ml <br> - Q. Which test is useful for follow-up of therapy? <br> - IgG4 level in CSF is useful for follow up of patients with HE <br> - Q. What are other common CSF findings? <br> - Elevated protein concentration has been reported in the CSF <br> - Q. What are neuroimaging changes seen with [[Hashimoto's encephalopathy]]? <br> - In many cases MRI may be normal - However it may be associated with nonspecific focal or diffuse hyperintensities of the subcortical white matter or ischemic lesion - These often improve with glucocorticoid therapy <br> - Q. How common are EEG changes seen in such patients? <br> - EEG changes are seen in 90% of the patients but they are generally non-specific - Mainly persistent or episodic slow wave changes are seen <br> - Q. What is the anti-NMDAR encephalitis and why is it an important differential diagnosis of this case? <br> - Anti-NMDAR encephalitis is a form of autoimmune encephalitis which it s a paraneoplastic syndrome and it is similar in clinical presentation of this condition - It must be ruled out in such patients <br> - Q. What is the drug of choice for HE ? <br> - Corticosteroids <br> - Q. Do the level of the [[Anti TPO antibody]] change with treatment? <br> - No <br> - Q. What is the new name for HE? <br> - SEAT - Steroid responsive encephalopathy with autoimmune thyroiditis <br> - Q. Does giving Anti-psychotic medications to such patients presenting with psychosis help? <br> - No - Antipsychotic medications may paradoxically worsen psychosis/psychiatricsymptoms in some cases (Tseng, 2011). - Hence to be used cautiously.