Hashimoto's encephalopathy is an autoimmune neuroendocrine
disorder caused when the thyroid antibodies seen in Hashimoto's
thyroiditis affect brain tissue.
Hashimoto's encephalopathy (HE), which has recently been designated steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is an autoimmune disorder that can cause memory impairment, cognitive changes, dementia and associated neurological symptoms. HE can occur in patients with hypothyroidism related to Hashimoto's thyroiditis and in patients with normal thyroid function. In some cases, particularly when HE occurs in association with non-thyroid related disorders such as Sjogren's syndrome or systemic lupus erythematosus (SLE), it's called nonvasculitic autoimmune meningoencephalitis (NAIM).
The first cases of Hashimoto's encephalopathy were reported in 1966 when the existence of thyroid antibodies in the blood of patients with the autoimmune hypothyroid disorder Hashimoto's thyroiditis was first demonstrated. SREAT is caused by the same thyroid antibodies that cause Hashimoto's thyroiditis although in SREAT, rather than destroying thyroid tissue, these antibodies attack and destroy brain cells known as neurons. Most experts believe that SREAT is under-diagnosed and that many patients thought to have Alzheimer's disease actually have SREAT, which is a treatable disorder. Hashimoto's encephalopathy has been reported worldwide, and cases have been documented in patients ranging from 12-82 years with women more likely to be affected than men.
Encephalopathy is a general term referring to an inflammatory brain disease that alters the brain's structure or function. Encephalopathy is suspected in patients showing signs of an altered mental state. Common symptoms of encephalopathy include stroke-like symptoms of memory loss, difficulty concentrating, hallucinations, irritability, restlessness, amnesia, diminished cognitive ability, myoclonus (involuntary muscle twitching), tremors, nystagmus (rapid, involuntary eye movement), muscle weakness, dementia, seizures, convulsions, difficulty swallowing, impaired speech, confusion, disorientation, psychosis, headache, right-sided hemiparesis or partial paralysis, and fine motor problems, including incoordination of arms, hands and fingers.
HE is diagnosed in patients with high titers of thyroid peroxidase (TPO) antibodies who show signs of cognitive impairment responsive to corticosteroids. Lymphocytic vasculitis of the veins and venules of the brain stem in HE supports the notion that HE may be an autoimmune vascular disorder. Vasculitis as a contributing factor to HE is also supported by the presence of anti-alpha-enolase antibodies in HE. These antibodies are also seen in other conditions of vasculitis including systemic lupus erythematosus (SLE) and ANCA-associated vasculitis. Other diagnostic changes seen in HE include an elevated cerebrospinal fluid protein, EEG abnormalities (diffusely slowed), and perfusion deficits in the presence of normal structural neuroimaging. Although hypothyroidism may be present, most patients have normal levels of circulating thyroid hormone, suggesting that inflammation rather than myxedema is responsible for the cognitive defects. CT scans may be normal or show cerebral swelling (patchy edema) secondary to diffuse white-matter edema appearing as diminished attenuation with increased signal intensity on T2-weighted matter MRI images. The variation in imaging test results may represent different stages or subtypes of HE.
HE may, like multiple sclerosis, also cause a relapsing form of encephalopathy with imaging test results varying depending if the disease is an active or relapsing mode. Relapsing white-matter edema is the usual presentation. Most patients have elevated titers of TPO antibodies and some patients have thyroglobulin antibodies. However, because 20 percent of the older population, especially women, may have these antibodies, antibody test results must be interpreted with caution. Patients with HE are also reported to occasionally have antinuclear antibodies (ANA) and anti-parietal cell antibodies. Fine needle aspiration (FNA) studies in patients with HE show lymphocytic thyroiditis.
Patients with SREAT show a good response to corticosteroids such as prednisone and related immunosuppressants because of the ability of these medications to reduce thyroid antibody production and reduce inflammation. Researchers in India report a case of SREAT that did not respond to corticosteroids but showed a very favorable response to plasma exchange, a technique used to remove circulating antibodies,
Resource: Pablo Castillo, Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis, Archives of Neurology, February 2006.
The copyright of the article Hashimoto's encephalopathy in Thyroid Disorders is owned by Elaine Moore. Permission to republish Hashimoto's encephalopathy in print or online must be granted by the author in writing.
I am now in the process of a study on a number of severe migraine patients
who have thyroid antibody disease. I believe this may be more common by far
than the traditionally described Hashimoto's encephalopathy.
Northwest Headache Clinic Kirkland Washington.
Jun 5, 2008 11:20 AM
Guest :
My sister (28 yrs old)was diagnosed with HE in December after experiencing
a seizure in October and another one in December, each putting her into a
coma. She has since responded well to prednisone, and is now in the
process of tapering off of it. She takes 40 mgs every other day. Does the
process of tapering off of prednisone cause fatigue? She seems very tired,
and I am just hoping this is not a relapse of the HE. She also had a total
thyroidectomy four months ago. Your comments are appreciated.
Jun 5, 2008 3:18 PM
Elaine Moore :
Tapering off prednisone could cause fatigue. However, after thyroidectomy,
thyroid hormone levels can fluctuate. Her dose of replacement hormone may
need adjusting. She might want to mention the fatigue to her doctor and see
if she can have thyroid function tests done, including tests for FT4, FT3,
and TSH.
Aug 19, 2008 10:56 PM
Guest :
Many Thanks to the neurologist in Washington investigating the relationship
between severe migraine and thyroid antibodies. I presented with an HE that
consisted primarily of severe, almost daily migraines. I also had profound
fatigue and more subtle focal deficits and ataxia that came and went, but
still not the stereotypical HE pattern if there is such a thing (each case
is different)- it took me 8 years to get diagnosed. I expect that many
patients will get diagnosed faster through the Kirkland doctor's efforts.
We now treat my migraines not just with verapamil and a triptan, but with
low dose steroids during the attacks. For the other guest who wrote in
about her sister: in addition to the prednisone taper causing fatigue, I
have read that steroids block the production and secretion of TSH and
perhaps even block the conversion of inactive thyroid hormone (T4) to
active hormone (T3). To what extent this can contribute to the fatigue of
the taper itself, I'm not sure. In theory, this could make one a little bit
hypothyroid, although your sister probably gets all of her hormone now
through medication (since her gland was removed) and so isn't affected by
TSH levels but may be affected by the latter mechanism. My TSH dropped in
half (to below normal) while I was on my first big pulse and taper of
prednisone. I did not feel better until a few days after the steroids were
stopped, but then I felt better than I had in years. I hope your
sister is doing well now.
Aug 30, 2008 10:37 AM
Guest :
I've been diagnosed with Hashimoto's and have recovered much of my
attention issues but I am still struggling with memory/word finding. I live
in the Bellevue, WA area. Could you tell me the name of the neurologist who
is familiar with Hashimoto's and helped you?
Aug 30, 2008 11:19 AM
Elaine Moore :
Hello Guest from Bellevue, WA
You might want to check out this
clinic because they're running a study you might be able to participate in.
Northwest Headache Clinic 13107 121st Way NE Kirkland, WA 98034 (425) 899-6200
Sep 28, 2008 1:59 AM
Guest :
My daughter was diagnosed in august 08 .She is 11.We are having a very hard
time finding a Dr who has treated children. If anyone out there knows of
someone even a chance Please let me know.Alex has had very bad headaches
since ahe was 4 years old.
Oct 28, 2008 2:32 PM
Guest :
I have HE and I have been struggling with it for about a year. I was
diagnosed first in feb. of 2008 but this diagnosis was only recently
confirmed. I am 17 years old now. I see a doctor named Dr. William
Gallentine at Duke Hospital in Durham NC. I beleive this might help the
guest with the 11 year old daughter. There are also good doctors at UNC,
like Dr. Greenwood (I think that's his name, I can't remember) and Dr.
Tracy Schaefer is the woman who diagnosed me, although I would NOT
recommend her as a Dr. In other news, I also have problems
with finding words!!! I thought it was just me. Thank you Guest from
Bellvue WA! I feel much better now! I really hope that your daughter
(guest with 11 yr. old daughter) finds help. HE is not easy to deal
with. I strongly recommend Plasmapheresis (also called plasma
exchange) and IVIG treatment. these treatments are wonderful because they
provide the results of Prednisone with out the horrible effects. really,
if you have HE please insist that your doctor at least try these
treatments. they are worth it for too many reasons to count. Durham,
NC
Nov 23, 2008 5:06 PM
Guest :
Thank you for your comments on H.E. My daughter who is 14 years old has
recently been diagnosed. She did respond well to prednisone buther doctors
are now going to try Plasma.Since once they tapered her off she relapsed.
What worked best for you and what were your symptoms. Thank You,
Dec 21, 2008 3:15 PM
Guest :
I have been diagnosed with H.E. I had 5 IVIG treatments in Aug. I had
previously had steroid treatments, but the side-effects were causing my
body havoc. I was much better for two weeks. Because of the relapse we went
to once a month. Because I relapsed quickly we went to twice a month. I
have not responded very well to my last two treatments. I have not noticed
any difference. I am beginning to loose hope that the IVIG treatements are
going to work. Any similar experiences.
Jan 29, 2009 5:11 AM
Guest :
I was diagnosed with Hashimoto's Thyroiditis years ago. My levels are
extremely elevated. I have been having seizures for over 6 months now and
they can't figure out why. When I suggested it might be HE, the doctors
just told me that it is too rare and refuse to try using corticosteroids on
me. The seizure medicine isn't helping any and I exhibit quite a few of
the symptoms mentioned in this article. I am at my wits end in trying to
get help.
Jan 29, 2009 10:08 AM
Elaine Moore :
Hi, Can you contact the clinic in WA that's listed in the first
comment here? They at least seem to know the problem exists. I've been
hearing from other people who are having a hard time getting a diagnosis
and proper treatment. I'm sorry you're having a hard time getting the
help you need and hope you can find a contact through these comments that
can help you. Best, Elaine
Jan 31, 2009 4:42 PM
Guest :
Hello, I live in Boise, ID. I was diagnosed with regular Hashimoto's
disease in the last year. I was referred to an immunologist/reumatologist
when it wasn't getting better, but worse. He ruled out Lupus (was having
high ana's up and down) and told me it looked like Hashimoto's
encepholopathy after reviewing my symptoms and mri. He said he did follow
up on a woman in Boise who could not get diagnosed here and had to go to
they Mayo Clinic to get diagnosed, and that I presented like she did. Does
anyone know where I can go that is near Idaho? I went to an neurologist
here, and he didn't know anything about Hashimoto's or Hashimoto's
encepholopathy and suggested maybe I just had sleep apnea or that I should
see a shrink. It was distressing and I don't know where to turn.
Feb 6, 2009 7:07 PM
Guest :
My wife was diagnosed wiyh HE in nov. 2008. she has had two series of
plasmapherises(12 sessions) and is now taking 60mg of prednisone daily. She
responds well to the pp treatments, but each time she relapses after about
10 days. We would love to hear from anyone with similar circumstances or
any additional help.
Feb 7, 2009 11:09 AM
Elaine Moore :
Hi, I've been reading lately of neurological symptoms in gluten
sensitivity aggravating Hashimoto's thyroiditis and encephalopathy. Studies
have also shown that in people with both gluten sensitivity and
Hashimoto's, a gluten free diet reduces thyroid antibodies as well as the
antibodies seen in gluten sensitivity. A gluten free diet involves avoiding
wheat, rye and barley. It sounds like your wife is getting adequate
treatment, but a trial of avoiding gluten might yield a solution to
avoiding relapses. Best, Elaine
Mar 4, 2009 8:15 PM
Guest :
I have HE/SREAT and was wondering if anyone else out there also has been
experiencing either shortness of breath and/or urinary urgency and
frequency? I started having these symptoms when I got sick with the
HE/SREAT, but have never heard of them being associated with the disease.
So far, no other causes of my symptoms have been found, so I'm wondering if
other HE/SREAT patients might also have them.
Mar 9, 2009 1:17 PM
Guest :
my 19 yr old daughter was diagnosed with HE today after 2 seizures one b4
xmas 2009 and anther oe mnth later both puttin her in a coma and bein
ventelate, we have spent all day lookin on the internet about HE she has
recovered very well mentally but mobility and tremours are a big problem,
she is on anti seizura meds only, durin the addmittions into ITU she has
suffered from alapesia, we are hopin that she returns back to universitiy
in sept but are worried incase she relaps, any information is greatly
recieved. thanks claire
Mar 9, 2009 2:36 PM
Elaine Moore :
Hi, I'm sorry to hear of your daughter's condition and glad that she
received a proper diagnosis. Did your daughter have a high titer of thyroid
antibodies? In the United States your daughter can go to the hospital's
medical records department and pick up copies of all her test results.
Typically, thyroid antibody levels are very high in HE and drugs to
slow down the immune system, such as corticosteroids, are used. It would be
good to see if her thyroid antibody levels declined during her
hospitalization.
It seems her doctors would have plans to
routinely test her thyroid hormone levels to make sure that they're not
rising. Some anticonvulsants also affect thyroid hormone levels and it's a
good idea to run thyroid hormone levels as well within a few months of
starting meds. Best to you and your family, Elaine
Mar 13, 2009 6:13 AM
Guest :
I was diagnosed with HE or Sreat a few days ago. My tyroid peroxidase level
was 1050 which I gather was a very high number. About 12 years ago I had
severe migraines & then over the years I have addeds symtoms such as
extreme fatigue, loss of cognative abilities, muscle twitching, vertigo,
pain in muscles and joints, foggy headedness, ect, ect. I have been unable
to work for for a little over 4 months and as a result I have lost my job.
I was just wondering if most patients that have treatment are able to
return to work or is this more of a permanent disability situation. Am I
likely to gain back some of the energy, cognative abilities ect? Are
relapses likely to occur with a frequency that would make it hard to retain
a job?
Mar 13, 2009 8:27 AM
Elaine Moore :
Hi, If you're in the U.S. you might want to check into the American
with disabilities act....the law changed in 2009 and people like you who
should get disability are more likely to get it.
Depending on
the severity of your condition, you should see improvement but it's hard to
say how much improvement you'll see. Best to you, Elaine
Mar 29, 2009 12:09 PM
Guest :
Doctors found my Hashimoto's about 5 year ago. The past two years I have
been suffering what they call Tension headaches.. chronic they are! I often
find myself losing short term memory as I feel like I have so much going on
in the mind that I can't concentrate. I keep wondering if I have a start of
HE? Anyone experience similar symtpoms? I am 29f. Pain is in back of
head..a burning pain. MRI has ruled out all major issues.
Apr 24, 2009 5:46 PM
Guest :
In response to the 3/13/09 and 3/29/09 posts, here are some of the things i
experienced with my HE. I went undiagnosed for about 7 years and was only
diagnosed 2 years ago. My illness seemed to start with a lot of headaches.
Some were tension-type headaches (or stiff neck) which prompted me to go to
physical therapy for neck exercises and massage. Others were more severe
migraine-like headaches behind my left eye- it would feel like someone
rammed a baseball bat into my eye socket. At times, these were almost
daily. Now they are about 12 episodes per month. I also experienced A
LOT of fatigue, which many days left me bed-ridden. I was in professional
school at the time, and I had to take a lot of time off for this, which was
frustrating because I used to be an avid hiker and quite a go-getter. I
also had cognitive slowing, problems with language (word-finding and
grammar), memory issues, personality changes (I became easily irritated
which is not how I used to be). I also experienced problems with balance
and coordination (also known as ataxia- the symptom which prompted referral
to a movement disorder neurologist who ultimately diagnosed the HE and
thyroiditis). I dropped and broke a lot of dishes and had trouble with fine
motor movements like painting and doing buttons or shoelaces. I also had a
lot of muscle twitches all over at random intervals and muscle groups, and
some myoclonic whole body jerks at night. I also had a lot of the symptoms
of hypothyroidism, but because my TSH was always normal, was reassured
repeatedly that my thyroid was okay. I thought I was going crazy before I
got diagnosed. Both my primary neurologist as well as the movement
disorder specialist have declared me disabled, even after several pulse and
taper treatments of steroids and being on thyroid replacement.
Unfortunately, i have spent so much time in school that I do not have the
social security credits to go on disability and am looking into home
employment, which will still be quite exhausting. I would urge the person
who has been unable to work to discuss her level of functioning with her
neurologist and get the ball rolling on disability status if she has enough
social security credits. If she does not have enough credits, perhaps her
state has a department of rehabilitation, which can offer guidance on
working with a disability. In my opinion, HE is a tough disease to function
with because it affects your whole brain and can really slow you down
mentally and physically.
May 23, 2009 9:09 PM
Elaine Moore :
Hello,
Corticosteroids in high doses have potentially serious
side effects, but they also offer many benefits in conditions such as
HE. See www.suite101.com/blog/daisyelaine/corticosteroid_therapy
You would need to ask your doctor if your symptoms suggest a
relapse and if a longer course of therapy is needed. Best, Elaine
Jun 20, 2009 8:24 AM
Elaine Moore :
Hi, You could post your email address in a message hear and ask others
to contact you. Or you could call the number for the place in Washington
and see if they know of any online support group. I wish you well, Elaine
Jul 15, 2009 3:03 PM
Elaine Moore :
Hi,
It's fortunate that the Ochsner Clinic diagnosed you so
quickly. HE responds well to steroids, and getting a proper diagnosis is
half of the battle. Keep Well, Elaine
Jul 15, 2009 4:10 PM
Elaine Moore :
Hi, It's unfortunate that your doctor dismissed your symptoms and
positive TPO antibody levels so easily. You might check with the Northwest
Headache Clinic in Washington to see if they have any referrals for your
area. You might also go to PubMed and look for journal articles on the
topic. The authors typically provide email addresses and may be able to
steer you to someone in your area. An endocrinologist associated with a
university may be a good choice. Best, Elaine
Jul 22, 2009 8:16 PM
Elaine Moore :
Hi, I'm glad to hear that you finally received a correct diagnosis and
are showing such a good response to steroids. Best to you and thanks for
sharing your story, Elaine
Jul 27, 2009 11:25 AM
Elaine Moore :
Hi, Corticosteroids are the main line treatment for HE. Your doctor
probably cut your dose to see if you could get by on a lower dose. Since
your symptoms returned, you likely need a higher dose to control your
symptoms. Best, Elaine
Jul 31, 2009 3:24 PM
Elaine Moore :
Hi, I'm sorry to hear your father was diagnosed with HE. However,
getting a proper diagnosis is often the hardest part of this disease. Most
patients respond well to corticosteroids. You might want to call the
neurology department at the University of Chicago and see if someone there
can help you. Best, Elaine
Aug 24, 2009 5:38 PM
Elaine Moore :
Hi, Does your daughter have the high thyroid antibodies seen in
Hashimoto's encephalopathy? She may have HE along with another condition
causing a seizure disorder. An MRI and CAT Scan would be helpful. Best,
Elaine
Aug 24, 2009 5:41 PM
Elaine Moore :
Hi, I forgot to mention that seizures are also associated with the use
of aspartame in diet sodas, sugarless chewing gums, etc. Best, Elaine
Aug 24, 2009 5:48 PM
Elaine Moore :
Hi, Spinal fluid protein is elevated and high levels of TPO antibodies
are seen in HE. These antibodies persist for several months after they're
produced so even with corticosteroids reducing production of new
antibodies, symptoms don't generally resolve at once. Best, Elaine
Aug 27, 2009 11:01 AM
Elaine Moore :
Hi, Lewy Body dementia causes some changes that are seen on brain
scans and other imaging tests. Patients with HE have high levels of thyroid
antibodies. I don't believe corticosteroids cause improvement in LBD but
they help in HE. Best, Elaine
Aug 31, 2009 9:58 AM
Elaine Moore :
Hi, I don't know of any doctors in Missouri offhand, but you might
want to try this link: www.drscore.com/physicianlist.cfm?w=MO Also, see if you can get your primary care physician to order tests for
thyroglobulin, TPO and TSH receptor antibodies so you'll have this
information for your appointment. You may want to look for a neurologist.
Best, Elaine
Sep 15, 2009 3:14 PM
Guest :
I was diagnosed with Hashimotos three years ago. I have been on Synthroid,
Levoxyl and now Armour Thyroid. My memory is non-existant, I have no
emotion and I feel like I am walking in a fog. I do not have a life and I
want mine back. I have tremors in on my left side and have lost my
confidence to do anything. I am afraid to drive. I live in Austin, TX.
Does anyone know who can help.
Oct 5, 2009 3:55 PM
Guest :
I have been worked up for a dx of HE since mid June and followed up today
with a dx of HE. Although right now I remain symptommatic, I am debating
FMLA vs disabillity because some days it is impossible to function. Any
words of advice?
Oct 5, 2009 4:32 PM
Elaine Moore :
Hi, Since you were just diagnosed I'd put in for FMLA and ask your
doctor how much time off he thinks you need to see if meds help. I'm
assuming he prescribed prednisone and may have to taper the dose and you
may have side effects to deal with. Ultimately, your doctor will have to ok
your request for sick time off or disability. I've heard from two
people recently whose HE improved using low dose naltrexone. You might want
to read my articles on LDN and ask your doctor about this possibility.
While there aren't any published studies on its use in HE, it offers
benefits in autoimmune disorders and for reducing inflammation. Because
it's inexpensive and side effects are minimal he or she might consider it.
Best, Elaine
