Psychiatric Presentation of Hashimoto's Encephalopathy

Manuel Arrojo, MD, Maria M. Perez-Rodriguez, MD, Márcia Mota, MD, Ricardo Moreira, MD,

From the Departments of Psychiatry ; and the Department of Neuroscience (E.B.-G.), Columbia
University Medical Center, New York, NY. July 2006

Address correspondence and reprint requests to Enrique Baca-Garcia, Department of Neuroscience,

Columbia University Medical Center, 1051 Riverside Drive, Suite 2917/unit 42, New York, NY 10032. E-
mail: ebacgar2@yahoo.es; merperez@yahoo.com

ABSTRACT

Introduction: Hashimoto's encephalopathy is an unusual condition that is associated with

Hashimoto's thyroiditis. Myoclonus, epileptic seizures, dementia, and disturbances of
consciousness are the most common features.

Case report: We present an atypical case of Hashimoto's encephalopathy in a 33-year-old

woman who presented with several brief and acute psychotic episodes. After treatment with
steroids, there was an improvement in the patient's psychiatric symptoms
and electroencephalogram, and antithyroglobulin antibody titers returned to normal levels.

Conclusions: It is our opinion that Hashimoto's encephalopathy should be considered in the

differential diagnosis of atypical psychosis, especially because this is a treatable syndrome. This
is particularly important in patients with a previous history of thyroid disease, despite current
normal thyroid function.

Key Words: Hashimoto's disease • encephalopathy • single-photon emission-computed

tomography • psychiatric symptoms

Abbreviations: CT = computed tomography; EEG = electroencephalogram.

A 33-year-old woman from Venezuela, with previous history of thyroid disease, had developed
three brief psychotic episodes between the ages of 26 and 32 years before being referred to St.
João Hospital in August 2005. Unfortunately, limited information was available on the patient's
previous endocrine history before her first visit to our hospital. It was established that the patient
had been diagnosed with hypothyroidism and had received treatment with thyroid hormone. We
thought this may represent the onset of Hashimoto's thyroiditis and may have been related to
the psychotic episodes. During the last of these episodes, after 3 days of severe sleep
disturbance, she suddenly felt that she was an angel, that her arms were wings, and
that Chavez (the president of Venezuela) was the devil. She was referred to a psychiatric unit.
During the hospital admission, she experienced fluctuations in consciousness, visual ("she saw
blood in the eyes of other patients") and auditory ("voices of people that spoke of Chavez")
hallucinations, psychomotor agitation, and mood swings (from euphoria to depressive mood).
The cerebral computed tomography (CT) scan was normal and the electroencephalogram (EEG)
showed slow rhythm with and waves. She was treated with antipsychotic drugs and
discharged after 1 week, with a diagnosis of brief psychotic disorder.

In August 2005, she suffered a fourth psychotic episode. She began to experience sleep
disturbances with total insomnia in the 2 days before hospital admission. In the Emergency
Department of St. João Hospital, she presented with perplexity, severe anxiety, and aggressive
behavior. During her admission, she suffered from episodes of disorientation, mystic (she
believed she was the Virgin Mary) and paranoid delusions, episodes of mutism, depressive
mood, and impulsive behavior. The EEG showed slow rhythm with and waves without
epileptiform activity and the cerebral CT scan was normal. Complete blood count and
biochemistry analyses were normal and serologies (syphilis, human immunodeficiency virus,
cytomegalovirus, and herpes virus) were negative. The Wechsler Adult Intelligence Scale
demonstrated normal intelligence. The results of the thyroid function tests were in the
normal range with the exception of the free T3, which was slightly below the normal range (T3 =
0.94 ng/ml) (reference range 0.60–1.81 ng/ml); free T3 = 2.2 pg/ml (reference range 2.3–4.2
pg/ml); T4 = 9.3 µg/dl (reference range 4.5–10.9 µg/dl); free T4 = 1.34 ng/dl (reference range
0.89–1.80 ng/dl); and TSH = 5.25 µIU/ml (reference range 0.35–5.50 µIU/ml). She was treated
with sodium divalproate, risperidone, and diazepam, and she was discharged after 2 weeks.

During ambulatory follow-up, a new EEG showed no improvement. The patient underwent a
complete thyroid immunologic study: antinuclear antibodies, antineutrophil cytoplasmic
antibodies, thyroid hormones, and antiperoxidase antibodies—all results were normal;
antithyroglobulin antibody titers were increased (72 UI/ml).

A hypoechoic image was found in the thyroid ultrasonography and cerebral single-photon
emission-computed tomography showed hypoperfusion in multiple areas The diagnosis
of Hashimoto's encephalopathy was made and the patient was referred to the Department of
Internal Medicine of S. João Hospital. High doses of intravenous methylprednisolone were
prescribed. Later, she began treatment with oral prednisone (60 mg/day) with normalization of
antithyroglobulin antibody titers and improvement in both her psychiatric symptoms and her
EEG after a few months.

At that time, the patient's psychotic symptoms had disappeared, and she remained symptom
free at the time of her last ambulatory visit (18 months after discharge). The patient had insight
into her behavior during the psychotic episode.

Hashimoto's encephalopathy is an unusual entity that is associated with Hashimoto's thyroiditis.

It has been described as a syndrome of encephalopathy and high serum antithyroid antibody
concentrations that is responsive to glucocorticoid therapy . However, there is no evidence that
the antibodies have a pathogenic role, but they might be markers of some other autoimmune
disorder affecting the brain .

The first description of Hashimoto's encephalopathy was made by Brain et al. and, as of 2003,
only 105 cases had been reported . Myoclonus, epileptic seizures, dementia, and
disturbances of consciousness are the most common features . Psychotic symptoms are
frequent .

Although the pathogenesis of the disease remains unclear, several authors have suggested
different hypotheses . Localized cerebral edema , autoimmune vasculitis , or a toxic effect of
thyrotropin-releasing hormone have been postulated as possible causes. Recently, it has been
suggested that the immunopathological basis for this syndrome may be similar to relapsing
acute disseminatedencephalomyelitis

In summary, this is a case of Hashimoto's encephalopathy with psychotic features: a young

woman with previous history of thyroid disease presenting with several brief and acute psychotic
episodes (preceded by severe sleep disturbance) and abnormal EEG.

It is our opinion that Hashimoto's encephalopathy should be considered in the differential

diagnosis of atypical psychosis, because it is a treatable syndrome . This is particularly important
in patients with a previous history of thyroid disease, despite current normal thyroid function.

Glenn A. Melvin, PhD, provided editorial assistance.

Hashimoto's encephalitis: Introduction
Hashimoto's encephalitis: A rare but serious condition that can occur when thyroid
antibodies, such as those present in Hashimoto's thyroiditis, involve the brain tissue and
cause neurological symptoms.

Hashimoto’s Encephalopathy, Steroid responsive encephalopathy associated with autoimmune

thyroiditis (SREAT), non - vasculitic autoimmune meningoencephalitis.

Background
Hashimoto’s encephalitis (inflammation / swelling of the brain) was first described in 1966. It is a
rare, probably autoimmune condition. ( a disorder in which the immune system mistakenly
attacks and destroys healthy body tissues). It is usually associated with high levels of thyroid
antibodies in the blood. Hashimoto’s encephalitis has been reported in children, adults and the
elderly all over the world. It is more common in females than males.

What are the symptoms?

The clinical presentation is often a depression of brain function, drowsiness and sometimes
coma. Two types have been described: (1) The relapsing and remitting type (symptoms at times
worse and other times better) which manifests with encephalopathy and stroke – like episodes.
(2) The diffuse progressive (widely dispersed) type which has a slow onset, progressive course
with occasional fluctuations and manifest with psychiatric symptoms such as confusion,
disorientation and psychosis. Either of these may present with tremors, jerks or epileptic
seizures.

What are the causes?

Currently, the exact cause is unknown. The thyroid antibodies are a marker rather than the
cause of the problem. Thyroid function is usually normal.

How is it diagnosed?
It is a diagnosis of exclusion and the differential is wide, ranging from stroke-like episodes,
Alzheimer’s disease, Creutzfeldt-Jacob disease, to rare inborn errors of metabolism. Useful
tests include Magnetic Resonance Imaging (MRI) of the brain, Electroencephalogram (EEG),
Cerebrospinal Fluid (CSF) findings, endocrine and metabolic screens, and viral studies.

How is it treated?
Most patients with Hashimoto’s encephalitis improve with high dose steroids (Prednisolone).
However, improvement may take weeks or even months. Because of the improvement with
steroids, some experts refer to the condition as “steroid responsive encephalopathy associated
with autoimmune thyroiditis (SREAT)”. Plasma exchange and intravenous immunoglobulin
(IVIG) have been used in some patients. The prognosis with treatment is generally good.
Steroids are often continued for many months.
Reaction

I am amazed by the article since it deals with delusions and hallucinations that captured
my attention .Although, in the said article, it was said that Hashimoto's encephalopathy should
be considered in the differential diagnosis of atypical psychosis, especially because this is a
treatable syndrome. Considering the fact that HE is a rare but also a serious condition that was
often mistakenly diagnosed as psychotic disorder.

According to some sources, Hashimoto’s encephalitis (inflammation / swelling of the

brain) was first described in 1966. It is a rare, probably autoimmune condition. ( a disorder in
which the immune system mistakenly attacks and destroys healthy body tissues). It is usually
associated with high levels of thyroid antibodies in the blood. Hashimoto’s encephalitis has been
reported in children, adults and the elderly all over the world. It is more common in females than
males.

Although, I still haven’t met anyone who have this rare condition, but maybe in the near
future if I have a chance to do so, it would be my pleasure to have a patient having HE because
having a knowledge about this condition can already aid me in dealing with my patient. By this
simple article, it can help nurses understand the condition further more.

And I also realized that hallucinations, delusion and the like is not only for psychotic
patients but it can

A simple test found her Thyroid antibodies (TPO) extremely high. Sometimes this causes an
autoimmune disorder and attacks parts of the body - in her case, her brain. They call this
Hashimoto's Encephalitis or Steroid responsive Encephalitis. Steroids have helped reduce the
antibody level, but we haven't seen much improvement with her mentally.