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Clinical Case Report

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Hypothalamic sydrome as an initial presentation
of Wernicke encephalopathy
A case report
Sha Zhu, MDa, Jun Qiang, MDa, Qing Xia, MDa, Yanshu Wang, MDa, Jun Zhang, MD, PhDb,
∗
Xianzeng Liu, MD, PhDa,b,
Abstract
Rationale: Wernicke encephalopathy (WE) is a syndrome characterized by an acute or subacute onset of ataxia, ophthalmoplegia,

and mental status changes. To our knowledge, hypothalamic syndrome is rare in WE.
Patient concerns: A 73-year-old female patient with acute cerebral infarct, who showed initial symptoms of vomiting, nausea,
ataxia, and subsequent anorexia, was treated with parenteral nutritional supplement for 20 days. Nevertheless, the patient still
developed refractory hyponatremia despite the appropriate sodium supplement given for a week following parenteral nutritional
supplement. In fact, after 14 days of parenteral nutritional supplement, the patient gradually showed hypotension and apathy.
Hyponatremia, hypotension, anorexia and apathy were signs of hypothalamic syndrome.
Diagnoses: Finally, the patient was diagnosed as WE by head magnetic resonance imaging, which showed symmetrical lesions in

T2-weighted imaging images and FLAIR high signal intensity in the periaqueduct, hypothalamus, thalamus, mammiliary bodies,
medulla oblongata, and vermis cerebelli.
Interventions: The patient was given thiamine supplementation.
Outcomes: The patient regained consciousness within 3 days. The sings of hyponatremia, hypotension, and apathy were relieved

subsequently.
Lessons: When patients develop unexplained hypothalamic syndrome, we should think of the possibility of WE. The concomitant
presence of hyponatremia, hypotension, anorexia, and apathy in WE is rare. Therefore, this case is reported here for discussion.
Abbreviations: CNS = central nervous system, CSWS = cerebral salt-wasting syndrome, MRI = magnetic resonance imaging,
SIADH = syndrome of inappropriate antidiuretic hormone secretion, WE = Wernicke encephalopathy.
Keywords: hypothalamic syndrome, thiamine, Wernicke encephalopathy

syndrome is characterized by endocrine and metabolic disorders,
along with vegetative nerve dysfunction, dysregulation of body
temperature, diet and sleep disorders, sexual dysplasia, neuropsychiatric changes, and epilepsy.
WE is an acute or subacute neuropsychiatric disorder triggered
by thiamine (vitamin B1) deficiency. First described by Carl
Wernicke in 1881, WE is featured by signs and symptoms such as
altered mental status, ataxia, and ocular signs, including
nystagmus and ophthalmoplegia. However, the above so-called
classical symptoms of WE may only be present in about 16% to
38% of the patients[1,2] and are relatively rare in nonalcoholic WE.
WE is most frequently associated with alcoholism, although the
incidence of nonalcoholic WE is becoming higher under conditions
of nutritional insufficiency, such as hyperemesis gravidarum,[3]
bariatric surgery, Crohn disease, prolonged parenteral feeding, or
even a hunger strike.[4] The diagnosis is confirmed in 0.4% to 2.8%
of autopsies, yet maybe overlooked in 68% of patients with
alcoholism and 94% of patients without alcoholism.[5]
To the knowledge of the authors, WE rarely causes
hypothalamic syndrome. Therefore, a rare case of WE-induced
hypothalamic syndrome is reported here for discussion.

1. Introduction
Hypothalamic syndrome is caused by a variety of processes,
including developmental abnormalities, primary tumors of the
central nervous system (CNS), vascular tumors, systemic tumors
affecting the CNS, and granulomatous diseases. Hypothalamic
Editor: N/A.
Written informed consent was obtained from the patient for publication of this
case repot and any related images.
The authors report no conflicts of inerest.
a
Department of Neurology, Peking University International Hospital, b Department
of Neurology, Peking University People’s Hospital, Beijing, China.
∗

Correspondence: Xianzeng Liu, Department of Neurology, Peking University
International Hospital, Beijing City, No. 1 Life Science Park life road, Changping
District, Beijing 102206, China (e-mail: liuxianzeng2004@sina.com); Sha Zhu,
Peking University International Hospital, Changping, Beijing 100193, China
(e-mail: zhusha@pkuih.edu.cn).

Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc.
This is an open access article distributed under the terms of the Creative
Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is
permissible to download, share, remix, transform, and buildup the work provided
it is properly cited. The work cannot be used commercially without permission
from the journal.
Medicine (2019) 98:26(e16181)

2. Case report

Received: 11 January 2019 / Received in final form: 28 April 2019 / Accepted: 3
June 2019

A 72-year-old female was admitted to our hospital with acute
cerebellar infarction and signs of nausea, vomiting, ataxia, and

http://dx.doi.org/10.1097/MD.0000000000016181

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examination. Neurological examination of the patient showed
slower pupillary response to light and more obvious signs of
nystagmus with paralysis of bilateral abduction nerves. Blood
tests showed that the white blood cells, C-reactive protein, and
procalcitonin of the patient were all elevated, although the signs
of fever were absent. The analysis of arterial blood gas indicated
respiratory alkalosis and lactic acidosis. The patient showed a pH
value of 7.31, an oxygen partial pressure of 157 mmHg, a carbon
dioxide partial pressure of 13 mmHg, and a lactic acid
concentration of 6.2 mmol/L. Chest computed tomography
showed signs of mild inflammation in both lungs. Routine urine
test and routine stool test did not show signs of infection. The
patient received head MRI again for fear of cerebral infarction
relapse. The MRI showed symmetrical lesions in T2-weighted
imaging images and FLAIR high signal intensity in the
periaqueduct, hypothalamus, thalamus, mammiliary bodies,
medulla oblongata and vermis cerebelli, which were consistent
with the diagnosis of WE (Fig. 2). On the same day, the patient
quickly fell into lethargy. At the time, the blood pressure of the
patient reached as low as 60/40 mmHg, with a heart rate of 130
bpm, a pH value of 7.31, an oxygen partial pressure of 65 mmHg,
a carbon dioxide partial pressure of 13 mmHg, and a lactic acid
concentration of 6.2 mmol/L in the arterial blood. Subsequently,
the patient was intubated and sent to intensive care unit, where an
electroencephalograph examination showed diffuse low-amplitude theta waves.
The patient was given 300 mg of intramuscular thiamine per
day for two weeks, followed by 100 mg of intramuscular
thiamine per day for another two weeks and then 300 mg of oral
thiamine tablet per day. At the same time, the patient received
anti-infection treatment and vasopressors. Within 3 days, the
patient became alert and her blood sodium level returned to
normal without the use of sodium supplementation. The blood
pressure of the patient was stabilized after one-week administration of vasopressors, although her signs of nystagmus did not
disappear until 45 days of thiamine supplementation was given.
Mini-Mental State Examination indicated impaired memory
functions. Unfortunately, the patient was not examined with
head MRI again after thiamine supplementation, so it is not clear
whether the intracranial lesions have improved. Nevertheless, the
signs of severe pneumonia of the patient were alleviated more
than 1 month later.

subsequent anorexia. The medical history of the patient included
hypertension and arrhythmia, although it was unclear whether
the patient ever experienced atrial fibrillation. The patient had no
diabetes, coronary heart disease, hyperlipidemia, and no history
of smoking and alcohol abuse. During physical examination, the
patient was conscious, fluent in speech, with both eyeballs
showing horizontal nystagmus. The limb muscle tone of the
patient was normal, with signs of ataxia in left upper and lower
limbs. Other items of the physical examination showed normal
results. The routine blood test, liver and kidney function,
electrolytes, blood glucose, and blood coagulation results of the
patient were also normal. The head magnetic resonance imaging
(MRI) of the patient showed bilateral cerebellar infarction (Fig. 1).
During hospitalization the appetite of the patient became
worse. After 1 month of hospitalization, the dizziness, nausea,
and vomiting of the patient were not alleviated. The patient then
received another head MRI examination. No other abnormalities
were found in this head MRI, except the marks of previous
cerebral infarction. After being treated with benzaisol and
ondansetron, the above symptoms of the patient gradually
improved. Nevertheless, the appetite of the patient got worse and
hence intravenous administration of nutritional support was
needed. The patient eventually developed hyponatremia after 1week administration of parenteral nutrition supplement, including high glucose, although the supplement of vitamins was not
sufficient. The blood sodium level of the patient was 121 to 128
mmol/L despite the use of an appropriate sodium supplement.
The blood osmolality and urine osmolality of the patient were
256 and 685 mOsm/Kg, respectively. On day 14 of parenteral
nutrition supplement, the patient presented with hypotension and
apathy. The blood pressure of the patient dropped to 95/60
mmHg and stayed there after all antihypertensive drugs were
stopped. The patient developed consciousness disorder on day 20
of parenteral nutrition supplement and was uncooperative in

3. Discussion
The patient in this study was first admitted because of acute
cerebral infarction characterized by dizziness, nausea, vomiting,
nystagmus, and ataxia. Therefore, WE was not considered until
the typical signs of WE were revealed by head MRI, which was
given when the patient showed signs of consciousness disturbance and nystagmus. Therefore, an opportunity for early
treatment was missed. Ataxia, ophthalmoplegia, and mental
confusion are characteristic symptoms of WE. Therefore, the
possibility of WE should not be excluded prematurely in
patients of posterior circulation infarction showing signs of
repeated nausea, vomiting, and anorexia, especially in patients
given intravenous nutrition supplement without thiamine
supplementation.
Thiamine is an essential vitamin with critical roles in glucose
metabolism. Thiamine cannot be synthesized in human body and
hence is mainly derived from food. Without supplement, the
thiamine reserve in the body can only last for about 18 days.[6]

Figure 1. Brain MRI DWI showing the bilateral cerebellar infaction (arrow).
DWI = diffusion-weighted imaging, MRI = magnetic resonance imaging.

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Figure 2. Brain MRI FLAIR demonstrating the symmetric lesions of hyperintensity at the periaqueduct and mammiliary bodies (A, arrow) and thalamic areas (B,
arrow). FLAIR = fluid attenuated inversion recovery, MRI = magnetic resonance imaging.

Hypotension is an uncommon sign of WE. After hyponatremia, the patient of this study showed hypotension
and lactic acidosis, and finally experienced circulatory
failure. Hypotension is associated with hypothalamic
damage, although lactic acidosis can also accelerate circulatory
failure.
Anorexia is one of the causes of nutrition supplemental
disorders and also a manifestation of WE. Thiamine deficiency
may induce anorexia by inhibiting hypothalamic adenosine
monophosphate-activated protein kinase activity in animal
experiments.[10] The patient of this study showed poor appetite
after developing repeated nausea and vomiting, although her
appetite got even worse after nausea and vomiting were
alleviated. It is speculated that anorexia might be an early
manifestation of WE in this patient.
Some studies have reported a mortality of 17% in patients
with acute WE. Therefore, the possibility of WE shall not be
excluded prematurely when unexplained signs of refractory
hyponatremia, hypotension, anorexia, and apathy are observed
in patients with nutrition supplemental disorders because an
early diagnosis and treatment of WE can avoid the onset of more
serious events.

Without thiamine, glucose is metabolized through less-efficient
anaerobic pathways, which in turn produce lactic acid during
glucose metabolism. Acidosis affects periventricular structures
and accounts for the clinical presentations of WE.[5] Eventually,
lactic acidosis may lead to circulatory failure. The patient in this
study was not offered thiamine supplementation when intravenous nutrition supplement was given, whereas high intravenous
glucose accelerated the consumption of thiamine. Finally, the
patient developed WE featured by disturbance of consciousness
and circulatory failure that could not be explained by pulmonary
infection.
In this study, the patient developed refractory hyponatremia,
hypotension, anorexia, and apathy, all of which were signs of
hypothalamic syndrome, in the early stage of WE. Such
hypothalamic symptoms are rare in WE. As far as the authors
know, hyponatremia and hypotension were only reported in
some WE cases, respectively.[7–9] Therefore, as a very rare
example, this case showed all above symptoms. Therefore, this
case was reported here to highlight the possibility of WE when
patients with nutrition supplemental deficiency developed
hypothalamic syndrome.
To our knowledge, 1 report of WE in the literature indicated
syndrome of inappropriate antidiuretic hormone secretion
(SIADH),[9] whereas another WE report revealed cerebral saltwasting syndrome (CSWS),[8] although both cases showed
hyponatremia. However, SIADH shows dilutional hyponatremia, a decreased urine volume, and euvolemia or hypervolemia
because of the excessive release of antidiuretic hormone, whereas
CSWS shows hyponatremia, serum hypoosmality, concentrated
urine, and natriuresis with dehydration. The patient in this study
showed intractable hyponatremia with normal urinary sodium,
normal osmotic pressure in the early stage of WE. In addition,
despite the use of appropriate sodium supplement, the hyponatremia of the patient was not improved. Therefore, the diagnosis
of SIADH seemed more suitable for this patient as all symptoms
of the patient were caused by hypothalamic damage. After
thiamine supplement was given to the patient, blood sodium
returned to normal.

Author contributions
Conceptualization: Jun Zhang.
Investigation: JUN Qiang, qing xia.
Supervision: yanshu wang.
Writing – original draft: SHA ZHU.
Writing – review & editing: Jun Zhang, Xianzeng Liu.

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