YAJEM-57214; No of Pages 3
American Journal of Emergency Medicine xxx (2017) xxx–xxx

Contents lists available at ScienceDirect

American Journal of Emergency Medicine
journal homepage: www.elsevier.com/locate/ajem

Multiple cerebral infarct with cerebral vasculitis in a young patient with
ulcerative colitis
Maeng Real Park, Mun Ki Min ⁎, Ji Ho Ryu, Dae Sub Lee, Kang Ho Lee
Department of Emergency Medicine, Pusan National University Yangsan Hospital, South Korea

a r t i c l e

i n f o

Article history:
Received 26 December 2017
Received in revised form 28 December 2017
Accepted 3 January 2018
Available online xxxx

a b s t r a c t
Ulcerative colitis (UC) is a chronic and debilitating disorder, characterized by inflammation of the colonic mucosa.
UC can be considered a systemic disorder but UC-related manifestations in the central nervous system (CNS) are
quite rare.
A 29-year-old man was admitted to the emergency department with repeated generalized tonic-clonic (GTC)
type seizures. Based on brain CT, brain metastasis or hemorrhagic infarct was suspected. Diffusion-weighted
image of brain MRI showed high signal in the left thalamus and heterogenous enhancement in the right parietal
and left frontal lobes. This image indicated a cerebral infarct, but could not completely rule out cerebral metastasis and vasculitis, or any other pathology. However, the brain biopsy revealed multiple thromboemboli with
acute inflammation and necrosis. Thus, the patient was diagnosed with multiple cerebral infarcts with cerebral
vasculitis, occurring as a complication of UC.
In conclusion, CNS manifestations of UC are rare. However, clinicians should consider uncommon diagnoses like
vasculitis and thromboembolism in patients with UC presenting with seizures.
© 2017 Published by Elsevier Inc.

1. Introduction

2. Case report

Ulcerative colitis (UC) is a chronic and debilitating disorder, characterized by inflammation of the colonic mucosa. However, UC can be
considered as a systemic disorder and a recent editorial in Gastroenterology states, “the effects of inflammatory bowel disease (IBD) extend to
every corner of the body.” [1,2]. Extraintestinal manifestations include
ocular (Uveitis and iritis), hepatic (primary sclerosing cholangitis and
autoimmune hepatitis), arthritic, skeletal, hematologic, respiratory,
and cutaneous manifestations [2,3].
However, UC-related manifestations in the central nervous system
(CNS) are quite rare. Cerebrovascular disorders have been documented
in 0.12 to 4% of all patients with IBD and they represent the most commonly described neurologic complications [4].
We report a case of UC where the patient presented to the emergency department for a seizure. Subsequently, the patient was diagnosed with multiple cerebral infarcts with cerebral vasculitis, caused
by UC-related complications.

A 29-year-old man was admitted to the emergency department with
repeated generalized tonic-clonic (GTC) type seizures. On arrival, he
had an occurrence of the GTC-type seizure for 1 min and he was in a stupor. His vital signs showed blood pressure, 130/80 mm Hg; heart rate,
99 beats/min; respiratory rate, 18/min; and body temperature, 38.5 °C.
He had been diagnosed with UC 3 years ago. For differential diagnosis, computed tomography (CT) of the brain was performed. The CT scan
revealed two low-density lesions with internal hemorrhage in the right
parietal and left frontal lobes (this did not rule out tumor, infarction
with hemorrhagic transformation, etc.) (Fig. 1).
Initial laboratory reports were as follows: arterial blood gas analysis
(pH, 7.05; pCO2, 30; pO2, 117; base excess, −21.0; oxygen saturation,
96%); complete blood count (white blood cell count, 16,410/μL; hemoglobin, 12.0 g/dL); aspartate aminotransferase/alanine aminotransferase (AST/ALT), 43/49 IU/L; blood urea nitrogen/creatinine, 7.4/
1.36 mg/dL; serum sodium/potassium/chloride (NA+/K+/Cl−), and
145/3.5/106 mmol/L. Chest CT revealed no remarkable findings and abdominal CT revealed ulcerative colitis in the active phase. Brain magnetic resonance imaging (MRI) was performed for additional
differential diagnosis. Brain MRI showed heterogeneous, enhancing,
T2-high-signal lesions in the right parietal and left frontal lobes, with internal diffusion restriction and hemorrhage, and focal diffusion restriction in the left thalamus (Fig. 2).

⁎ Corresponding author at: Pusan National University Yangsan Hospital, Beomeo-ri,
Mulgeum-eup, Yangsan-si, Gyeongsangnam-do 626-770, South Korea.
E-mail address: minmk99@pusan.ac.kr (M.K. Min).

https://doi.org/10.1016/j.ajem.2018.01.006
0735-6757/© 2017 Published by Elsevier Inc.

Please cite this article as: Park MR, et al, Multiple cerebral infarct with cerebral vasculitis in a young patient with ulcerative colitis, American Journal of Emergency Medicine (2017), https://doi.org/10.1016/j.ajem.2018.01.006

2

M.R. Park et al. / American Journal of Emergency Medicine xxx (2017) xxx–xxx

and necrosis. We obtained a thoracic echocardiogram to locate the
source of emboli. The echocardiogram revealed normal heart function.
There was no specific source of emboli as per the previously performed
chest CT and abdominal CT. Since the specific source of emboli could not
be found, multiple cerebral infarcts with cerebral vasculitis were
thought to be a complication caused by UC, which could have led to
seizures.
After brain biopsy, he was transferred to the gastroenterology unit
for the treatment of UC. During hospitalization, he developed right facial
palsy and involuntary movement of the right upper limb. On follow-up
brain MRI, appearance of new diffusion restriction lesions was detected
in the left middle cerebral artery region, near the previous infarct lesion.
Diffuse narrowing of both cervical internal carotid, left vertebral, and
common carotid arteries (vasculitis), along with subtle beaded appearance of both middle cerebral artery distal branches, and suspicious irregular susceptibility weighted image dark signal along the expected
course of the vessel was seen (cerebral vasculitis) (Fig. 3). His condition
improved after methylprednisolone administration, and he recovered
from neurological deficits by the time of discharge.

Fig. 1. The brian CT showed two low-density lesions with internal hemorrhage in the right
parietal and left frontal lobes. But, this CT couldn't rule out tumor, infarction with
hemorrhagic transformation, etc.

3. Discussion

He improved from stupor to the drowsy state 10 h after admission.
However, the brain MRI was unable to indicate the final diagnosis
from among the several differential diagnoses (brain metastasis, cerebral infarct, vasculitis, etc.). Hence, the patient underwent brain biopsy.
The biopsy revealed multiple thromboemboli, with acute inflammation

UC is an idiopathic chronic IBD, usually affecting patients between
the age of 20 and 30 years, with a second peak age between the age of
70 and 80 years [5]. UC-associated vasculitis in the CNS is a rare neurological manifestation of systemic IBD. As mentioned earlier, cerebrovascular manifestations are seen in 0.12–4% of cases with irritable bowel
syndrome [6]. CNS manifestations include arterial and venous

Fig. 2. Brain MRI showed heterogenous, enhancing, T2-high-signal lesions in the right parietal and left frontal lobes, with internal diffusion restriction and hemorrhage, and focal diffusion
restriction in the left thalamus.

Fig. 3. On follow-up brain MRI, appearance of new diffusion restriction lesions was detected in the left middle cerebral artery region, near the previous infarct lesion. Diffuse narrowing of
both cervical internal carotid, left vertebral, and common carotid arteries (vasculitis), along with subtle beaded appearance of both middle cerebral artery distal branches, and suspicious
irregular susceptibility weighted image dark signal along the expected course of the vessel was seen (cerebral vasculitis).

Please cite this article as: Park MR, et al, Multiple cerebral infarct with cerebral vasculitis in a young patient with ulcerative colitis, American Journal of Emergency Medicine (2017), https://doi.org/10.1016/j.ajem.2018.01.006

M.R. Park et al. / American Journal of Emergency Medicine xxx (2017) xxx–xxx

thrombosis, seizures, leukoencephalitis, and vasculitis [7]. Our patient
presented with repeated GTC-type seizures. Based on brain CT, brain
metastasis or hemorrhagic infarct was suspected. Diffusion-weighted
image of brain MRI showed high signal in the left thalamus and heterogeneous enhancement in the right parietal and left frontal lobes. This
image indicated a cerebral infarct, but could not completely rule out cerebral metastasis and vasculitis, or any other pathology. However, the
brain biopsy revealed multiple thromboemboli with acute inflammation and necrosis. Thus, the patient was diagnosed with multiple cerebral infarcts with cerebral vasculitis, occurring as a complication of UC.
With respect to the pathogenesis of UC-associated vasculitis, most
authors think it is an immune-mediated mechanism, possibly because
of genetic susceptibility and human leukocyte antigen status, Tlymphocyte-mediated cytotoxicity, or immune complex deposition
[2,8,9].
Regarding the pathogenesis of thromboembolism, a non-specific hypercoagulable state has been vaguely hypothesized. This state was associated with increased fibrinogen levels and factor VII activity,
thrombocytosis, and shortened partial thromboplastin time [2]. Several
studies have reported that patients with UC have a three-to four times
increased risk of thromboembolism [2,10,11].
Patients of UC with concurrent cerebral vasculitis recovered from
their neurological deficits with prednisolone treatment or prednisolone
combined with cyclophosphamide treatment [7,12-14]. Our patient was
treated with methylprednisolone and recovered from his neurologic
deficit.
In conclusion, CNS manifestations of UC are rare. However, clinicians
should consider uncommon diagnoses like vasculitis and thromboembolism in patients with UC presenting with seizures.

3

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Please cite this article as: Park MR, et al, Multiple cerebral infarct with cerebral vasculitis in a young patient with ulcerative colitis, American Journal of Emergency Medicine (2017), https://doi.org/10.1016/j.ajem.2018.01.006