International Journal of Celiac Disease, 2017, Vol. 5, No. 2, 81-82
Available online at http://pubs.sciepub.com/ijcd/5/2/3
©Science and Education Publishing
DOI:10.12691/ijcd-5-2-3
Celiac Disease Hiding Behind Cerebral White Matter
Lesions: A Case Report
Mohammed Elhassan
1,*
, Guha Venkatraman
2
1
Department of Medicine, University of California in San Francisco (UCSF)/Fresno, Fresno, CA, USA
2
Department of Medicine, St. Barnabas Medical Center, Livingston, NJ, USA
*Corresponding author: melhassan@fresno.ucsf.edu
Abstract Celiac disease, or celiac enteropathy, is a relatively common autoimmune disease with myriad of
presentations, including intestinal and extra-intestinal manifestations. It is also a less common cause of cerebral
white matter lesions in the absence of other etiologies. If such patients are symptomatic, diagnosis can be
challenging, especially when gastrointestinal symptoms are absent. The differential diagnosis in such cases is wide
and high index of suspicion is needed to make the diagnosis. We presented a case of celiac disease who presented
with neurological symptoms and white matter lesions in brain magnetic resonance imaging without gastrointestinal
symptoms.
Keywords: celiac disease, leukoencephalopathies
Cite This Article: Mohammed Elhassan, and Guha Venkatraman, “Celiac Disease Hiding Behind Cerebral
White Matter Lesions: A Case Report.” International Journal of Celiac Disease, vol. 5, no. 2 (2017): 81-82.
doi: 10.12691/ijcd-5-2-3.
1. Background
Celiac disease (CD) is common (almost 1% of the Western
population [1]) and has many extra-gastrointestinal
manifestations [2]. Some neurological complications such
as ataxia, peripheral neuropathy, and epilepsy are well-
recognized [3]. However, neurological symptoms with
white matter lesions (WMLs) in brain imaging without
specific underlying etiology is a less recognized variant
[4,5]. Here we describe a patient who suffered from
unexplained neurological symptoms without accompanying
gastrointestinal manifestations and with brain MRI showing
WMLs who was found, after extensive work up, to have CD.
2. Case Report
A 58-year-old woman with a history of depression and
aortic regurgitation was referred for neurological evaluation
as she was suffering from intermittent headaches and
impaired memory for three years and episodic left arm and
leg weakness for one year. She attributed her symptoms to
an injury she endured three years ago, when she fell and
hit the back of her head. She denied diarrhea or weight
loss and did not report history of alcohol or illicit drug sue.
Family history was not significant for gastrointestinal or
neurological disease. On neurological exam, the patient
was fully oriented, cooperative, and had no speech or
short term memory deficits. Cranial nerves, motor
function and coordination were intact. The sensory exam
was remarkable for a mild decrease in vibration sense in
the right foot. The rest of her examination was
unremarkable. Brain magnetic resonance imaging (MRI)
showed nonspecific WMLs scattered in the left frontal and
parietal lobes without enhancement. Laboratory studies
were unyielding, including: complete blood count, complete
metabolic panel, thyroid function tests, serum iron,
vitamin B12, vitamin E, and heavy metals. Evaluation for
Human Immunodeficiency Virus, Human T-Lymphotropic
Virus 1 and 2, Lyme disease, Ehrlichiosis, Babesiosis, and
anaplasmosis were unremarkable. Additionally, autoimmune
antibodies were not present and examination of cerebrospinal
fluid including assessment for oligoclonal bands was
unremarkable. Magnetic resonance angiography (MRA) of
the head and neck did not show stenotic or vasculitic
changes and a 72-hour ambulatory electroencephalogram
did not reveal seizure activity. In an attempt to find other
rare causes of her clinical picture, celiac disease work
up was requested. Anti-tissue transglutaminase IgG,
anti-gliadin IgA and anti-gliadin IgG came back positive
at 49 U/ml (normal <5), 27 (normal <11), and 25 (normal <11),
respectively. Subsequently, the patient underwent duodenal
biopsy and CD was confirmed with Marsh 3c histology.
She was referred to a center specialized in treating patients
with celiac disease and was started with intravenous
immunoglobulins in addition to the gluten-free diet as
decided by the specialist, with subsequent improvement in
her symptoms and decrease in her antibody titers.
3. Discussion
This case supports the notion that evaluation for CD is
reasonable part of the work-up of patients with neurological
symptoms when etiology is unknown, particularly with
the presence of WMLs, even in the absence of enteropathy.