Journal of the Neurological Sciences 260 (2007) 261 – 264
www.elsevier.com/locate/jns

Short communication

Sudden bilateral blindness in Wernicke's encephalopathy:
Case report and review of the literature
Rainer Surges ⁎, Sandra Beck, Wolf-Dirk Niesen, Cornelius Weiller, Michel Rijntjes
Department of Neurology, University of Freiburg, Breisacherstr, 64, 79106 Freiburg, Germany
Received 5 February 2007; received in revised form 20 March 2007; accepted 3 April 2007
Available online 18 May 2007

Abstract
We report on a patient suffering from bilateral sudden blindness as initial symptom of Wernicke's encephalopathy (WE). A 37-year-old
male alcoholic was admitted to a psychiatric clinic because of excessive alcohol consumption (3.4‰). 24 h later he developed acute bilateral
blindness with no light perception, downbeat nystagmus, bilateral ocular abduction deficits, cerebellar ataxia as well as a slight psychomotor
slowing and mild disorientation. MRI including diffusion-weighted imaging and MR-angiography 3 h after symptom onset did not reveal
findings suggestive for ischemic stroke. Immediate iv-application of thiamine led to a nearly complete remission of the neuroophthalmologic
symptoms within 12 h. Although we critically discuss other potential etiologies, we conclude that the complex clinical picture with initial
sudden blindness is an unusual presentation of WE.
© 2007 Elsevier B.V. All rights reserved.
Keywords: Wernicke's encephalopathy; Acute blindness; Downbeat nystagmus; Stroke; Alcohol; Diffusion-weighted imaging; Metabolic brain disease

1. Introduction

2. Case report

WE is caused by a thiamine deficit leading to disturbances
in cerebral glucose metabolism. Typical early symptoms
include oculomotor deficits (frequently bilateral abduction
deficits due to VI nerve palsies, and vertical/horizontal
nystagmus), ataxia and behavioural/mental disturbances [1].
To date, associated loss of vision has been reported in 8 cases
only and mainly developed progressively over a couple of
days or could be explained by papilledema or retinal
hemorrhage [cf. Table 1, [2–8]]. In the present case bilateral
blindness occurred suddenly without acute retinal affection
and as an initial symptom. This sudden onset raises the
question of other underlying etiologies, especially of brain
ischemia, revealing the absence of highly sensitive diagnostic tools in WE and the presence of the diagnostic gap
between transient ischemic attack and abnormalities in
diffusion-weighted imaging (DWI) in MRI.

A 37-year old male with chronic alcohol abuse was
admitted to a nearby psychiatric clinic due to alcohol
intoxication (blood alcohol level 3.4‰). 24 h later he
suffered from acute, stroke-like bilateral blindness with no
light perception (“as if somebody has switched off the
light”) and was referred to our centre within 2 h. His
medical history comprised a perinatal neuroophthalmologic affection of unknown etiology with a residual scotoma
within the temporal visual field on the right eye as well as a
discrete esotropia (with deviation of the right bulbus).
These neuroophthalmologic problems were stable over
more than 30 years. There has been a history of heavy
alcohol abuse and chronic tobacco abuse since several
years. On admission, the malnourished patient (172 cm,
58 kg, body mass index 19.6 kg/m 2 ) was awake, oriented
to time and person, co-operative without apparent mnestic
deficits, but with a slight psychomotor slowing and
disorientation to his own situation. He denied headache
and had no history of migraine. The neuroophthalmologic
picture was complex: he presented prominent esotropia

⁎ Corresponding author. Tel.: +49 761 2705296; fax: +49 761 2705310.
E-mail address: rainer.surges@uniklinik-freiburg.de (R. Surges).
0022-510X/$ - see front matter © 2007 Elsevier B.V. All rights reserved.
doi:10.1016/j.jns.2007.04.005

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R. Surges et al. / Journal of the Neurological Sciences 260 (2007) 261–264

Table 1
Review of visual loss during Wernicke's encephalopathy
Ref. Age/
sex

Clinical course of
visual loss

Neuroophthalmology

Acuity/funduscopy/ VEP (visually
Presumed site of
visual field
evoked potentials) action
(initial examination)

[2]

2 patients. Blindness
on day 28/35 a, onset
unclear, both patients
died.
Incomplete loss of vision.
Onset and evolution not
specified.

Nystagmus

–/Papilledema
on day 21 + 28 a/–

[3]

20/F

[4]

40/
M

Progressive visual loss
over a couple of days.
Incomplete recovery
within 3 months upon
thiamine.

Horizontal/vertical
gaze palsies; LR+

Perception of
light only/
normal/–

Uncooperative
patient

Cortical

[5]

64/F

Progressive visual loss
over a couple of days.
Substantial improvement
within 1 month upon
thiamine.
Blindness, onset unclear.
Recovery within 1 month
upon thiamine.
Progressive visual loss
over 10 days to blindness.
Substantial improvement
within 24 h upon thiamine.
Sudden onset blindness
(no light perception).
Recovery within a couple
of days upon thiamine.
Sudden onset blindness
(no light perception).
Recovery within 12 h
upon thiamine.

Nystagmus upon
horizontal gaze

–/normal/–

Flash VEP not
reproducible

Unclear

[6] b 39/
M
[7]

21/F

[8]

47/F

⁎

37/
M

Horizontal/vertical
Reduced/
nystagmus; Horizontal/ papilledema +
vertical gaze palsies
peripapillary
hemorrhage/–

Cranial imaging/MRI

MRI: symmetrical
hyperintensities
within the thalamus
and midbrain
(DWI, T2,
FLAIR sequences)
MRI: discrete white
matter lesions and
prominent bilateral
hyperintensities in
nucleus lentiformis (T2).
SPECT: unilateral
occipital hypoperfusion
and bilaterally in
thalamus and cerebellum.
MRI: symmetrical
hyperintensities within
the thalamus, midbrain
(T2, FLAIR)

Nystagmus

Horizontal/vertical
nystagmus; Incomplete
gaze palsies in all
directions
Vertical nystagmus on
upgaze; Incomplete
vertical/horizontal gaze
palsies; Esotropia, LR-,
Downbeat nystagmus;
Esotropia (bilateral
abduction deficits);
Mydriasis, LR+

–/bilateral
papilledema +
retinal
hemorrhages/–

–/atrophy of
the right optic
disc/–

MRI: normal

Pattern VEP
pathologic

Optic
nerves

Contrast enhanced
CT: normal

Not adequately
feasible

Cortical

MRI: normal

⁎: our patient.
a
Days after first and continued parenteral nutrition incidentally without thiamine.
b
Only abstract in English. LR: pupillary light reaction.

due to severe bilateral abduction defects, a spontaneous
downbeat nystagmus and a mydriasis with positive,
relatively slow pupillary light reaction. There was no
light perception, the menace reflex (brusk hand movements
in front of the patient) as well as the “mirror test” (mirror in
front of the patient to test whether there is an ocular pursuit
movement) were both negative, arguing against visual loss
of non-organic origin. The patient additionally presented
cerebellar ataxia with bilateral intention tremor on both
arms and legs, whereas there was no tremor at rest. Clinical
signs of polyneuropathy were completely absent, since all
tendon reflexes (ankle jerks included) were brisk without
Babinskis sign and sensation (vibration sensation in both
arms and legs included) was entirely preserved. Blood

analysis yielded an elevated Gamma-GT (145 U/l; normal
b 66 U/l) only. Other liver enzymes, cholinesterase and
alcohol level were normal. Thiamine was not examined.
ECG was normal. Blood pressure was in a range between
120–150/70–90 mm Hg during his stay in our hospital.
Stroke-MRI within 3 h after symptom onset revealed no
particularities (see Fig. 1). Since the symptoms were timely
related to excessive alcohol consumption, 100 mg intravenous thiamine was rapidly applied and continued during
the next days. Within 12 h, downbeat nystagmus as well as
vision had completely recovered, the bilateral abduction
deficits were markedly improved (a slight esotropia with
deviation of the right eye known since childhood
persisted). Ophthalmological examination the next day

R. Surges et al. / Journal of the Neurological Sciences 260 (2007) 261–264

263

Fig. 1. Cranial MRI. A: Axial DWI image without apparent diffusion deficits. B: “Time of Flight” angiography with normal flow signal in all cerebral arteries,
especially the basilar artery (asterisk) and posterior cerebral artery (pca). C: Coronal FLAIR sequence that only showed artefacts due to movement. D: Axial T2
image without particularities.

revealed the known visual field defect on the right eye due
to an atrophy of the optic disc but most importantly without
papilledema or retinal hemorrhage. Due to developing
delirium tremens with visual hallucinations after 24 h,
visually evoked potentials and Goldman perimetry were
not adequately feasible and the patient was re-referred to
the psychiatric clinic. The intention tremor and ataxia
persisted in a milder form for a further few days. On
discharge some weeks later, the patient was completely
independent and the neurological examination only
yielded the known scotoma and the discrete esotropia.
Unfortunately, further examinations (e.g. visually evoked
potentials and additional ophthalmologic examination)
were not done.
3. Discussion
WE is clinically diagnosed, since there are no highly
sensitive tools to prove this pathological entity. A low
serum level of thiamine and a low erythrocyte transketolase

activity can support the diagnosis but lack sensitivity and
may not be available immediately. Suggestive MRI findings
(bilateral lesions around the third/fourth ventricle, in the
medial parts of the thalamus, corpora mamillaria, mesencephalon, tegmentum and periaqueductal) can strengthen
the diagnosis, but sensitivity only amounts to about 50%
[9]. Newer works report on DWI abnormalities within the
typical localization of WE-associated lesions that might
precede lesions detected by other MRI sequences [3,10–
16]. In our patient DWI was performed 3 h after sudden
symptom onset and was without particularities, whereas the
earliest time scale reporting DWI abnormalities was 29 h so
far [12]. The most interesting feature is the sudden bilateral
blindness during the initial phase of the disease. Although
there are various toxicologic and metabolic etiologies
known to cause acute loss of vision [17], the most important
differential diagnosis to be ruled out is a transient ischemic
attack (TIA) in the vertebrobasilar territory. TIA cannot be
excluded completely, due to a diagnostic and methodological gap between DWI abnormalities and the occurrence of

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R. Surges et al. / Journal of the Neurological Sciences 260 (2007) 261–264

TIA: DWI abnormalities in TIA are reported in 35–44%
only with an increasing probability of DWI abnormalities
beyond 60 min [18,19]. In view of the normal Stroke-MRI
3 h after acute onset and still in the presence of the full
clinical picture, the bilateral pattern of neurological symptoms (especially bilateral abduction deficits and the
cerebellar ataxia, which does not fit a top of the basilar
syndrome, but rather WE), and the fast clinical recovery
following thiamine substitution within a clinical context of
chronic alcohol abuse, we suggest an unusual presentation
of WE.
Thiamine pyrophosphate is an essential coenzyme for
cerebral glucose metabolism and involved in the regulation of
the osmotic gradient across the cell membranes. Functional
loss of cortical cells during lack of thiamine is attributed to
disturbances in metabolism of glucose, local accumulation of
lactate, pyruvate and alanin leading to local acidosis [20] and
extra-/intracellular edema that may lead to demyelinisation,
glial proliferation, hemorrhagic lesions and necrosis at the
WE-typical lesion sites [21]. Concerning the acute vision loss
in our patient, the fast clinical recovery suggests a reversible
metabolic dysfunction which is not necessarily detected by
the recent DWI techniques, as outlined above. Because of the
preserved pupillary light reaction and the absence of acute
retinal damages we rather propose a cortical site of action,
what has already been reported before [4]. In contrast, e.g. a
nutritional (“tobacco–alcohol”) amblyopia is rather unprobable because of the preserved pupillary light reaction as well
as the fast clinical onset and recovery. An episode of a
relapsing chronic inflammatory demyelinating disease could
be suggested by the residual atrophy of the optic disc, but the
perinatal onset, the absence of clinical progression over more
than 30 years and the recent MRI imaging argue against this
hypothesis.
4. Conclusion
In summary, we report an unusual presentation of WE
with initial and sudden onset of bilateral blindness,
oculomotor deficits, cerebellar ataxia and a slight psychomotor slowing in a young male alcoholic. Because of the
diagnostic dilemma mentioned above, we suggest immediate
application of thiamine as well as diagnostic work-up to
detect ischemic strokes in rapidly evolving clinical pictures
within the context of chronic alcohol abuse.
Acknowledgement
We thank PD Dr. Ansgar Berlis from the Section of
Neuroradiology at the Department of Neurosurgery for
providing us the MRI images.

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