Short communicationSudden bilateral blindness in Wernicke's encephalopathy: Case report and review of the literature
Introduction
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], [3], [4], [5], [6], [7], [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.
Section snippets
Case report
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
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
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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