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Sudden bilateral blindness in Wernicke's encephalopathy: Case report and review of the literature

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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.

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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