Wernicke-Korsakoff Syndrome Workup
- Author: Glen L Xiong, MD; Chief Editor: David Bienenfeld, MD more...
Approach Considerations
Initial laboratory studies are directed at the differential diagnostic possibilities that can be identified and corrected rapidly.
Maintain a high level of suspicion for thiamine deficiency to avoid iatrogenic precipitation of Wernicke-Korsakoff syndrome. Heightened awareness should lead to prophylactic supplementation in at-risk patients.
This syndrome is most commonly observed in patients with alcoholism. Consequently, when these patients present to an emergency department, they are routinely administered thiamine prior to glucose infusion.
Several other categories of patients are at increased risk for thiamine deficiency, including inpatients receiving total parental nutrition, which necessitates vigilant monitoring for indicative signs and symptoms to ensure prompt treatment.
Coding
The Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR) does not consider Wernicke-Korsakoff syndrome as a distinct entity. However, if dementia is prominent, for Axis I purposes, dementia due to [General Medical Condition] (294.1) may be used for coding purposes. If acute confusion is prominent, then the code for delirium [due to medical condition] (293.0) may be considered.
The International Statistical Classification of Diseases and Related Health Problems (ICD-9) code includes (291.1) Alcohol-induced persisting amnestic disorder; Wernicke-Korsakoff Syndrome (alcohol). Since the ICD-9 codes link the diagnosis to alcohol-related conditions, non-alcohol associated Wernicke-Korsakoff syndrome would require other nonspecific codes, eg, (265.1) Other and unspecified manifestations of thiamine deficiency; Other vitamin B-1 deficiency states.
Laboratory Tests
Laboratory studies to detect various morbidities in the diagnosis of Wernicke-Korsakoff syndrome include the following:
- Electrolytes - To rule out an acute metabolic derangement causing mental confusion
- Complete blood count (CBC) - To rule out an acute infectious process, thrombocytosis, or erythrocytosis predisposing to thrombosis and infarction
- Coagulation panel - To evaluate for a potential bleeding diathesis
- Arterial blood gas - To evaluate for hypoxemia, hypercarbia, acidosis, or alkalosis
- Serum/urine toxic drug screen - To rule out acute toxic ingestion; this is most helpful if results are available rapidly
- Liver-associated enzymes - May provide evidence of alcohol abuse or liver dysfunction
Serum thiamine levels
Serum thiamine levels have been used in case reports to confirm Wernicke-Korsakoff syndrome. However, studies have not directly examined the correlation between a critical serum thiamine level and the development of neurologic symptoms and Wernicke-Korsakoff syndrome itself. One small study did show that the serum thiamine level correlated well with cerebellar vermal atrophy.[12] For now, the role of serum thiamine levels remains uncertain, although it may eventually play a part in confirming the diagnosis of Wernicke-Korsakoff syndrome and/or monitoring adequacy of treatment.
Lumbar puncture/CSF analysis
Seriously consider this procedure for any confused patient with fever and/or headache, particularly elderly patients, to rule out an infectious etiology. Patients with Wernicke-Korsakoff syndrome have a protein content that is within the reference range or mildly elevated without pleocytosis on CSF analysis.
Imaging Studies
CT brain scan (noncontrast)
Computed tomography (CT) scanning can help in the rapid assessment for hemorrhage, mass effect, edema, and large, subacute stroke.
MRI of the brain with contrast
Magnetic resonance imaging (MRI) demonstrates acute lesions of Wernicke-Korsakoff syndrome in the dorsal medial thalamic, periventricular region of the third ventricle, periaqueductal area, mamillary bodies, and dorsal medulla. Contrast enhancement of the mamillary bodies were correlated with alcohol[13] and nonalcoholic causes,[14] so whether MRI findings may be specific to the etiology of Wernicke-Korsakoff Syndrome remains uncertain.
MRI can be a useful diagnostic procedure in patients presenting with a suggestive history and stupor or coma, in whom ataxia and ophthalmoplegia are not detectable.
Thomson AD, Marshall EJ. The natural history and pathophysiology of Wernicke's Encephalopathy and Korsakoff's Psychosis. Alcohol Alcohol. Mar-Apr 2006;41(2):151-8. [Medline]. [Full Text].
McEntee WJ, Mair RG. Memory enhancement in Korsakoff's psychosis by clonidine: further evidence for a noradrenergic deficit. Ann Neurol. May 1980;7(5):466-70. [Medline].
Kesler A, Stolovitch C, Hoffmann C, Avni I, Morad Y. Acute ophthalmoplegia and nystagmus in infants fed a thiamine-deficient formula: an epidemic of Wernicke encephalopathy. J Neuroophthalmol. Sep 2005;25(3):169-72. [Medline].
Chiossi G, Neri I, Cavazzuti M, Basso G, Facchinetti F. Hyperemesis gravidarum complicated by Wernicke encephalopathy: background, case report, and review of the literature. Obstet Gynecol Surv. Apr 2006;61(4):255-68. [Medline].
Singh S, Kumar A. Wernicke encephalopathy after obesity surgery: a systematic review. Neurology. Mar 13 2007;68(11):807-11. [Medline].
Alcaide ML, Jayaweera D, Espinoza L, Kolber M. Wernicke's encephalopathy in AIDS: a preventable cause of fatal neurological deficit. Int J STD AIDS. Oct 2003;14(10):712-3. [Medline].
Harrison RA, Vu T, Hunter AJ. Wernicke's encephalopathy in a patient with schizophrenia. J Gen Intern Med. Dec 2006;21(12):C8-C11. [Medline].
Yae S, Okuno S, Onishi H, Kawanishi C. Development of Wernicke encephalopathy in a terminally ill cancer patient consuming an adequate diet: a case report and review of the literature. Palliat Support Care. Dec 2005;3(4):333-5. [Medline].
Ueda K, Takada D, Mii A, Tsuzuku Y, Saito SK, Kaneko T, et al. Severe thiamine deficiency resulted in Wernicke's encephalopathy in a chronic dialysis patient. Clin Exp Nephrol. Dec 2006;10(4):290-3. [Medline].
Harper C, Fornes P, Duyckaerts C, Lecomte D, Hauw JJ. An international perspective on the prevalence of the Wernicke-Korsakoff syndrome. Metab Brain Dis. Mar 1995;10(1):17-24. [Medline].
Sechi G, Serra A. Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management. Lancet Neurol. May 2007;6(5):442-55. [Medline].
Maschke M, Weber J, Bonnet U, Dimitrova A, Bohrenkamper J, Sturm S, et al. Vermal atrophy of alcoholics correlate with serum thiamine levels but not with dentate iron concentrations as estimated by MRI. J Neurol. Jun 2005;252(6):704-11. [Medline].
Zuccoli G, Gallucci M, Capellades J, Regnicolo L, Tumiati B, Giadas TC, et al. Wernicke encephalopathy: MR findings at clinical presentation in twenty-six alcoholic and nonalcoholic patients. AJNR Am J Neuroradiol. Aug 2007;28(7):1328-31. [Medline].
Fei GQ, Zhong C, Jin L, Wang J, Zhang Y, Zheng X, et al. Clinical characteristics and MR imaging features of nonalcoholic Wernicke encephalopathy. AJNR Am J Neuroradiol. Jan 2008;29(1):164-9. [Medline].
Traviesa DC. Magnesium deficiency: a possible cause of thiamine refractoriness in Wernicke-Korsakoff encephalopathy. J Neurol Neurosurg Psychiatry. Aug 1974;37(8):959-62. [Medline].
Hack JB, Hoffman RS. Thiamine before glucose to prevent Wernicke encephalopathy: examining the conventional wisdom. JAMA. Feb 25 1998;279(8):583-4. [Medline].
Harper CG, Sheedy DL, Lara AI, Garrick TM, Hilton JM, Raisanen J. Prevalence of Wernicke-Korsakoff syndrome in Australia: has thiamine fortification made a difference?. Med J Aust. Jun 1 1998;168(11):542-5. [Medline].
Day E, Bentham P, Callaghan R, Kuruvilla T, George S. Thiamine for Wernicke-Korsakoff Syndrome in people at risk from alcohol abuse. Cochrane Database Syst Rev. 2004;CD004033. [Medline].
Cochrane M, Cochrane A, Jauhar P, Ashton E. Acetylcholinesterase inhibitors for the treatment of Wernicke-Korsakoff syndrome--three further cases show response to donepezil. Alcohol Alcohol. Mar-Apr 2005;40(2):151-4. [Medline].
Rustembegovic A, Kundurovic Z, Sapcanin A, Sofic E. A placebo-controlled study of memantine (Ebixa) in dementia of Wernicke-Korsakoff syndrome. Med Arh. 2003;57(3):149-50. [Medline].
Luykx HJ, Dorresteijn LD, Haffmans PM, Bonebakker A, Kerkmeer M, Hendriks VM. Rivastigmine in Wernicke-Korsakoff's syndrome: five patients with rivastigmine showed no more improvement than five patients without rivastigmine. Alcohol Alcohol. Jan-Feb 2008;43(1):70-2. [Medline].

