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Wernicke Encephalopathy

Wernicke's Encephalopathy

Wernicke encephalopathy is a potentially life-threatening condition of abnormal carbohydrate metabolism occurring on the grounds of thiamine deficiency. Alcohol abuse, which impairs thiamine absorption, is often the underlying cause. Principal symptoms are ophthalmoplegia, ataxia and acute changes in consciousness. The diagnosis is often missed, which is why a thorough clinical and laboratory workup is necessary to recognize it early on.


Presentation

Signs and symptoms of Wernicke encephalopathy (WE) stem from alterations of carbohydrate metabolism (inadequate absorption, accelerated metabolism or increased intake) together with the deficiency of thiamine (vitamin B1), as thiamine is a vital coenzyme of the Krebs cycle, the key step in metabolism of carbohydrates and energy production [1] [2] [3] [4]. Conditions that disrupt this process include hyperemesis (especially in pregnancy, known as hyperemesis gravidarum), acquired immunodeficiency syndrome (AIDS), administration of intravenous dextrose and other forms of parenteral nutrition, gastrointestinal tract (GI) surgery, but also malignancy and severe infections, all reducing the thiamine levels in the central nervous system (CNS) [1] [3] [5]. Chronic alcoholism, however, is by far the most widely recognized caused of WE, as a combination of poor diet, reduced storage of thiamine and long-term impairment of thiamine absorption from the GI tract [6]. Regardless of the cause, accumulation of toxic metabolites from impaired carbohydrate formation and degradation occurs, leading to a typical clinical presentation comprised of an altered mental state, ataxia, and ophthalmoplegia [4] [7]. Many reports have confirmed, however, that not all signs of WE may be present and that the diagnosis is often not recognized [1] [3] [4] [5] [7]. In fact, up to 68% of alcohol abusers and 94% of patients in whom alcoholism is not the cause of WE remain undiagnosed [1] [2]. WE can be life-threatening, as ischemia of the CNS can lead to irreversible neuronal damage or even death in the absence of an early diagnosis, which is why it is considered to be a medical emergency [1] [7].

Malnutrition
  • Wernicke encephalopathy secondary to thiamine deficiency should be considered as a possible cause of acute mental status changes in patients with acute pancreatitis and malnutrition.[ncbi.nlm.nih.gov]
  • This has become important as there has been a rise in malnutrition following the increasing incidence of bariatric surgery for obesity. A case report example involving review of the clinical presentation and treatment.[ncbi.nlm.nih.gov]
  • Wernicke’s encephalopathy due to thiamine (vitamin B 1 ) deficiency can be a complication of hyperemesis gravidarum; it can also occur in the context of alcohol use, bariatric surgery, or malnutrition.[nejm.org]
  • Causes  Chronic alcoholism – Malnutrition – reduced thiamine uptake and utilization  Prolonged starvation  Hyperemesis Gravidarum  Bariatric Surgery (Bariatric BeriBeri)  Malabsorption Syndromes  Infants on formula diet deficient in thiamine 5 6[slideshare.net]
  • Etiology Causes Thiamine deficiency is characteristically associated with chronic alcoholism, because it affects thiamine uptake and utilization. [ 2 ] In long-term alcoholics, malnutrition can reduce intestinal thiamine absorption by 70%, decreasing[emedicine.medscape.com]
Prisoner of War
  • Cerebral beriberi (Wernicke's encephalopathy); review of 52 cases in a Singapore prisoner-of-war hospital. Lancet 1947;1:11-7. 22. Donnino M. Gastrointestinal beriberi: A previously unrecognized syndrome. Ann Intern Med 2004;141:898-9. 23.[ruralneuropractice.com]
  • Cerebral beriberi (Wernicke's encephalopathy); review of 52 cases in a Singapore prisoner-of-war hospital. Lancet . 1947 Jan 4. 1(6436):11-7. [Medline] . Smithline HA, Donnino M, Greenblatt DJ.[emedicine.medscape.com]
Diplopia
  • We present a developmentally appropriate adolescent boy who presented with upper and lower extremity glove-and-stocking paresthesias, distal weakness, vertigo, high-pitched voice, inattention, ataxia, and binocular diplopia after a voluntary 59-kg weight[ncbi.nlm.nih.gov]
  • A 68-year-old woman under treatment with fedratinib (investigational JAK2 inhibitor) developed memory impairment, diplopia, and ataxia compatible with WE.[ncbi.nlm.nih.gov]
  • She noted diplopia and oscillopsia 2 months later, which led her to have full orthoptic and neuro-ophthalmic evaluations. After being treated with chewable vitamins with thiamine, she noted a tremendous improvement in her symptoms.[ncbi.nlm.nih.gov]
  • The second patient was a man who lost 10 kg after surgical gastrectomy; he developed diplopia, ophthalmoplegia, cerebellar ataxia, lower limb paresthesias, and amnesia.[ncbi.nlm.nih.gov]
  • In neurology clinic follow-up 1 month later, her headaches, diplopia, and nystagmus had improved but she continued to use a cane for ambulation.[pediatrics.aappublications.org]
Strabismus
  • We recommend inquiring about any obesity surgery in one's history and including Wernicke encephalopathy in possible differential diagnoses in those patients who have a recent onset of strabismus or nystagmus, altered mental status, and/or gait ataxia.[ncbi.nlm.nih.gov]
  • Simultaneously, unsteady gait and strabismus occurred.[ncbi.nlm.nih.gov]
  • Common signs are confusion, apathy, diplopia, strabismus, nystagmus, weakness or paralysis of eye abduction, ptosis, vestibular dysfunction without hearing loss, and ataxia.[clinicaladvisor.com]
Pupillary Abnormality
  • Less frequently noted manifestations are: pupillary abnormalities such as sluggishly reactive pupils, ptosis, scotomata, and anisocoria.[emedicine.medscape.com]
  • abnormalities such as sluggishly reactive pupils, ptosis, scotomata, and anisocoria OTHER SYMPTOMS Vestibular dysfunction Hypotension.[rnpedia.com]
Ataxia
  • The second patient was a man who lost 10 kg after surgical gastrectomy; he developed diplopia, ophthalmoplegia, cerebellar ataxia, lower limb paresthesias, and amnesia.[ncbi.nlm.nih.gov]
  • Here we describe a teenage girl who develops vomiting after Roux-en-Y gastric bypass and presented with nystagmus, irritability, and ataxia.[ncbi.nlm.nih.gov]
  • Wernicke's classic triad (confusion, ocular abnormalities, and ataxia) manifested in only 46.9% (23 of 49) of the patients.[ncbi.nlm.nih.gov]
  • It is associated with a classic triad of symptoms consisting of ataxia, ocular motor cranial neuropathies, and changes in consciousness.[ncbi.nlm.nih.gov]
  • Wernicke encephalopathy is caused by thiamine deficiency in the central nervous system, and is defined by the triad of confusional symptoms, ocular alterations and ataxia. Some other factors may also predispose alcoholic patients to this deficiency.[ncbi.nlm.nih.gov]
Confusion
  • Two patients had confusion, ataxia, or ocular changes and low serum thiamine levels, which resolved with parenteral thiamine.[ncbi.nlm.nih.gov]
  • Wernicke's classic triad (confusion, ocular abnormalities, and ataxia) manifested in only 46.9% (23 of 49) of the patients.[ncbi.nlm.nih.gov]
  • In a chronic alcoholic patient with progressive confusion, which was consistent with the clinical diagnosis of Wernicke encephalopathy, T2-weighted, FLAIR and diffusion weighted (DWI) MR imaging depicted brain abnormalities located in both medial thalamic[ncbi.nlm.nih.gov]
  • A 52-year-old woman with alcohol abuse presented with recent worsening of vision, imbalance, and confusion. Examination revealed counting fingers acuity in both eyes with central scotomas, color vision loss, horizontal nystagmus, and gait ataxia.[ncbi.nlm.nih.gov]
  • After the administration of thiamine, the patient's confused mental state resolved within 3 d, and her dystaxia gradually improved over the next 5 d.[ncbi.nlm.nih.gov]
Nystagmus
  • An examination revealed spontaneous upbeat nystagmus ( Video 1 ), gaze-evoked nystagmus ( Video 2 ), and gait ataxia. She had no ophthalmoplegia.[nejm.org]
  • Here we describe a teenage girl who develops vomiting after Roux-en-Y gastric bypass and presented with nystagmus, irritability, and ataxia.[ncbi.nlm.nih.gov]
  • Examination revealed counting fingers acuity in both eyes with central scotomas, color vision loss, horizontal nystagmus, and gait ataxia. Thiamine was initiated as treatment for a presumptive diagnosis of Wernicke encephalopathy (WE).[ncbi.nlm.nih.gov]
  • We recommend inquiring about any obesity surgery in one's history and including Wernicke encephalopathy in possible differential diagnoses in those patients who have a recent onset of strabismus or nystagmus, altered mental status, and/or gait ataxia.[ncbi.nlm.nih.gov]
  • Examination disclosed slow saccades, nystagmus, and impaired abduction of both eyes as well as memory loss and ataxia.[ncbi.nlm.nih.gov]
Confabulation
  • Korsakoff’s syndrome Characterised by: Anterograde amnesia (inability to form new memories) Retrograde amnesia (inability to remember old memories) Confabulation (fabricating/false perceptions of memories) Hallucinations The damage to the memory is mostly[dundeemedstudentnotes.wordpress.com]
  • Chronic thiamine deficiency , especially in patients with alcohol use disorder , frequently evolves into Korsakoff syndrome , which is characterized by irreversible personality changes, anterograde and retrograde amnesia , and confabulation .[amboss.com]
  • Wernicke's encephalopathy Pathology Symptoms nystagmus, gaze palsies, and ophthalmoplegia, especially of the lateral rectus muscles), gait ataxia, confusion, confabulation, and short-term memory los Treatments intravenous or intramuscular injection of[house.wikia.com]
  • Confabulation - Patient fills in gaps of memory with data that can be recalled at that moment 1. 1 Wernicke’s Encephalopathy AMITESHWAR SINGH INTERN, KMC MANIPAL. 2. 2 Dr Carl Wernicke A Polish neurologist, who described this neuropsychiatric syndrome[slideshare.net]
  • […] originally described as characterised by the triad of: acute confusion ataxia ophthalmoplegia Wernicke encephalopathy can evolve into the chronic form of thiamine deficiency known as Korsakoff psychosis , characterised by: memory loss (global amnesia) confabulation[radiopaedia.org]
Amnesia
  • The second patient was a man who lost 10 kg after surgical gastrectomy; he developed diplopia, ophthalmoplegia, cerebellar ataxia, lower limb paresthesias, and amnesia.[ncbi.nlm.nih.gov]
  • A 24-year-old woman who had recently served a 4-month prison sentence and underwent a short period of dieting manifested slow response, weakness, language disorder and amnesia. Brain magnetic resonance imaging (MRI) revealed typical lesions of WE.[ncbi.nlm.nih.gov]
  • Korsakoff syndrome is a chronic form of amnesia resulting from thiamine deficiency. The syndrome can develop from unrecognized or undertreated Wernicke encephalopathy.[ncbi.nlm.nih.gov]
  • Korsakoff’s syndrome Characterised by: Anterograde amnesia (inability to form new memories) Retrograde amnesia (inability to remember old memories) Confabulation (fabricating/false perceptions of memories) Hallucinations The damage to the memory is mostly[dundeemedstudentnotes.wordpress.com]
  • Of patients surviving WE, an important percentage will manifest WKS, characterized by the following: retrograde amnesia (inability to recall information), anterograde amnesia (inability to assimilate new information), decreased spontaneity and initiative[emedicine.medscape.com]

Workup

The diagnosis of WE can be made only if clinical suspicion exists for this condition, which is based on findings obtained during history taking and a physical examination, the two most important parts of the workup. Information about preexisting conditions that could've caused WE may be provided either by the patient him/herself or from family members/friends if the patient presents with confusion and is unable to provide adequate answers, whereas the progression of symptoms is also of great importance. Moreover, gait disturbances, ophthalmoplegia or nystagmus, and an altered mental state are all confirmed during the physical examination, and the diagnosis should be suspected in all patients who present with two of the three mentioned symptoms accompanied by dietary insufficiency (known as the Caine criteria) [1] [2] [3] [4]. If clinical criteria are fulfilled, laboratory and imaging studies must be promptly employed to confirm WE. Although no specific markers in blood suggest WE, a thorough biochemical workup comprised of a complete blood count (CBC), liver and kidney function tests, serum electrolytes, arterial blood gas (ABG) analyses and evaluation of inflammatory parameters is vital. On the other hand, alterations in the T2 signal intensity in the mammillary bodies, periaqueductal areas, and the medial thalami are characteristic features of WE on magnetic resonance imaging (MRI), the recommended imaging study in this group of patients [1] [5] [7]. Signal intensities of the cerebellum, cerebral cortex, as well as cranial nerve, dentate, caudate and red nuclei are less common but reported signs of WE on MRI [5]. Thiamine levels are not routinely measured, as its exact concentration in the CNS cannot be determined based on serum values, indicating that the diagnosis rests on clinical and MRI findings.

Slowing
  • A 24-year-old woman who had recently served a 4-month prison sentence and underwent a short period of dieting manifested slow response, weakness, language disorder and amnesia. Brain magnetic resonance imaging (MRI) revealed typical lesions of WE.[ncbi.nlm.nih.gov]
  • After high-dose intravenous thiamine repletion, she experienced slow resolution of her symptoms.[ncbi.nlm.nih.gov]
  • Examination disclosed slow saccades, nystagmus, and impaired abduction of both eyes as well as memory loss and ataxia.[ncbi.nlm.nih.gov]
  • Electroencephalography showed generalised slowing without focal features.[mja.com.au]
  • He became unresponsive and MRI showed new areas of T2 FLAIR hyperintensity in the posterior pons, medial thalamus, and bilateral mamillary bodies, while EEG showed diffuse generalized slowing, consistent with brain injury.[path.upmc.edu]
Generalized Slowing
  • He became unresponsive and MRI showed new areas of T2 FLAIR hyperintensity in the posterior pons, medial thalamus, and bilateral mamillary bodies, while EEG showed diffuse generalized slowing, consistent with brain injury.[path.upmc.edu]
Glucose Increased
  • Because glucose increases thiamine demand and will worsen encephalopathy , IV glucose infusions must be administered AFTER thiamine ![amboss.com]

Treatment

  • Here we review representative cases of both conditions to highlight specific and relevant neurologic features that prompted effective diagnosis and treatment.[ncbi.nlm.nih.gov]
  • Although thiamine is the cornerstone of treatment of Wernicke encephalopathy, there are no universally accepted guidelines with regard to its optimal dose, mode of administration, frequency of administration or duration of treatment.[ncbi.nlm.nih.gov]
  • Precocious diagnosis and treatment are essential to prevent irreversible brain injury.[ncbi.nlm.nih.gov]
  • Treatment should be instituted immediately when the diagnosis is suspected clinically without waiting for laboratory confirmation.[ncbi.nlm.nih.gov]
  • Early diagnosis and treatment are critical to avoid persistent neurologic impairment.[ncbi.nlm.nih.gov]

Prognosis

  • Early thiamine treatment in symptomatic patients may improve prognosis.[ncbi.nlm.nih.gov]
  • The prognosis of the disease largely depends on the time from diagnosis to thiamine supplementation.[ncbi.nlm.nih.gov]
  • Early recognition and diagnosis of Wernicke encephalopathy is pivotal for the prognosis of this medical emergency, especially in patients with liver failure which predisposes individuals to develop hepatic encephalopathy.[ncbi.nlm.nih.gov]
  • To analyze the differences in characteristics and prognosis between alcoholic and nonalcoholic patients with Wernicke encephalopathy (WE).[ncbi.nlm.nih.gov]
  • Even with psychiatric and psychological interventions, the prognosis for patients with Korsakoff syndrome remains poor. Etiology Wernicke encephalopathy and Korsakoff syndrome are caused by a severe deficiency of thiamine ( vitamin B1 ) .[amboss.com]

Etiology

  • Etiology Wernicke encephalopathy and Korsakoff syndrome are caused by a severe deficiency of thiamine ( vitamin B1 ) .[amboss.com]
  • Classic signs of inebriation such as slurred speech, poor coordination and an alcohol-like odor can be caused by other etiologies, while many true alcoholics show no signs at all of their disease.[ems1.com]
  • Etiology Causes Thiamine deficiency is characteristically associated with chronic alcoholism, because it affects thiamine uptake and utilization. [ 2 ] In long-term alcoholics, malnutrition can reduce intestinal thiamine absorption by 70%, decreasing[emedicine.medscape.com]
  • […] portions of the GI tract, recurrent vomiting or chronic diarrhea, cancer chemotherapy and systemic diseases (renal failure with dialysis, AIDS) Sites Mammillary bodies, medial thalamus / wall of third ventricle, periaqueductal gray matter, midbrain tectum Etiology[pathologyoutlines.com]

Epidemiology

  • In this review, we provide an update on the factors and clinical settings that predispose to Wernicke's encephalopathy, and discuss the most recent insights into epidemiology, pathophysiology, genetics, diagnosis, and treatment.[ncbi.nlm.nih.gov]
  • Epidemiology  The 6 incidence can be as high as 12.5% in a population of alcoholics.  The prevalence approximately 2%.  The male-to-female ratio is 1.7 : 1  Average age at onset is 50 years. 7.[slideshare.net]
  • ., malabsorption) Epidemiology alcoholics are mostly affected Presentation Symptoms Wernicke's encephalopathy confusion opthalmoplegia ataxia Korsakoff's psychosis memory loss confabulation hallucination personality change Evaluation Clinical diagnosis[medbullets.com]
  • […] syndrome resulting from thiamine (vitamin B1) deficiency Korsakoff syndrome: a disproportionate impairment in memory relative to other features of cognition secondary to thiamine deficiency that usually follows or accompanies Wernicke encephalopathy Epidemiology[pathologyoutlines.com]
  • Nutritionally deficient patients receiving glucose should also receive thiamine, but urgent administration of glucose should not be delayed pending thiamine administration. [ 5 ] Epidemiology Occurrence in the United States Autopsy studies indicate that[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Increased lactate and typical MR imaging findings are discussed in the context of the known pathophysiology of Wernicke encephalopathy.[ncbi.nlm.nih.gov]
  • As clinical symptoms of this spectrum of diseases have nonspecific neurologic alterations, radiologists should have current radiologic information and understand the imaging findings pertaining to the pathophysiology to support diagnosis.[ncbi.nlm.nih.gov]
  • We have therefore investigated levels of the astrocytic glutamate transporters EAAT1 and EAAT2 in order to evaluate their role in the pathophysiology of this disorder.[ncbi.nlm.nih.gov]
  • In this review, we provide an update on the factors and clinical settings that predispose to Wernicke's encephalopathy, and discuss the most recent insights into epidemiology, pathophysiology, genetics, diagnosis, and treatment.[ncbi.nlm.nih.gov]
  • Direct Download Pathophysiology secondary to thiamine deficiency (vitamin B1). Thiamine is a cofactor for pyruvate dehydrogenase.[blog.ercast.org]

Prevention

  • Although Wernicke encephalopathy (WE) is a preventable and treatable disease it still often remains undiagnosed during life. To create practical guidelines for diagnosis, management and prevention of the disease.[ncbi.nlm.nih.gov]
  • Early intervention may correct the symptoms and prevent irreversible brain damage, and the quality of life for the patient may improve.[ncbi.nlm.nih.gov]
  • Early intervention may correct the symptoms and prevent irreversible brain damage and the quality of life for the patient may improve.[ncbi.nlm.nih.gov]
  • An early diagnosis is imperative for a prompt therapy that might prevent or minimize the irreversible brain damage related to this condition.[ncbi.nlm.nih.gov]
  • Precocious diagnosis and treatment are essential to prevent irreversible brain injury.[ncbi.nlm.nih.gov]

References

Article

  1. Zhao P, Zhao Y, Wei Z, Chen J, Yan L. Wernicke encephalopathy in a patient with liver failure: Clinical case report. Gara. N, ed. Medicine (Baltimore). 2016;95(27):e3651.
  2. Day GS, del Campo CM. Wernicke encephalopathy: a medical emergency. CMAJ. 2014;186(8):E295.
  3. Chamorro AJ, Marcos-Martin M, Martin-Polo J, Garcia-Diez LC, Luna G. Wernicke encephalopathy in alcoholics with diabetic ketoacidosis. Intern Med. 2009;48(13):1187-1189.
  4. Udyavara Kudru C, Kaniyoor Nagiri S, Rao S. Wernicke’s encephalopathy in a patient with gastric carcinoma: a diagnosis not to miss. BMJ Case Rep. 2014;2014:bcr2013203511.
  5. Ha ND, Weon YC, Jang JC, Kang BS, Choi SH. Spectrum of MR imaging findings in Wernicke encephalopathy: are atypical areas of involvement only present in nonalcoholic patients? AJNR Am J Neuroradiol. 2012;33(7):1398-1402.
  6. Delavar Kasmaei H, Baratloo A, Soleymani M, Nasiri Z. Imaging-Based Diagnosis of Wernicke Encephalopathy: A Case Report. Trauma Mon. 2014;19(4):e17403.
  7. Zuccoli G, Gallucci M, Capellades J, et al. Wernicke encephalopathy: MR findings at clinical presentation in twenty-six alcoholic and nonalcoholic patients. AJNR Am J Neuroradiol. 2007;28(7):1328-1331.

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Last updated: 2017-08-09 13:41