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Wernicke Encephalopathy and Korsakoff Syndrome

Wernicke Encephalopathy and Korsakoff Syndrome

Wernicke encephalopathy is an acute, reversible condition that is caused by severe thiamine deficiency. This condition is most commonly seen in alcohol abusers. The classic triad of symptoms is encephalopathy, oculomotor dysfunction, and gait ataxia, although all 3 features are only present in one-third of patients. Korsakoff syndrome is a severe and late neuropsychiatric manifestation of Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome. Korsakoff syndrome presents with personality changes, anterograde and retrograde amnesia, and confabulation. Some of these changes are irreversible. Both Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome and Korsakoff syndrome may be diagnosed clinically. Treatment includes absolute abstinence from alcohol and thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies supplementation.

Last updated: 24 Jan, 2022

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Contents

Overview

Definition

Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome and Korsakoff syndrome are neurologic conditions that arise because of thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies (vitamin B1) deficiency. Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome is an acute, reversible condition. Korsakoff syndrome is an irreversible syndrome that is caused by more severe or prolonged thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies deficiency and occurs after incomplete recovery from Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome.

Epidemiology

  • Incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency of Wernicke lesions: 0.8%–2.8% of autopsies in general population 
  • Korsakoff syndrome impacts up to 12.5% of chronic alcohol abusers.
  • Men are more commonly affected than women.
  • Average age at onset of Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome: 50 years

Etiology

Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome and Korsakoff syndrome are caused by a severe deficiency of thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies. This deficiency is most commonly due to:

  • Chronic alcohol use:
    • Inadequate intake
    • Poor absorption Absorption Absorption involves the uptake of nutrient molecules and their transfer from the lumen of the GI tract across the enterocytes and into the interstitial space, where they can be taken up in the venous or lymphatic circulation. Digestion and Absorption
    • Decreased hepatic storage
    • Improper utilization 
  • Inadequate intake of thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies:
    • Anorexia Anorexia The lack or loss of appetite accompanied by an aversion to food and the inability to eat. It is the defining characteristic of the disorder anorexia nervosa. Anorexia Nervosa
    • Starvation
    • Thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies-deficient diets 
  • Increased loss of thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies:
    • Diarrhea Diarrhea Diarrhea is defined as ≥ 3 watery or loose stools in a 24-hour period. There are a multitude of etiologies, which can be classified based on the underlying mechanism of disease. The duration of symptoms (acute or chronic) and characteristics of the stools (e.g., watery, bloody, steatorrheic, mucoid) can help guide further diagnostic evaluation. Diarrhea
    • Hyperemesis
    • Dialysis Dialysis Renal replacement therapy refers to dialysis and/or kidney transplantation. Dialysis is a procedure by which toxins and excess water are removed from the circulation. Hemodialysis and peritoneal dialysis (PD) are the two types of dialysis, and their primary difference is the location of the filtration process (external to the body in hemodialysis versus inside the body for PD). Peritoneal Dialysis and Hemodialysis 
  • Malnutrition Malnutrition Malnutrition is a clinical state caused by an imbalance or deficiency of calories and/or micronutrients and macronutrients. The 2 main manifestations of acute severe malnutrition are marasmus (total caloric insufficiency) and kwashiorkor (protein malnutrition with characteristic edema). Malnutrition in children in resource-limited countries can also cause thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies deficiency.
Structure of thiamine

Molecular structure of thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies:
Levels are deficient in Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome and Korsakoff syndrome

Image: “Structure of thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies” by NEUROtiker. License: Public Domain

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Wernicke Encephalopathy and Korsakoff’s Syndrome

Pathophysiology

  • Thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies is an essential coenzyme used for numerous bodily functions:
    • Glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance metabolism
    • Neurotransmitter synthesis Synthesis Polymerase Chain Reaction (PCR)
    • Lipid metabolism Lipid Metabolism Lipid metabolism is the processing of lipids for energy use, energy storage, and structural component production. Lipid metabolism uses fats from dietary sources or from fat stores in the body. A complex series of processes involving digestion, absorption, and transport are required for the proper metabolism of lipids. Lipid Metabolism
  • Thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies is converted into its more active form, thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies diphosphate (TDP).
  • Thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies deficiency is most often caused by alcoholism and malnutrition Malnutrition Malnutrition is a clinical state caused by an imbalance or deficiency of calories and/or micronutrients and macronutrients. The 2 main manifestations of acute severe malnutrition are marasmus (total caloric insufficiency) and kwashiorkor (protein malnutrition with characteristic edema). Malnutrition in children in resource-limited countries.
  • Thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies deficiency leads to: 
    • Oxidative damage
    • Cellular apoptosis Apoptosis A regulated cell death mechanism characterized by distinctive morphologic changes in the nucleus and cytoplasm, including the endonucleolytic cleavage of genomic DNA, at regularly spaced, internucleosomal sites, I.e., DNA fragmentation. It is genetically-programmed and serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth. Ischemic Cell Damage 
  • Deficiency of ATP and increase in harmful free radicals Free radicals Highly reactive molecules with an unsatisfied electron valence pair. Free radicals are produced in both normal and pathological processes. They are proven or suspected agents of tissue damage in a wide variety of circumstances including radiation, damage from environment chemicals, and aging. Natural and pharmacological prevention of free radical damage is being actively investigated. Ischemic Cell Damage causes damage to myelin sheaths and decreases neurotransmitter production, resulting in impaired axonal conduction. 
  • Wernicke lesions are most often found in the periventricular region, diencephalon Diencephalon The paired caudal parts of the prosencephalon from which the thalamus; hypothalamus; epithalamus; and subthalamus are derived. Development of the Nervous System and Face ( thalamus Thalamus The thalamus is a large, ovoid structure in the dorsal part of the diencephalon that is located between the cerebral cortex and midbrain. It consists of several interconnected nuclei of grey matter separated by the laminae of white matter. The thalamus is the main conductor of information that passes between the cerebral cortex and the periphery, spinal cord, or brain stem. Thalamus: Anatomy, hypothalamus Hypothalamus The hypothalamus is a collection of various nuclei within the diencephalon in the center of the brain. The hypothalamus plays a vital role in endocrine regulation as the primary regulator of the pituitary gland, and it is the major point of integration between the central nervous and endocrine systems. Hypothalamus, epithalamus, subthalamus), and midbrain Midbrain The middle of the three primitive cerebral vesicles of the embryonic brain. Without further subdivision, midbrain develops into a short, constricted portion connecting the pons and the diencephalon. Midbrain contains two major parts, the dorsal tectum mesencephali and the ventral tegmentum mesencephali, housing components of auditory, visual, and other sensorimotor systems. Brain Stem: Anatomy.
  • In Korsakoff syndrome, long-term damage to components of the limbic system Limbic system The limbic system is a neuronal network that mediates emotion and motivation, while also playing a role in learning and memory. The extended neural network is vital to numerous basic psychological functions and plays an invaluable role in processing and responding to environmental stimuli. Limbic System: Anatomy leads to memory Memory Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. Psychiatric Assessment loss and apathy. 
  • Petechial lesions and small-vessel hemorrhage are seen in the mesencephalon Mesencephalon Development of the Nervous System and Face, mammillary bodies Mammillary bodies A pair of nuclei and associated gray matter in the interpeduncular space rostral to the posterior perforated substance in the posterior hypothalamus. Limbic System: Anatomy, ventricle walls, and the dorsomedial nucleus Nucleus Within a eukaryotic cell, a membrane-limited body which contains chromosomes and one or more nucleoli (cell nucleolus). The nuclear membrane consists of a double unit-type membrane which is perforated by a number of pores; the outermost membrane is continuous with the endoplasmic reticulum. A cell may contain more than one nucleus. The Cell: Organelles of the thalamus Thalamus The thalamus is a large, ovoid structure in the dorsal part of the diencephalon that is located between the cerebral cortex and midbrain. It consists of several interconnected nuclei of grey matter separated by the laminae of white matter. The thalamus is the main conductor of information that passes between the cerebral cortex and the periphery, spinal cord, or brain stem. Thalamus: Anatomy.
  • Damage to the medial dorsal nucleus Nucleus Within a eukaryotic cell, a membrane-limited body which contains chromosomes and one or more nucleoli (cell nucleolus). The nuclear membrane consists of a double unit-type membrane which is perforated by a number of pores; the outermost membrane is continuous with the endoplasmic reticulum. A cell may contain more than one nucleus. The Cell: Organelles leads to memory Memory Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. Psychiatric Assessment deficit. 
  • Atrophy Atrophy Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes. Cellular Adaptation of the limbic system Limbic system The limbic system is a neuronal network that mediates emotion and motivation, while also playing a role in learning and memory. The extended neural network is vital to numerous basic psychological functions and plays an invaluable role in processing and responding to environmental stimuli. Limbic System: Anatomy, including anterior thalamic nuclei Anterior thalamic nuclei Three nuclei located beneath the dorsal surface of the most rostral part of the thalamus. The group includes the anterodorsal nucleus, anteromedial nucleus, and anteroventral nucleus. All receive connections from the mamillary body and brain fornix, and project fibers to the cingulate body. Thalamus: Anatomy and mammillary bodies Mammillary bodies A pair of nuclei and associated gray matter in the interpeduncular space rostral to the posterior perforated substance in the posterior hypothalamus. Limbic System: Anatomy is seen after prolonged periods of deficiency.
  • The location of the lesions affects the symptoms of disease:
    • Hypothalamus Hypothalamus The hypothalamus is a collection of various nuclei within the diencephalon in the center of the brain. The hypothalamus plays a vital role in endocrine regulation as the primary regulator of the pituitary gland, and it is the major point of integration between the central nervous and endocrine systems. Hypothalamus lesions lead to autonomic dysfunction Autonomic Dysfunction Anterior Cord Syndrome.
    • Medulla lesions lead to ataxia Ataxia Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or peripheral nerve diseases. Motor ataxia may be associated with cerebellar diseases; cerebral cortex diseases; thalamic diseases; basal ganglia diseases; injury to the red nucleus; and other conditions. Ataxia-telangiectasia.
    • Mammillary body lesions cause amnestic syndrome.
Diencephalon

The diencephalon Diencephalon The paired caudal parts of the prosencephalon from which the thalamus; hypothalamus; epithalamus; and subthalamus are derived. Development of the Nervous System and Face is strongly impacted in Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome.

Image: “ Diencephalon Diencephalon The paired caudal parts of the prosencephalon from which the thalamus; hypothalamus; epithalamus; and subthalamus are derived. Development of the Nervous System and Face” by Arcadian. License: Public Domain, edited by Lecturio.

Clinical Presentation

History

  • Suspected in individuals with a history of: 
    • Chronic excessive alcohol consumption
    • Malnutrition Malnutrition Malnutrition is a clinical state caused by an imbalance or deficiency of calories and/or micronutrients and macronutrients. The 2 main manifestations of acute severe malnutrition are marasmus (total caloric insufficiency) and kwashiorkor (protein malnutrition with characteristic edema). Malnutrition in children in resource-limited countries/ malabsorption Malabsorption General term for a group of malnutrition syndromes caused by failure of normal intestinal absorption of nutrients. Malabsorption and Maldigestion:
      • History of bowel resection
      • History of bariatric surgery Bariatric surgery Bariatric surgery refers to a group of invasive procedures used to surgically reduce the size of the stomach to produce early satiety, decrease food intake (restrictive type) and/or alter digestion, and artificially induce malabsorption of nutrients (malabsorptive type). The ultimate goal of bariatric surgery is drastic weight loss. Bariatric Surgery
      • History of inflammatory bowel disease
  • History often provided by caregivers, family, first responders:
    • Amnesia, both anterograde and retrograde
    • Disorientation Disorientation St. Louis Encephalitis Virus 
    • Confabulation ( memory Memory Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. Psychiatric Assessment fabrication to make up for memory Memory Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. Psychiatric Assessment lapses)
    • Personality changes, including apathy, indifference, and executive function 
    • Hallucinations Hallucinations Subjectively experienced sensations in the absence of an appropriate stimulus, but which are regarded by the individual as real. They may be of organic origin or associated with mental disorders. Schizophrenia
  • In alcohol abusers, may present with acute withdrawal symptoms and/or delirium Delirium Delirium is a medical condition characterized by acute disturbances in attention and awareness. Symptoms may fluctuate during the course of a day and involve memory deficits and disorientation. Delirium tremens

Examination

  • Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome presents with a classic triad of examination findings: 
    • Encephalopathy Encephalopathy Hyper-IgM Syndrome: 
    • Oculomotor dysfunction: 
      • Nystagmus Nystagmus Involuntary movements of the eye that are divided into two types, jerk and pendular. Jerk nystagmus has a slow phase in one direction followed by a corrective fast phase in the opposite direction, and is usually caused by central or peripheral vestibular dysfunction. Pendular nystagmus features oscillations that are of equal velocity in both directions and this condition is often associated with visual loss early in life. Albinism
      • Lateral rectus Lateral rectus Orbit and Extraocular Muscles: Anatomy palsy Palsy paralysis of an area of the body, thus incapable of voluntary movement Cranial Nerve Palsies
      • Diplopia Diplopia A visual symptom in which a single object is perceived by the visual cortex as two objects rather than one. Disorders associated with this condition include refractive errors; strabismus; oculomotor nerve diseases; trochlear nerve diseases; abducens nerve diseases; and diseases of the brain stem and occipital lobe. Myasthenia Gravis
    • Gait ataxia Gait ataxia Impairment of the ability to coordinate the movements required for normal ambulation (walking) which may result from impairments of motor function or sensory feedback. This condition may be associated with brain diseases (including cerebellar diseases and basal ganglia diseases); spinal cord diseases; or peripheral nervous system diseases. Friedreich’s Ataxia: 
  • Additional examination findings in Korsakoff syndrome:
    • Confabulation
    • Amnesia
  • Other signs: 
    • Vestibular dysfunction 
    • Fatigability
    • Apathy
    • Drowsiness
    • Impaired vision Vision Ophthalmic Exam and hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss
    • Peripheral neuropathy Peripheral Neuropathy Neurofibromatosis Type 2 involving lower extremities
    • Cardiovascular signs and symptoms:
      • Tachycardia Tachycardia Abnormally rapid heartbeat, usually with a heart rate above 100 beats per minute for adults. Tachycardia accompanied by disturbance in the cardiac depolarization (cardiac arrhythmia) is called tachyarrhythmia. Sepsis in Children
      • Exertional dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea
      • Elevated cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) output
      • Hypertension Hypertension Hypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter. Hypertension 
    • Hypothermia Hypothermia Hypothermia can be defined as a drop in the core body temperature below 35°C (95°F) and is classified into mild, moderate, severe, and profound forms based on the degree of temperature decrease. Hypothermia
    • Sleep Sleep A readily reversible suspension of sensorimotor interaction with the environment, usually associated with recumbency and immobility. Physiology of Sleep apnea
    • Tremor Tremor Cyclical movement of a body part that can represent either a physiologic process or a manifestation of disease. Intention or action tremor, a common manifestation of cerebellar diseases, is aggravated by movement. In contrast, resting tremor is maximal when there is no attempt at voluntary movement, and occurs as a relatively frequent manifestation of parkinson disease. Myotonic Dystrophies
    • Seizure
    • Delirium Delirium Delirium is a medical condition characterized by acute disturbances in attention and awareness. Symptoms may fluctuate during the course of a day and involve memory deficits and disorientation. Delirium tremens

Diagnosis

Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome and Korsakoff syndrome are primarily clinical diagnoses.

  • Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome and Korsakoff syndrome should be suspected in any individual with chronic alcohol abuse or any form of malnutrition Malnutrition Malnutrition is a clinical state caused by an imbalance or deficiency of calories and/or micronutrients and macronutrients. The 2 main manifestations of acute severe malnutrition are marasmus (total caloric insufficiency) and kwashiorkor (protein malnutrition with characteristic edema). Malnutrition in children in resource-limited countries.
  • Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome may be diagnosed with the presence of 2 of 3 prototypical symptoms:
    • Ophthalmoplegia Ophthalmoplegia Paralysis of one or more of the ocular muscles due to disorders of the eye muscles, neuromuscular junction, supporting soft tissue, tendons, or innervation to the muscles. Orbital and Preseptal Cellulitis
    • Ataxia Ataxia Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or peripheral nerve diseases. Motor ataxia may be associated with cerebellar diseases; cerebral cortex diseases; thalamic diseases; basal ganglia diseases; injury to the red nucleus; and other conditions. Ataxia-telangiectasia
    • Confusion
  • Individuals with only 1 of these symptoms may be treated empirically. 
  • Imaging and laboratory measurements are not sufficient for diagnosis.
  • Laboratory tests: 
    • Reduced serum thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies levels
    • Reduced erythrocyte transketolase Transketolase An enzyme of the transferase class that catalyzes the conversion of sedoheptulose 7-phosphate and d-glyceraldehyde 3-phosphate to d-ribose 5-phosphate and d-xylulose 5-phosphate in the pentose phosphate pathway. Pentose Phosphate Pathway activity
    • Increased serum lactate and pyruvate Pyruvate Derivatives of pyruvic acid, including its salts and esters. Glycolysis 
    • Consider serum alcohol level to evaluate for signs of intoxication.
    • Consider metabolic workup for alcoholic Alcoholic Persons who have a history of physical or psychological dependence on ethanol. Mallory-Weiss Syndrome (Mallory-Weiss Tear) ketoacidosis Ketoacidosis A life-threatening complication of diabetes mellitus, primarily of type 1 diabetes mellitus with severe insulin deficiency and extreme hyperglycemia. It is characterized by ketosis; dehydration; and depressed consciousness leading to coma. Metabolic Acidosis.
    • Consider CBC, liver function tests Liver function tests Liver function tests, also known as hepatic function panels, are one of the most commonly performed screening blood tests. Such tests are also used to detect, evaluate, and monitor acute and chronic liver diseases. Liver Function Tests, and measurement of serum chemistry levels to evaluate for stigmata of chronic liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver: Anatomy disease.
    • Consider measurement of ammonia Ammonia A colorless alkaline gas. It is formed in the body during decomposition of organic materials during a large number of metabolically important reactions. Note that the aqueous form of ammonia is referred to as ammonium hydroxide. Acid-Base Balance level to rule out hepatic encephalopathy Encephalopathy Hyper-IgM Syndrome.
  • Additional testing: if diagnosis of delirium Delirium Delirium is a medical condition characterized by acute disturbances in attention and awareness. Symptoms may fluctuate during the course of a day and involve memory deficits and disorientation. Delirium or confusion is uncertain
    • Urine Urine Liquid by-product of excretion produced in the kidneys, temporarily stored in the bladder until discharge through the urethra. Bowen Disease and Erythroplasia of Queyrat/blood toxicity Toxicity Dosage Calculation screen
    • Drug levels of selected prescribed medications
    • Thyroid function tests Thyroid Function Tests Blood tests used to evaluate the functioning of the thyroid gland. Ion Channel Myopathy
    • Vitamin B12 Vitamin B12 A cobalt-containing coordination compound produced by intestinal microorganisms and found also in soil and water. Higher plants do not concentrate vitamin B 12 from the soil and so are a poor source of the substance as compared with animal tissues. Intrinsic factor is important for the assimilation of vitamin B 12. Folate and Vitamin B12 level
    • Urinalysis Urinalysis Examination of urine by chemical, physical, or microscopic means. Routine urinalysis usually includes performing chemical screening tests, determining specific gravity, observing any unusual color or odor, screening for bacteriuria, and examining the sediment microscopically. Urinary Tract Infections (UTIs) in Children/ urine Urine Liquid by-product of excretion produced in the kidneys, temporarily stored in the bladder until discharge through the urethra. Bowen Disease and Erythroplasia of Queyrat culture
    • Blood gas testing
    • Chest X-ray X-ray Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source. Pulmonary Function Tests
  • Neuroimaging Neuroimaging Non-invasive methods of visualizing the central nervous system, especially the brain, by various imaging modalities. Febrile Infant may show:
    • Cytotoxic Cytotoxic Parvovirus B19 edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema (bilateral and symmetric)
    • Contrast enhancement of the thalamus Thalamus The thalamus is a large, ovoid structure in the dorsal part of the diencephalon that is located between the cerebral cortex and midbrain. It consists of several interconnected nuclei of grey matter separated by the laminae of white matter. The thalamus is the main conductor of information that passes between the cerebral cortex and the periphery, spinal cord, or brain stem. Thalamus: Anatomy and mammillary bodies Mammillary bodies A pair of nuclei and associated gray matter in the interpeduncular space rostral to the posterior perforated substance in the posterior hypothalamus. Limbic System: Anatomy 
  • MRI findings are highly specific; however, CT has little value.
  • Restricted diffusion in the mesial dorsal thalami

    Korsakoff syndrome:
    Restricted diffusion Diffusion The tendency of a gas or solute to pass from a point of higher pressure or concentration to a point of lower pressure or concentration and to distribute itself throughout the available space. Diffusion, especially facilitated diffusion, is a major mechanism of biological transport. Peritoneal Dialysis and Hemodialysis in the thalami is a classic finding.

    Image: “MRI DWI DWI Imaging of the Head and Brain sequence” by Jto410. License: CC BY-SA 3.0
  • Wernicke’s encephalopathy

    Wernicke’s encephalopathy Encephalopathy Hyper-IgM Syndrome:
    MRI of head demonstrating periventricular hyperintensities at the medial thalami bilaterally, consistent with Wernicke’s encephalopathy Encephalopathy Hyper-IgM Syndrome. Orange arrows identifying areas of abnormality.

    Image: “Bilateral Blindness Blindness The inability to see or the loss or absence of perception of visual stimuli. This condition may be the result of eye diseases; optic nerve diseases; optic chiasm diseases; or brain diseases affecting the visual pathways or occipital lobe. Retinopathy of Prematurity Following Chemoradiation for Locally Advanced Oropharyngeal Carcinoma” by Zeng KL, Kuruvilla S, Sanatani M, Louie AV. License: CC BY 3.0

Management

In addition to treatment of the presenting encephalopathy Encephalopathy Hyper-IgM Syndrome, the clinician Clinician A physician, nurse practitioner, physician assistant, or another health professional who is directly involved in patient care and has a professional relationship with patients. Clinician–Patient Relationship should also have a high clinical suspicion for acute alcohol intoxication Alcohol intoxication An acute brain syndrome which results from the excessive ingestion of ethanol or alcoholic beverages. Alcohol Use Disorder, hepatic encephalopathy Encephalopathy Hyper-IgM Syndrome, alcohol withdrawal Alcohol withdrawal With chronic alcohol use, body increases activity of excitatory receptors (e.g., glutamate) while decreasing activation of inhibitory receptors (e.g., GABA). Cessation of alcohol use leads to unchecked glutamate activation, presenting as withdrawal. Alcohol Use Disorder syndrome, and alcoholic Alcoholic Persons who have a history of physical or psychological dependence on ethanol. Mallory-Weiss Syndrome (Mallory-Weiss Tear) ketoacidosis Ketoacidosis A life-threatening complication of diabetes mellitus, primarily of type 1 diabetes mellitus with severe insulin deficiency and extreme hyperglycemia. It is characterized by ketosis; dehydration; and depressed consciousness leading to coma. Metabolic Acidosis.

Wernicke encephalopathy Encephalopathy Hyper-IgM Syndrome

Treatment should be prompt because memory Memory Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. Psychiatric Assessment deficits may be irreversible.

  • Thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies supplementation as soon as possible:
    • Route: IV or IM
    • Duration: Continue treatment until symptoms resolve.
  • Laboratory studies evaluating thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies level and other vitamin/mineral levels (e.g., magnesium Magnesium A metallic element that has the atomic symbol mg, atomic number 12, and atomic weight 24. 31. It is important for the activity of many enzymes, especially those involved in oxidative phosphorylation. Electrolytes)
  • Hypoglycemic individuals should be given both glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance and thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies because glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance will deplete the remaining store of thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies.
  • Long-term vitamin replacement therapy can be given with oral vitamin supplements.
  • Educate about the importance of lifestyle changes (e.g., diet, abstinence from alcohol).
  • Referral for community social support:
    • Psychiatrist/addictionologist
    • Substance abuse counselor
    • Provide a list of local Alcoholics Anonymous Alcoholics Anonymous An organization of self-proclaimed alcoholics who meet frequently to reinforce their practice of abstinence. Alcoholic Liver Disease meetings/contact info.

Korsakoff syndrome

Korsakoff syndrome is serious and may require a high level of care for life, as memory Memory Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. Psychiatric Assessment deficits are often permanent.

  • Thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies (IV or IM)
  • Psychiatric evaluation
  • Rehabilitation services
  • Acetylcholinesterase inhibitors may be considered to improve cognitive functioning. 
  • Absolute abstinence from alcohol
  • Referral for community social support:
    • Psychiatrist/addictionologist
    • Substance abuse counselor
    • Provide a list of local Alcoholics Anonymous Alcoholics Anonymous An organization of self-proclaimed alcoholics who meet frequently to reinforce their practice of abstinence. Alcoholic Liver Disease meetings/contact info.

Differential Diagnosis

  • Cerebellar stroke: cerebrovascular event leading to impaired perfusion of O2 to the posterior fossa of the brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification. Cerebellar strokes account for 1%–4% of all strokes and can be thromboembolic or hemorrhagic in nature. Symptoms include headache Headache The symptom of pain in the cranial region. It may be an isolated benign occurrence or manifestation of a wide variety of headache disorders. Brain Abscess, vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia, vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo, unilateral hearing loss Unilateral hearing loss Partial or complete hearing loss in one ear. Hearing Loss, and contralateral sensory Sensory Neurons which conduct nerve impulses to the central nervous system. Nervous System: Histology loss ( pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways and temperature). Diagnosis is clinical and confirmed by neuroimaging Neuroimaging Non-invasive methods of visualizing the central nervous system, especially the brain, by various imaging modalities. Febrile Infant. Acute thrombolytic therapy can be used in selected acute cases. Long-term management consists of risk factor modification of further ischemic events. 
  • Delirium Delirium Delirium is a medical condition characterized by acute disturbances in attention and awareness. Symptoms may fluctuate during the course of a day and involve memory deficits and disorientation. Delirium tremens: severe form of ethanol Ethanol A clear, colorless liquid rapidly absorbed from the gastrointestinal tract and distributed throughout the body. It has bactericidal activity and is used often as a topical disinfectant. It is widely used as a solvent and preservative in pharmaceutical preparations as well as serving as the primary ingredient in alcoholic beverages. Ethanol Metabolism withdrawal characterized by tremor Tremor Cyclical movement of a body part that can represent either a physiologic process or a manifestation of disease. Intention or action tremor, a common manifestation of cerebellar diseases, is aggravated by movement. In contrast, resting tremor is maximal when there is no attempt at voluntary movement, and occurs as a relatively frequent manifestation of parkinson disease. Myotonic Dystrophies, anxiety Anxiety Feelings or emotions of dread, apprehension, and impending disaster but not disabling as with anxiety disorders. Generalized Anxiety Disorder, nausea Nausea An unpleasant sensation in the stomach usually accompanied by the urge to vomit. Common causes are early pregnancy, sea and motion sickness, emotional stress, intense pain, food poisoning, and various enteroviruses. Antiemetics, vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia, insomnia Insomnia Insomnia is a sleep disorder characterized by difficulty in the initiation, maintenance, and consolidation of sleep, leading to impairment of function. Patients may exhibit symptoms such as difficulty falling asleep, disrupted sleep, trouble going back to sleep, early awakenings, and feeling tired upon waking. Insomnia, auditory hallucinations Hallucinations Subjectively experienced sensations in the absence of an appropriate stimulus, but which are regarded by the individual as real. They may be of organic origin or associated with mental disorders. Schizophrenia, diaphoresis, and severe hypertension Hypertension Hypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter. Hypertension. Diagnosis is clinical but supported by laboratory evaluation and neuroimaging Neuroimaging Non-invasive methods of visualizing the central nervous system, especially the brain, by various imaging modalities. Febrile Infant. Treatment includes IV fluids IV fluids Intravenous fluids are one of the most common interventions administered in medicine to approximate physiologic bodily fluids. Intravenous fluids are divided into 2 categories: crystalloid and colloid solutions. Intravenous fluids have a wide variety of indications, including intravascular volume expansion, electrolyte manipulation, and maintenance fluids. Intravenous Fluids for rehydration Rehydration Dengue Virus, thiamine Thiamine Also known as thiamine or thiamin, it is a vitamin C12H17N4OSCl of the vitamin B complex that is essential to normal metabolism and nerve function and is widespread in plants and animals Water-soluble Vitamins and their Deficiencies and magnesium Magnesium A metallic element that has the atomic symbol mg, atomic number 12, and atomic weight 24. 31. It is important for the activity of many enzymes, especially those involved in oxidative phosphorylation. Electrolytes supplementation, and benzodiazepines Benzodiazepines Benzodiazepines work on the gamma-aminobutyric acid type A (GABAA) receptor to produce inhibitory effects on the CNS. Benzodiazepines do not mimic GABA, the main inhibitory neurotransmitter in humans, but instead potentiate GABA activity. Benzodiazepines for agitation Agitation A feeling of restlessness associated with increased motor activity. This may occur as a manifestation of nervous system drug toxicity or other conditions. St. Louis Encephalitis Virus and seizure prophylaxis Prophylaxis Cephalosporins. 
  • Hepatic encephalopathy Encephalopathy Hyper-IgM Syndrome: altered mental status Altered Mental Status Sepsis in Children in the setting of chronic liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver: Anatomy disease due to elevated ammonia Ammonia A colorless alkaline gas. It is formed in the body during decomposition of organic materials during a large number of metabolically important reactions. Note that the aqueous form of ammonia is referred to as ammonium hydroxide. Acid-Base Balance levels. May be associated with ataxia Ataxia Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or peripheral nerve diseases. Motor ataxia may be associated with cerebellar diseases; cerebral cortex diseases; thalamic diseases; basal ganglia diseases; injury to the red nucleus; and other conditions. Ataxia-telangiectasia, personality changes, and altered level of consciousness Altered Level of Consciousness Intracerebral Hemorrhage. Diagnosis is clinical but supported by evidence of hepatic decompensation and elevated serum ammonia Ammonia A colorless alkaline gas. It is formed in the body during decomposition of organic materials during a large number of metabolically important reactions. Note that the aqueous form of ammonia is referred to as ammonium hydroxide. Acid-Base Balance. Lactulose Lactulose A synthetic disaccharide used in the treatment of constipation and hepatic encephalopathy. It has also been used in the diagnosis of gastrointestinal disorders. Laxatives is the mainstay of acute management for lowering ammonia Ammonia A colorless alkaline gas. It is formed in the body during decomposition of organic materials during a large number of metabolically important reactions. Note that the aqueous form of ammonia is referred to as ammonium hydroxide. Acid-Base Balance levels. Long-term management consists of efforts to stabilize hepatic function and hepatic transplantation. 
  • Lewy body dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders: condition associated with deposition of Lewy bodies Lewy bodies Intracytoplasmic, eosinophilic, round to elongated inclusions found in vacuoles of injured or fragmented neurons. The presence of lewy bodies is the histological marker of the degenerative changes in lewy body disease and parkinson disease but they may be seen in other neurological conditions. They are typically found in the substantia nigra and locus coeruleus but they are also seen in the basal forebrain, hypothalamic nuclei, and neocortex. Parkinson’s Disease (clumps of protein) in the cortical region of the brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification. Lewy body dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders is a progressive disease that presents with dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders, cognitive decline, and visual hallucinations Hallucinations Subjectively experienced sensations in the absence of an appropriate stimulus, but which are regarded by the individual as real. They may be of organic origin or associated with mental disorders. Schizophrenia. Diagnosis is based on the clinical presentation Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor. The time frame of symptom onset is an important piece of the history, as this entity presents more gradually than Wernicke/Korsakoff syndrome. There is no cure for Lewy body dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders. Treatment is supportive and aimed at reducing behavioral changes.

References

  1. Yuen T. S. (2020). Wernicke encephalopathy. UpToDate. Retrieved August 2, 2021, from https://www.uptodate.com/contents/wernicke-encephalopathy
  2. Covell T, Siddiqui W. (2021). Korsakoff syndrome. https://www.ncbi.nlm.nih.gov/books/NBK539854/
  3. Salen P N. (2018). Wernicke encephalopathy. MedScape. Retrieved August 2, 2021, from https://emedicine.medscape.com/article/794583
  4. Xiong G L. (2018). Wernicke-Korsakoff syndrome. MedScape. Retrieved August 2, 2021, from https://emedicine.medscape.com/article/288379
  5. Sabatini, J. S., Schutz-Pereira, G. L., Feltrin, F., Teive, H., Camargo, C. (2016). Wernicke’s encephalopathy with chorea: neuroimaging findings. Dementia & neuropsychologia. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5619281/
  6. Patel, S., Topiwala, K., Hudson, L. (2018). Wernicke’s encephalopathy. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6199146/
  7. Arts, N. J., Walvoort, S. J., Kessels, R. P. (2017). Korsakoff’s syndrome: a critical review. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5708199/
  8. Vasan, S., Kumar, A. (2020). Wernicke encephalopathy. https://www.ncbi.nlm.nih.gov/books/NBK470344/

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