Wernicke-Korsakoff syndrome

20,029views

00:00 / 00:00

Videos

Notes

Wernicke-Korsakoff syndrome

Pathology

Central nervous system disorders

Spina bifida

Chiari malformation

Dandy-Walker malformation

Syringomyelia

Tethered spinal cord syndrome

Aqueductal stenosis

Septo-optic dysplasia

Cerebral palsy

Spinocerebellar ataxia (NORD)

Transient ischemic attack

Ischemic stroke

Intracerebral hemorrhage

Epidural hematoma

Subdural hematoma

Subarachnoid hemorrhage

Saccular aneurysm

Arteriovenous malformation

Broca aphasia

Wernicke aphasia

Wernicke-Korsakoff syndrome

Kluver-Bucy syndrome

Concussion and traumatic brain injury

Shaken baby syndrome

Epilepsy

Febrile seizure

Early infantile epileptic encephalopathy (NORD)

Tension headache

Cluster headache

Migraine

Idiopathic intracranial hypertension

Trigeminal neuralgia

Cavernous sinus thrombosis

Alzheimer disease

Vascular dementia

Frontotemporal dementia

Lewy body dementia

Creutzfeldt-Jakob disease

Normal pressure hydrocephalus

Torticollis

Essential tremor

Restless legs syndrome

Parkinson disease

Huntington disease

Opsoclonus myoclonus syndrome (NORD)

Multiple sclerosis

Central pontine myelinolysis

Acute disseminated encephalomyelitis

Transverse myelitis

JC virus (Progressive multifocal leukoencephalopathy)

Adult brain tumors

Acoustic neuroma (schwannoma)

Pituitary adenoma

Pediatric brain tumors

Brain herniation

Brown-Sequard Syndrome

Cauda equina syndrome

Treponema pallidum (Syphilis)

Vitamin B12 deficiency

Syringomyelia

Friedreich ataxia

Neurogenic bladder

Meningitis

Neonatal meningitis

Encephalitis

Brain abscess

Epidural abscess

Cavernous sinus thrombosis

Creutzfeldt-Jakob disease

Central and peripheral nervous system disorders

Sturge-Weber syndrome

Tuberous sclerosis

Neurofibromatosis

von Hippel-Lindau disease

Amyotrophic lateral sclerosis

Peripheral nervous system disorders

Spinal muscular atrophy

Poliovirus

Guillain-Barre syndrome

Charcot-Marie-Tooth disease

Trigeminal neuralgia

Bell palsy

Winged scapula

Thoracic outlet syndrome

Carpal tunnel syndrome

Ulnar claw

Erb-Duchenne palsy

Klumpke paralysis

Sciatica

Myasthenia gravis

Lambert-Eaton myasthenic syndrome

Autonomic nervous system disorders

Orthostatic hypotension

Horner syndrome

Nervous system pathology review

Congenital neurological disorders: Pathology review

Headaches: Pathology review

Seizures: Pathology review

Cerebral vascular disease: Pathology review

Traumatic brain injury: Pathology review

Spinal cord disorders: Pathology review

Dementia: Pathology review

Central nervous system infections: Pathology review

Movement disorders: Pathology review

Neuromuscular junction disorders: Pathology review

Demyelinating disorders: Pathology review

Adult brain tumors: Pathology review

Pediatric brain tumors: Pathology review

Neurocutaneous disorders: Pathology review

Assessments

Wernicke-Korsakoff syndrome

Flashcards

0 / 15 complete

USMLE® Step 1 questions

0 / 3 complete

Flashcards

Wernicke-Korsakoff syndrome

of complete

Questions

USMLE® Step 1 style questions USMLE

of complete

An 85-year-old man is brought in by his niece to the clinic after finding him confused outside his house. He denies the incident and says that he was “just walking his dog around the neighborhood” after having a meal with his friends. After the consultation, his niece states that “He doesn't own a dog, he lives alone, and most of his meals consist of reheating canned foods.” Past medical history includes hypertension, hypercholesterolemia and type 2 diabetes. Medications include hydrochlorothiazide, atorvastatin and metformin.  Family history is noncontributory. He has been drinking 5-6 beers daily for the past 30 years. Temperature is 37.0°C (98.6°F), pulse is 92/min, respirations are 17/min, and blood pressure is 135/85 mmHg. He is oriented to place and person but not time. Immediate and delayed memory recall is poor. Pupils are equal and reactive, and horizontal nystagmus is elicited on lateral gaze. Fundoscopy is normal. There is no nuchal rigidity on examination. Motor strength is 5/5 in the upper extremities and 2/4 in bilateral lower extremities. Deep tendon reflexes of the knee and ankle are 1+ bilaterally. MRI reveals necrosis in the mamillary bodies and medial thalamus. Which of the following is the most likely diagnosis?

External References

First Aid

2022

2021

2020

2019

2018

2017

2016

Ataxia

Wernicke-Korsakoff syndrome p. 64, 528, 595

Mammillary bodies p. 521, 528

Wernicke-Korsakoff syndrome p. 595

Memory loss

Wernicke-Korsakoff syndrome p. 64, 528, 595

Ophthalmoplegia p. 64

Wernicke-Korsakoff syndrome p. 64, 595

Vitamin B1 deficiency

Wernicke-Korsakoff syndrome p. 595

Wernicke-Korsakoff syndrome p. 528, 595

vitamin B1 deficiency p. 64

Transcript

Content Reviewers

Rishi Desai, MD, MPHYifan Xiao, MD

Marisa Pedron

Contributors

Royce Rajan, MD, MBA

Ursula Florjanczyk, MScBMC

Evan Debevec-McKenney

Tanner Marshall, MS

Wernicke Korsakoff syndrome is named after Carl Wernicke and Sergei Korsakoff, the physicians who discovered the condition in the late 1800s. Wernicke Korsakoff syndrome is caused by Vitamin B1 or thiamine deficiency and it refers to a spectrum of disease. Wernicke's encephalopathy is the acute, reversible stage of the syndrome, and if left untreated it can later lead to Korsakoff syndrome, which is chronic and irreversible.

Thiamine is typically stored in the liver and absorbed in the jejunum and ileum, and then moves throughout the body, where it’s involved in numerous cellular processes that require thiamine. The enzyme thiamine pyrophosphate synthetase transfers a pyrophosphate group from ATP to thiamine, turning it into the coenzyme thiamine pyrophosphate - which is the metabolically active form of thiamine. Now, as a coenzyme, thiamine pyrophosphate functions to assist other enzymes such as pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase, and transketolase carry out reactions, particularly regarding glucose metabolism. Furthermore, within the brain, thiamine pyrophosphate helps metabolize lipids and carbohydrates as well as maintain normal amino acid and neurotransmitter levels. In some neurons, thiamine even helps with propagation of a neural impulses down the axon.

Given it’s multifaceted role, a deficiency of thiamine can have serious consequences. Specifically, thiamine deficiency impairs glucose metabolism and this leads to a decrease in cellular energy. One of the major causes of thiamine deficiency, and therefore Wernicke Korsakoff syndrome, is alcohol abuse. Alcohol leads to decreased thiamine levels in various ways. First, alcohol interferes with the conversion of thiamine to its active form, thiamine pyrophosphate by blocking the phosphorylation of thiamine. Second, thiamine is normally absorbed through the first portion of the small intestine called the duodenum. However, ethanol prevents this absorption process, and it is believed that alcohol does this by reducing the gene expression for thiamine transporter-1 within the intestinal brush border. Third, chronic alcohol abuse can lead to fatty liver or cirrhosis which interferes with the storage of thiamine within the liver. Other causes of thiamine deficiency are inadequate intake like in malnutrition and anorexia or due to malabsorption like in stomach cancer and inflammatory bowel disease.

Summary

Wernicke-Korsakoff syndrome (WKS) is a neurological disorder caused by a deficiency of thiamine (vitamin B1), often due to chronic alcohol abuse. It is a combination of Wernicke encephalopathy and Korsakoff syndrome, presenting with confusion, ophthalmoplegia, and ataxia. Wernicke encephalopathy can progress to Korsakoff syndrome, which causes severe memory loss, disorientation, and confabulation. Treatment includes thiamine supplements, rehabilitation, and cognitive therapy to manage symptoms.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "THE NATURAL HISTORY AND PATHOPHYSIOLOGY OF WERNICKE'S ENCEPHALOPATHY AND KORSAKOFF'S PSYCHOSIS" Alcohol and Alcoholism (2005)
  5. "Health problems and care needs in patients with Korsakoff’s syndrome: A systematic review" Journal of Psychiatric and Mental Health Nursing (2020)
Elsevier

Copyright © 2023 Elsevier, except certain content provided by third parties

Cookies are used by this site.

USMLE® is a joint program of the Federation of State Medical Boards (FSMB) and the National Board of Medical Examiners (NBME). COMLEX-USA® is a registered trademark of The National Board of Osteopathic Medical Examiners, Inc. NCLEX-RN® is a registered trademark of the National Council of State Boards of Nursing, Inc. Test names and other trademarks are the property of the respective trademark holders. None of the trademark holders are endorsed by nor affiliated with Osmosis or this website.

RELX