Wernicke-Korsakoff syndrome
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Synopsis

Emergent Care / Stabilization:
Acute Wernicke encephalopathy is a clinical diagnosis, and prompt treatment can produce recovery. The treatment is high-dose thiamine replacement to prevent progression to Korsakoff syndrome.
Diagnosis Overview:
Wernicke-Korsakoff syndrome is a collection of clinical manifestations that result from vitamin B1 (thiamine) deficiency. The acute clinical manifestations of thiamine deficiency are known as Wernicke encephalopathy (WE). The classic clinical triad for diagnosis is confusion, ophthalmoplegia, and ataxia. Korsakoff syndrome occurs due to chronic thiamine deficiency and is an irreversible manifestation of acute symptoms. Most often, this condition is noted in patient populations with inadequate nutrient absorption or intake of thiamine. Wernicke-Korsakoff syndrome can occur from any condition leading to thiamine deficiency. It is most commonly seen in patients with alcohol use disorder, anorexia nervosa and other eating disorders, a history of gastrointestinal surgery, conditions with hyperemesis, and any other condition causing malnutrition.
WE presents with confusion (disorientation, inattention, memory dysfunction), ophthalmoplegia (horizontal and vertical nystagmus), and ataxia (unsteady, wide-based gait with short-spaced steps). Acute WE is a clinical diagnosis, and prompt treatment can produce recovery. The treatment is high-dose thiamine replacement to prevent Korsakoff syndrome.
Korsakoff syndrome, also known as alcohol amnestic disorder, is a late neuropsychiatric manifestation of chronic WE. Memory loss is the most serious effect; the patient usually has severe impairment of short-term memory. Confabulations are also a common characteristic of patients with Korsakoff syndrome. In alcohol use disorder, thiamine deficiency and alcohol's direct neurotoxic effects combine to produce deficits in anterograde and retrograde memory and apathy. Recovery in Korsakoff syndrome patients is uncommon.
Acute Wernicke encephalopathy is a clinical diagnosis, and prompt treatment can produce recovery. The treatment is high-dose thiamine replacement to prevent progression to Korsakoff syndrome.
Diagnosis Overview:
Wernicke-Korsakoff syndrome is a collection of clinical manifestations that result from vitamin B1 (thiamine) deficiency. The acute clinical manifestations of thiamine deficiency are known as Wernicke encephalopathy (WE). The classic clinical triad for diagnosis is confusion, ophthalmoplegia, and ataxia. Korsakoff syndrome occurs due to chronic thiamine deficiency and is an irreversible manifestation of acute symptoms. Most often, this condition is noted in patient populations with inadequate nutrient absorption or intake of thiamine. Wernicke-Korsakoff syndrome can occur from any condition leading to thiamine deficiency. It is most commonly seen in patients with alcohol use disorder, anorexia nervosa and other eating disorders, a history of gastrointestinal surgery, conditions with hyperemesis, and any other condition causing malnutrition.
WE presents with confusion (disorientation, inattention, memory dysfunction), ophthalmoplegia (horizontal and vertical nystagmus), and ataxia (unsteady, wide-based gait with short-spaced steps). Acute WE is a clinical diagnosis, and prompt treatment can produce recovery. The treatment is high-dose thiamine replacement to prevent Korsakoff syndrome.
Korsakoff syndrome, also known as alcohol amnestic disorder, is a late neuropsychiatric manifestation of chronic WE. Memory loss is the most serious effect; the patient usually has severe impairment of short-term memory. Confabulations are also a common characteristic of patients with Korsakoff syndrome. In alcohol use disorder, thiamine deficiency and alcohol's direct neurotoxic effects combine to produce deficits in anterograde and retrograde memory and apathy. Recovery in Korsakoff syndrome patients is uncommon.
Codes
ICD10CM:
E51.2 – Wernicke's encephalopathy
F04 – Amnestic disorder due to known physiological condition
F10.26 – Alcohol dependence with alcohol-induced persisting amnestic disorder
SNOMEDCT:
21007002 – Wernicke's disease
69482004 – Korsakoff's psychosis
E51.2 – Wernicke's encephalopathy
F04 – Amnestic disorder due to known physiological condition
F10.26 – Alcohol dependence with alcohol-induced persisting amnestic disorder
SNOMEDCT:
21007002 – Wernicke's disease
69482004 – Korsakoff's psychosis
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
Like all clinically diagnosed neurologic conditions, the differential is broad. The history and clinical picture can be used to narrow down this differential in conjunction with additional laboratory analysis, neurologic imaging, and possibly a lumbar puncture.
Toxicologic:
Toxicologic:
- Serotonin syndrome
- Alcohol intoxication (see toxic alcohol poisoning)
- Illicit drug use or adverse drug reactions
- Hypoglycemia
- Hyperglycemia
- Hepatic encephalopathy
- Hyponatremia
- Inborn errors of metabolism in children
- Delirium
- Psychosis or drug-induced psychosis
- Thyroid disease (see thyroiditis)
- Other endocrine deficiencies – parathyroid, pancreas, pituitary, adrenal
- Epidural hematoma
- Subdural hematoma
- Ischemic stroke (see, eg, basilar artery occlusion)
- Intracerebral hemorrhage
- Third ventricle tumor
- Paraneoplastic encephalitis
- Acute disseminated encephalomyelitis
- Normal pressure hydrocephalus
- Lewy body dementia
- Alzheimer dementia
- Frontotemporal dementia
- Corticobasal degeneration
- Progressive supranuclear palsy
- Miller-Fisher variant of Guillain-Barré syndrome
- Seizure (eg, temporal lobe epilepsy) or post-ictal state
- Infectious encephalitis (eg, herpes simplex virus encephalitis)
- Neurosyphilis
- Creutzfeldt-Jakob disease
- Human immunodeficiency virus / AIDS dementia
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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References
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Last Reviewed:03/29/2023
Last Updated:03/30/2023
Last Updated:03/30/2023