Related articles: Wernicke's encephalopathy
Definition
Wernicke's encephalopathy is an acute neuropsychiatric syndrome that results from a severe deficiency of thiamine (vitamin B1).
The most common cause of this disorder is alcoholism. The excessive intake of alcohol interferes, in fact, with the absorption of thiamine at the gastro-intestinal level and with its deposition in the liver. Furthermore, malnutrition associated with alcoholism often prevents adequate intake of vitamin B1.
Wernicke's encephalopathy may also result from other conditions that result in prolonged hyponutrition or vitamin deficiency (eg frequent dialysis, hyperemesis, anorexia nervosa, treatment for re-feeding after prolonged fasting, bariatric surgery, gastric cancer and AIDS). At the base of the disease, there may also be genetic abnormalities that result in a deficiency of transketolase, an enzyme that metabolizes thiamine.
Most common symptoms and signs *
- Anosmia
- Apathy
- Ataxia
- Coma
- Muscle cramps
- Difficulty concentrating
- Language difficulties
- Temporal and spatial disorientation
- Dyspnoea
- Hand and wrist pain
- ecolalia
- Papilla edema
- Sore legs
- Hypertension
- undernourishment
- Orthostatic hypotension
- Hypothermia
- hypovitaminosis
- Lethargy
- Nystagmus
- Ophthalmoplegia
- Paresthesia
- Memory loss
- Loss of coordination of movements
- Loss of balance
- Drowsiness
- Confusional state
- Fainting
- Tachycardia
- Tremors
- Dizziness
- He retched
Further indications
Wernicke's encephalopathy is characterized by the sudden onset of symptoms, such as confusion, nystagmus, partial ophthalmoplegia, language disorders, ataxic gait and decay of mental faculties.
In Wernicke's encephalopathy, an alteration of vestibular function is frequent, without hearing loss. The state of confusion is characterized by profound disorientation, apathy, indifference, inability to concentrate and drowsiness.
Other dysfunctions often related to Wernicke's encephalopathy include inability to distinguish odors, tremor, agitation, hypothermia, signs of cardiovascular changes (tachycardia, exertional dyspnea and orthostatic hypotension) and syncope. Thiamine deficiency also produces symptoms that result from the involvement of the peripheral nervous system, such as tingling, cramping, numbness and pain in the legs and hands.
In untreated patients, Wernicke's encephalopathy can progress to stupor, coma and death.
The diagnosis is mainly clinical and is based on the recognition of the underlying hypotension or vitamin deficiency. To exclude other etiologies, the patient must undergo blood tests, liver function tests, toxicological screening, brain imaging and cerebrospinal fluid analysis.
The treatment consists of immediate administration of thiamine parenterally, continued for at least 3-5 days. Ocular symptoms usually begin to disappear within 24 hours, while ataxia and confusion may persist for days and months.
Supportive therapy consists of rehydration, correction of electrolyte alterations and general nutritional treatment, which includes multivitamin complexes. Patients with advanced illnesses require hospitalization. The cessation of alcohol intake is clearly mandatory.
The prognosis related to Wernicke's encephalopathy depends on a timely diagnosis; following the therapy, the disorder can regress, persist or degenerate in Korsakoff psychosis (a condition characterized by severe memory defects, ataxia, apathy, disorientation, confabulations, hallucinations, paralysis of the muscles that control the eye and coma). If untreated, however, the disorder progresses; mortality ranges from 10 to 20% of cases.
