Summary. An Orphanet summary for this disease is currently under development. However, other data related to the disease are accessible from the Additional. Pittella JE, de Castro LP. Wernicke’s encephalopathy manifested as Korsakoff’s syndrome in a patient with promyelocytic leukemia. South Med. Wernicke encephalopathy (WE) is an acute neurological condition characterized by a clinical Diekfuss JA, De Larwelle J, McFadden SH.
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Sarayu Vasan ; Anil Kumar. Authors Sarayu Vasan 1 ; Anil Kumar 2.
Wernicke encephalopathy WE is an acute neurological condition characterized by a clinical triad of ophthalmoparesis with nystagmus, ataxia, and confusion. This is a life-threatening illness caused by thiamine deficiency, which primarily affects the peripheral and central nervous systems.
Korsakoff syndrome is a neuropsychiatric disorder associated with memory disturbances in which there are significant deficits in anterograde and retrograde memory. Immediate memory is maintained, but short-term memory is diminished with intact sensorium. The disorder is associated with patients fabricating stories in the setting of clear consciousness. Confabulations can be spontaneous or provoked with provoked confabulation commonly seen in chronic Korsakoff syndrome and spontaneous confabulation usually noted in the acute Wernicke state.
Thiamine deficiency is characteristically associated with severe alcohol use disorder. Several studies indicate that prevalence rates via analysis of clinical records are lower in comparison to necropsy studies as the diagnosis is easily overlooked or missed.
This vitamin acts as a cofactor for several enzymes in the Krebs cycle and the pentose phosphate pathway, including alpha-keto-glutamic acid oxidation and pyruvate decarboxylation. Thiamine-dependent enzymes function as a connection between glycolytic and citric acid cycles. Therefore, deficiency of thiamine will lead to decreased levels of alpha-keto-glutarate, acetate, citrate, acetylcholine and accumulation of lactate and pyruvate. This deficiency can cause metabolic imbalances leading to neurologic complications including neuronal cell death.
Neuronal death in the mammillary bodies and thalamus were implicated in multiple cases of Wernicke encephalopathy studied. The lesions are usually symmetrical in the midbrain, hypothalamus, and cerebellum. Other oculomotor symptoms include cranial nerve involvement of oculomotor, abducens, and vestibular nuclei causing conjugate gaze palsies. Also, gait can worsen, and in many cases, patients are unable to walk.
Physical examination may include a complete neurological exam with cerebellar testing. Disorientation and altered sensorium characterize encephalopathy. Some other warning signs could include hyperthermia and hypotension. The patient could also present with peripheral neuropathy and commonly includes the lower extremity, and an examination would reveal distal sensory loss.
However, a complete blood count and the comprehensive metabolic panel can be completed to exclude other causes of central nervous system abnormalities. The aim of treatment is prompt and quick correction of the thiamine deficiency in the brain.
Parenteral administration of thiamine is most effective and provides for rapid administration, however, in some cases, there are persistent neurological deficits, and the acute condition can progress to chronic Korsakoff syndrome. All malnourished patient may need higher doses of thiamine. Because the disorder can present with various manifestations, the patient is best managed by a neurologist and an intensivist.
Other specialists may be required according to organ involvement. The role of the nurse, dietitian, social worker, and pharmacist cannot be overemphasized. These patients are sick, frail and malnourished.
A dietary consult should be done to assess the calorie needs and determine how to provide the food as well as thiamine. Since many WE patients are managed as outpatients, the pharmacist should encourage abstinence from alcohol. Since the enceffalopatia of therapy is thiamine, the importance of compliance is vital. At the same time, the electrolyte deficiencies should be corrected. Finally, the family should be educated about the prognosis of these individuals and make preparations for long-term care, in case the patient develops korsakoff syndrome.
WE is a serious medical disorder which carries an enormous morbidity and mortality. However, survivors of WE may develop korsakoff psychosis and require long-term institutionalization.
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Turn recording back on. National Center for Biotechnology InformationU. StatPearls Publishing; Jan. Show details Treasure Island FL: StatPearls Publishing ; Jan. Introduction Wernicke encephalopathy WE is an acute neurological condition characterized by a clinical triad of ophthalmoparesis with nystagmus, ataxia, and confusion.
Etiology Thiamine deficiency is characteristically associated with severe alcohol use disorder. Differential Diagnosis Differential diagnoses include: Questions To access free multiple choice questions on this topic, click here. Missing the early signs of thiamine deficiency. A case associated with a liquid-only diet. Preventing Wernicke’s encephalopathy in anorexia nervosa: Convergence Spasm in Wernicke Encephalopathy.
PMC ] [ PubMed: Prevalence and predictors of postoperative thiamine deficiency after vertical sleeve gastrectomy.
Surg Obes Relat Dis. Wernicke’s encephalopathy in crack-cocaine addiction. MRI picture worth a thousand words. Oxf Med Case Reports. Diagnosis makes a difference: Perceptions of older persons with dementia symptoms. Subclinical thiamine deficiency identified by preoperative evaluation in an ovarian cancer patient: Diagnosis and the need for preoperative thiamine measurement. Johnson JM, Fox V. Treatment for Cognitive Impairment in Korsakoff’s Syndrome. The alcohol withdrawal syndrome.
Clinical and pathological features of alcohol-related brain damage.
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Epub Nov 8. Wernicke-Korsakoff syndrome in the course of thyrotoxicosis – a case report. Review [Wernicke encephalopathy and Korsakoff’s psychosis: Clear Turn Off Turn On. Wernicke Encephalopathy – StatPearls. Support Center Support Center.