Wernicke–Korsakoff syndrome (WKS) is the combined presence of Wernicke encephalopathy (WE) and Korsakoff syndrome. Due to the close relationship between these two disorders, people with either are usually diagnosed with WKS as a single syndrome. It mainly causes vision changes, ataxia and impaired memory.The cause of the disorder is thiamine (vitamin B1) deficiency. This can occur due to Wernicke encephalopathy, eating disorders, malnutrition, and alcohol abuse. These disorders may manifest together or separately. WKS is usually secondary to prolonged alcohol abuse. Wernicke encephalopathy and WKS are most commonly seen in people with an alcohol use disorder. Failure in diagnosis of WE and thus treatment of the disease leads to death in approximately 20% of cases, while 75% are left with permanent brain damage associated with WKS. Of those affected, 25% require long-term institutionalization in order to receive effective care. Signs and symptoms
The syndrome is a combined manifestation of two namesake disorders, Wernicke encephalopathy and alcoholic Korsakoff syndrome. It involves an acute Wernicke-encephalopathy phase, followed by the development of a Korsakoff syndrome phase. Wernicke encephalopathy
WE is characterized by the presence of a triad of symptoms:
Ocular disturbances (ophthalmoplegia)
Changes in mental state (confusion)
Unsteady stance and gait (ataxia)This triad of symptoms results from a deficiency in vitamin B1 which is an essential coenzyme. The aforementioned changes in mental state occur in approximately 82% of patients symptoms of which range from confusion, apathy, inability to concentrate, and a decrease in awareness of the immediate situation they are in. If left untreated, WE can lead to coma or death.
Congenital hemolytic anemia refers to hemolytic anemia which is primarily due to congenital disorders. Types
Basically classified by causative mechanism, types of congenital hemolytic anemia include:
Genetic conditions of RBC Membrane
Hereditary spherocytosis
Hereditary elliptocytosis
Genetic conditions of RBC metabolism (enzyme defects). This group is sometimes called congenital nonspherocytic (hemolytic) anemia, which is a term for a congenital hemolytic anemia without spherocytosis, and usually excluding hemoglobin abnormalities as well, but rather encompassing defects of glycolysis in the erythrocyte.Glucose-6-phosphate dehydrogenase deficiency (G6PD or favism)
Pyruvate kinase deficiency
Aldolase A deficiency
Hemoglobinopathies/genetic conditions of hemoglobin
Sickle cell anemia
Congenital dyserythropoietic anemia
Thalassemia

See also
Hematopoietic ulcer
List of circulatory system conditions

References


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