Abstract

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Wernicke’s encephalopathy (WE) is the best known neurological complication of thiamine deficiency, characterized by ocular, symptoms, mental confusion and ataxia. we report herewith a male child with unusual features, suggestive of WE and with thiamine treatment, the response was dramatic.Participated in the national seminar on Consumerism in DNR College, Bvrm

Abstract

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Wernicke’s encephalopathy (WE) is the best known neurological complication of thiamine deficiency, characterized by ocular, symptoms, mental confusion and ataxia. (1) Though a well known problem, noted primarily in alcoholics, WE is also described in malnutrition, prolonged parenteral nutrition, persons on slimming diet, dialysis, and haemotological malignancies.(2) (3). WE is said to be a rare entity in infants and children and we report herewith a male child with unusual features, suggestive of WE and with thiamine treatment, the response was dramatic.

case:A 12 yr old boy presented to ER with lethargy, confusion following repeated vomiting for 3 days. He was apparently well before and during the beginning of the holy month for Muslims (Ramadan); he started his fasting, which consists of withholding all forms oral intake of food and water from day break till sun set. This was his first religious fasting ever done and he started performing it. On the 12 th day of his fasting, at day break, he felt dizzy, vomited several times with abdominal discomfort. He complained of diminished vision and later with persistent vomiting he became drowsy, disoriented. He was brought to the hospital; on initial evaluation he was diagnosed as a case of encephalopathy and admitted for further workup. Neurological examination revealed that he was drowsy, arousable with stimulation but could not sustain his conscious state. He was afebrile, stable vital signs with mild dehydration and there were no meningeal signs. His cranial nerves examination revealed that he had ptosis of right eye lid with intact pupillary light response as well extra ocular eye movements. There was no long tract signs except he was ataxic while made to stand which was attributed to his altered sensorium. Routine investigations including complete blood count and biochemistry were normal. Computerized tomography (CT) of the brain was normal and CSF examination showed normal proteins and less than 4 lymphocytes. He was hydrated with intravenous fluids and on 2nd day examination he was found to be ataxic with tremors of his upper limbs. With the clinical signs of encephalopathy, cerebellar ataxia and possible ophtahlmoplegia, a diagnosis of wernick encephalopathy was entertained and after collecting blood for vitamin B1 levels, he was started on thiamine 2mg/Kg by intravenous route for seven days. He became fully conscious the next day, and ataxia improved in the next few days. He was on regular oral thiamine 50 mg 2 times daily and he was periodically assessed. At time of discharge he was symptom free and even the ptosis improved. He was followed up after 4 weeks and there were no recurrence of symptoms. Blood thiamine level was 36 ng/ml ( normal 50 ng/ml) which normalized later

Discussion

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Wernick described in 1878 four adult patients of encephalopathy with ophthalmoplegia with malnutrition. The autopsy then confirmed the characteristic hemorrhagic lesions in dorsomedial thalamus and periaqueductal/periventricular gray mater. The disease is said to be caused by thiamine deficiency. Beriberi is the systemic counterpart of thiamine deficiency and often manifests in cardiovascular collapse. Thiamine is an important co-enzyme in glucose metabolism and it is converted into its active form, thiamine pyrophosphate. There are three important enzyme systems in the tri-carboxylic acid cycle in the liver, kidney, heart and brain which are dependant upon thiamine.(4) A triad of clinical symptoms, namely, global mental confusion, ocular abnormalities and ataxia are important in the diagnosis of WE.(5) However, most of the patients may not manifest all the above symptoms always; instead a variable combination of presentations is sufficient to diagnose WE. Therefore, a high index of suspicion is necessary in the high risk patients with any of the above myriad of clinical symptoms. (6) Extra neurological involvement had been also described in WE in children with predominant cardiovascular symptoms namely, chest pain, dyspnoea, tachycardia and heart failure. Confirmation of the diagnosis is possible by reduced red blood cell transketolase levels and high serum pyurvate and lactate but the results could not be obtained immediately. Magnetic resonance imaging of the brain may reveal abnormal T2 bright signals in the dorsomedial thalamic nuclei and putamen with loss of volume of the mamillary bodies. WE is both preventable and treatable condition and treatment should be initiated any patient who presents with any of the above clinical signs and symptoms, especially in the high risk group. The onset of the disease may be acute, sub acute or chronic and symptoms of classical triad may not be evident. Therefore, thiamine therapy is warranted if any component of Wernicke'e encephalopathy triad is present an appropriate clinical setting (7). If untreated, patents may progress to hypotension, hypothermia and respiratory failure and so early treatment could avoid such complications and fatality, provided the awareness of this disease present among physicians. Although known in malnutrition or in patients on parenteral nutrition, our patient was an apparently healthy boy who responded to thiamine with the clinical suspicion and management.

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