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Authors: Jennifer S. Mattingley, MD Internal Medicine Residency Gundersen Lutheran Medical Foundation La Crosse, Wisconsin Linda C. Groon, MD Department of Internal Medicine Gundersen Lutheran Health System La Crosse, Wisconsin Address for correspondence: Jennifer Mattingley, MD Internal Medicine Residency Gundersen Lutheran Medical Foundation 1836 South Avenue La Crosse, WI 54601 Telephone: (608) 782-7300 Fax: (608) 775-4511 email: jsmattin@gundluth.org W Wernicke Encephalopathy: Is the Gundersen Lutheran Alcohol Detoxification Protocol Sufficient? ABSTRACT ernicke encephalopathy (WE) is an acute neuropsychiatric condition resulting from thiamine deficiency. It is classically characterized by a symptom triad of ataxia, mental status changes, and ocular abnormalities, although all 3 need not be present for the diagnosis. Up to 19% of patients with WE have none of the classic triad of symptoms at presentation and may present with irritability and fatigue alone. 1-3 Late-stage findings include hyperthermia, increased muscle tone, and choreic dyskinesias. 4,5 Thiamine deficiency is common in alcoholics, and therefore diagnosis and treatment of WE is often focused on care during alcohol detoxification. However, WE has also been well described in patients with Crohn disease, anorexia nervosa, acquired immune deficiency syndrome (AIDS), hyperemesis gravidarum, and in patients who have undergone chemotherapy or gastric bypass surgery. 6 Treatment is aimed at replenishing thiamine stores, especially to the areas of the brain most susceptible to thiamine deficiency. Although replacing thiamine is standard practice in patients with symptoms suggestive of WE and is often begun prophylactically in alcohol detoxification, there is increasing evidence that traditional dosages of thiamine replacement are suboptimal in improving outcomes of WE patients. We present a case report of a patient with WE, review the current literature, and discuss potential changes to standard practice of thiamine replacement. CASE REPORT A 35-year-old white man presented to the emergency department of Gundersen Lutheran Medical Center with complaints of inability to walk and slurred speech. He had a history of chronic alcohol abuse and previous alcohol withdrawal seizures. He stated that he believed he had a seizure 3 days prior We describe the case of a 35-year-old man who presented with central nervous system symptoms suggestive of Wernicke encephalopathy (WE). After ruling out other possible causes, and based upon clinical findings, WE was diagnosed. WE is caused by a thiamine deficiency and because thiamine deficiency is common among people who abuse alcohol, thiamine replacement is a component of Gundersen Lutheran’s alcohol detoxification protocol. However, review of the literature suggests that the current thiamine replacement regimen may not be sufficient, especially for patients with symptoms of WE. to his presentation. Since that time he had been unable to walk and had had multiple falls. He also stated he was unable to “see straight.” He was, however, ambivalent about these symptoms. He had continued to drink alcohol. He stated he was drinking “less than usual,” but was unwilling to quantify further. He also admitted to not eating for several days. On initial examination he was awake and oriented to person. Vitals included heart rate of 120 beats per minute and blood pressure of 139/102 mm Hg. The patient was afebrile. Neurologic examination revealed marked vertical, horizontal, and rotary nystagmus. Bilateral sixth cranial nerve palsies were present. Function of the remaining cranial nerves was intact. Strength was normal in the upper and lower extremities. Occasional choreiform movements of the left upper extremity, especially with provocation, were noted. Deep tendon reflexes were brisk but without clonus, and Babinski sign was absent bilaterally. There was marked bilateral upper extremity ataxia and gait ataxia. Because of truncal ataxia, he could not sit up without support. He had marked dysarthria with a paucity of spontaneous speech. Due to his marked dysarthria and poor level of cooperation, it was difficult to fully assess cognition. On skin examination, multiple areas of ecchymosis and abrasions were noted on his extremities, trunk, and face. Laboratory test results showed a white blood cell (WBC) count of 14 500/μL, hemoglobin concentration of 17.9 g/dL, and a platelet count of 102 ×10 3 /μL. Potassium was markedly depleted at 2.4 mEq/L. Sodium was 134 mEq/L, chloride 80 mEq/L, and magnesium 2.0 mEq/L (decreased to 1.3 mEq/L within 48 hours). Other electrolyte and glucose values were within reference range. Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) concentrations were marginally elevated at 73 U/L and 51 U/L, respectively (Table). International normalized ratio (INR) was slightly elevated at 1.2. Findings from noncontrast computed Gundersen Lutheran Medical Journal • Volume 5, Number 1, July 2008 13
tomographic scan of the head were normal. Findings from subsequent magnetic resonance imaging of the head performed the following day were also without specific abnormality. No infectious etiology was identified, and his electrolytes were replaced. Given the clinical findings and exclusion of other causes of central nervous system dysfunction, WE was diagnosed. Within hours of the patient’s admission, his nystagmus and Laboratory Test Results Upon Admission Analyte Value Reference Range WBC count, /μL 14 500 4500 -11 000 Hemoglobin concentration, g/dL 17.9 14.0 -17.5 Platelet count, ×10 3 /μL 102 150-350 Potassium concentration, mEq/L Sodium concentration, mEq/L Chloride concentration, mEq/L Magnesium concentration, mEq/L 2.4 3.5-5.0 134 136 -142 80 96 -106 2.0 1.3 -2.1 AST concentration, U/L 73 10 -30 ALT concentration, U/L 51 10 -40 Abbreviations: WBC, white blood cell; AST, aspartate aminotransferase; ALT, alanine aminotransferase. SI conversions: To convert WBC count to ×10 9 /L, multiply by 0.001; hemoglobin to g/L, multiply by 10.0; platelet count to ×10 9 /L, multiply by 1.0; potassium, sodium, and chloride to mmol/L, multiply by 1.0; magnesium to mmol/L, multiply by 0.50; AST to μkat/L, multiply by 0.0167; ALT to μkat/L, multiply by 0.01667. opthalmoplegia had resolved. Thiamine 100 mg intravenously (IV) had been administered in the emergency department prior to admission per the existing alcohol detoxification protocol of thiamine 100 mg orally, by IV, or intramuscularly (IM) daily for 3 days. Initial inpatient orders were to continue thiamine 100 mg IV daily for 3 days, again per the existing protocol. Prompted by review of literature on WE, however, within 24 hours his thiamine dosage was increased to 250 mg IV 3 times daily for 5 days. He then received thiamine orally during the remainder of his 3-week hospital stay. His ataxia improved but did not completely resolve. At the time of discharge the patient was still unable to walk due to ataxic gait, and his upper extremity ataxia remained prominent, as well. He continued to be dysarthric. Neuropsychiatric testing demonstrated significant memory deficits with mild immediate recall deficits and severe short and long delayed recall deficits. DISCUSSION Wernicke encephalopathy is an acute syndrome classically characterized by ataxia, altered mental status, and ocular abnormalities of nystagmus and ophthalmoplegia. Carl Wernicke first described it as a superior hemorrhagic polioencephalitis in 1881. He reported case studies of 3 patients, all of whom died within 2 weeks of presentation. Two of the patients were men with chronic alcoholism, while the third patient (a woman) had persistent vomiting secondary to pyloric stenosis from sulphuric acid ingestion. 7 In the 1940s, Campbell and Russell were the first to implicate thiamine deficiency as the underlying etiology of the encephalopathy. 8 Based upon postmortem studies identifying typical brainstem lesions, WE has a prevalence of 0.8% to 2.8% in the general adult population of the United States and 12.5% in the population that misuses alcohol. 1,9,10 This is a much higher incidence than what is diagnosed clinically. As only 10% of patients with WE present with the classic triad of signs, it is suggested that up to 90% of individuals with WE go undiagnosed. 1,11 There is a definite male predominance (1.7:1). 12 Ethnic-specific differences in presentation of thiamine deficiency also exist. Europeans more commonly develop encephalopathy and peripheral neuropathy (dry beriberi), while the majority of Asians manifest with cardiovascular effects (wet beriberi). 13 Thiamine deficiency leads to pathologic lesions within the brain, especially the periaqueductal grey matter, mamillary bodies, and medial thalamus, all of which have a high thiamine content and metabolism. 14 Neuronal cells convert thiamine to thiamine pyrophosphate, the metabolically active form, which is essential for ATP synthesis, myelin sheath production, and synthesis of neurotransmitters. 15 Within 4 days of developing deficient thiamine levels, there is marked decrease in activity of alpha-ketoglutaratedehydrogenase within astrocytes. By 7 days there is reduction in activity of transketolase and subsequent increase in nitric oxide from endothelial cell dysfunction. This leads to astrocytic and neuronal edema and disruption of the blood-brain barrier. Within 2 weeks of thiamine deficiency there is evidence of neuronal DNA fragmentation and necrosis, which leads to irreversible lesions 16 -26 within the brain. Average thiamine requirement for an adult is 0.5 mg of thiamine per 1000 kcal (4184 kilojoules) although needs are significantly higher in children and in patients who are critically ill, pregnant, or lactating. 27 Requirements are also related to carbohydrate intake as well as total caloric intake. 28 Diets high in carbohydrates increase the demand for thiamine because it is vital in carbohydrate metabolism. Chronic alcoholics are often malnourished with suboptimal thiamine intake and have decreased ability for vitamin storage in the liver, while alcohol itself increases the need for carbohydrate metabolism. 29 Thus, chronic alcoholics can often become significantly thiamine depleted. Absorption of thiamine occurs in the duodenum by an active, carrier-mediated, and rate-limited process. Therefore, the maximum amount of thiamine that can be absorbed from a single oral dose is ~ 4.5 mg. 30 In alcoholics, enteral absorption of thiamine is diminished and erratic. 29 Alcohol alone can decrease intestinal absorption of thiamine by 50%; malnutrition can 14 Gundersen Lutheran Medical Journal • Volume 5, Number 1, July 2008
Page 1 and 2: Th e validity of clarke sign in ass
Page 3 and 4: editor David E. Hartman, PhD, BC-AN
Page 5 and 6: perioperative stroke with carotid-a
Page 7 and 8: Authors: Denise Milton, MS, PT John
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Page 11 and 12: it should mimic common ADLs. Accord
Page 13 and 14: an individual has been anorexic, th
Page 15: 15. Lask B, Bryant-Waugh R. Early-o
Page 19 and 20: 8. Campbell ACP, Russell WR. Wernic
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Page 31 and 32: 52. Anonymous. Book notices and rev
Page 33 and 34: 394 Risty et al Surgery September 2
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