Wernicke’s Encephalopathy After Laparoscopic Cardiomyotomy for Achalasia

By Kennedy, R Hunt, S; Ahmad, J; Menezes, C; Et al

ABSTRACT. Achalasia is an incurable neuromuscular disorder of me esophagus, resulting from destruction of the esophageal myenteric plexus. This leads to aperistalsis and failure of the lower esophageal sphincter to relax after swallowing. Symptoms of achalasia are gradual in onset and include dysphagia, regurgitation, and weight loss. Severe malnutrition can ensue. Wernicke’s encephalopathy (WE) is a serious, potentially fatal, neurologic disorder caused by thiamine deficiency (vitamin B1), classically described as presenting with a triad of ocular abnormalities, ataxia, and confusion. The incidence is uncertain, and many cases likely go unrecognized. It is usually diagnosed in the alcoholic population. We describe its onset after the successful surgical treatment of achalasia. (Journal of Parenteral and Enteral Nutrition 31:324-325, 2007) Case Presentation

A 28-year-old man presented through the emergency department with dysphagia, vomiting, regurgitation, and dehydration. His diet had been limited for 2 months. He was morbidly obese, with a weight of 160 kg and body mass index (BMI) of 49.2 kg/m^sup 2^. There was no history of alcohol excess. Upper gastrointestinal endoscopy showed dilatation of the lower third of his esophagus. A barium swallow confirmed a dilated esophagus and demonstrated a classic “bird’s- beak” tapering of the esophagogastric junction, in keeping with achalasia (Figure 1). He did not tolerate esophageal manometry, but his Achalasia Disease Severity Score1 was 26/31. He underwent laparoscopic cardiomyotomy with anterior fundoplication.2 Free fluids were commenced on postoperative day 1 and soft diet on day 2. On day 3, he became confused and uninterested. Blood pressure and temperature were normal. His pupils were sluggishly reactive, with marked bilateral vertical nystagmus. He had severe truncal ataxia, being unable to sit or stand unaided. His serum glucose, inflammatory markers, and electrolytes were normal. Computed tomography (CT) of his brain was unremarkable. He was treated for Wernicke’s encephalopathy (WE) with IV Pabrinex (vitamin B and C complex; Link Pharmaceuticals Ltd, Horsham, West Sussex, United Kingdom) and made an excellent and rapid clinical response. Magnetic resonance imaging (MRI) of his brain demonstrated signal change in the posteromedial thalamus bilaterally, in keeping with WE (Figure 2). He was discharged from the hospital on the 10th postoperative day, with minimal residual neurologic impairment, receiving oral thiamine replacement.

Figure 1. Barium meal showing the classic “bird’s-beak” picture of achalasia.

DISCUSSION

WE is a rare neurologic disorder caused by thiamine deficiency. The classic triad of ocular abnormalities, ataxia, and a global confusional state is present in only 16%-30%3; hence, diagnosis is often difficult and late.4 Although primarily considered a disease of alcoholics, up to 20% of cases may occur in the nonalcoholic population.5 It has been described after prolonged IV feeding, bariatric surgery, and refeeding after starvation.6,7 Mortality rates range from 10% to 20%, and residual nystagmus and ataxia is seen in up to 60% of patients.

Figure 2. MRI. Diffusion-weighted sequence shows abnormal high signal bilaterally in the posteromedial thalamus.

Although the pathogenesis of WE has not been well defined, there is little debate that thiamine deficiency is the cause. Thiamine is an essential cofactor in the oxidative decarboxylation of pyruvate to acetyl CoA, providing substrate for the Krebs cycle within mitochondria. Dysfunction of this process in thiamine deficiency may result in focal lactic acidosis within vulnerable brain structures such as the mamillary bodies and posteromedial thalamus. Apoptotic cell death can then ensue via ?G-methyl-D-aspartate receptor- mediated excitotoxicity.8 This cell death may produce the neurologic symptomatology. Diets rich in carbohydrates especially increase the metabolic demands of thiamine and may exacerbate this process in those who are thiamine deficient. Diets rich in bread, cereal, whole grains, lean meats, fish, and peas contain high levels of thiamine and are protective. IV thiamine is the recommended treatment for WE, and patients respond rapidly; however, failure to promptly recognize and treat can lead to permanent memory disability (Korsakoff s syndrome) or death.

Achalasia is a rare progressive neuromuscular disorder of the esophagus, causing difficulty swallowing and weight loss.9 It appears that our patient’s thiamine deficiency occurred as a result of malnutrition secondary to severe dysphagia. Successful cardiomyotomy allowed him to introduce a normal diet. Refeeding, especially with high-carbohydrate foods, appears to have increased the metabolic demands on his depleted thiamine stores and precipitated WE. The combination of clinical features, rapid improvement after therapy, and the subsequent MRI findings confirmed the diagnosis of WE. WE has been reported after gastric bypass for morbid obesity7; however, it has never before been described after treatment for achalasia.

CONCLUSIONS

WE can occur after successful treatment for achalasia and may be associated with prolonged malnutrition and refeeding. Rapid treatment with IV thiamine is essential to avoid permanent neurologic disability. Clinicians should be aware that even the obese patient can be profoundly malnourished.

REFERENCES

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R. Kennedy, MRCSI*; S. Hunt, MRCP[dagger]; J. Ahmad, MRCS[dagger]; C. Menezes, MRCS*; W. B. Clements, FRCS*; and J. A. Kennedy, FRCS*

From the * Department of Upper Gastrointestinal Surgery and the t Department of Neurology, Royal Victoria Hospital, Belfast, Northern Ireland, United Kingdom

Received for publication December 31, 2006.

Accepted for publication January 8, 2007.

Correspondence: Raymond Kennedy, MRCSI, RVH Belfast, 24 Ormonde Park, Northern Ireland, United Kingdom BTlO OLS. Electronic mail may be sent to rpg.kennedy@btinternet.com.

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