WERNICKE'S ENCEPHALOPATHY PRESENTING WITH SEVERE DYSPHAGIA: A CASE REPORT

MIKAEL TRUEDSSON 0 BODIL OHLSSON 0 KLAS SJBERG 0 0 Department of Medicine, Lund University, University Hospital , S-20502 Malm, Sweden A 62-year-old man developed dysphagia 4 weeks before the classic symptoms and signs of Wernicke's encephalopathy appeared. Adequate treatment with parenteral thiamine resulted in complete recovery of all symptoms, including his dysphagia. This extraordinary presentation with dysphagia, and the unusual course of the disease, encouraged us to present this case history. INTRODUCTION Wernickes encephalopathy is caused by a deficiency of thiamine and is characterized by the triad of ophthalmoplegia, ataxia and mental confusion. Pathological lesions of Wernickes encephalopathy are characteristically distributed symmetrically in structures surrounding the third and fourth ventricles, including the mamillary body, dorsomedial thalamus, periaqueductal grey matter, anterior superior cerebellar vermis, ocular motor nuclei and vestibular nuclei (Charness, 1993). Most frequently, the encephalopathy is a consequence of alcohol misuse, but it may occur in any disease which causes sufficient malnutrition, such as gastric carcinoma and pyloric obstruction (Barrie, 1947), hyperemesis gravidarum (Chaturachinda and McGregor, 1968), prolonged parenteral feeding (Nadel and Burger, 1976) and hunger strike (Pentland and Mawdsley, 1982). Wernickes encephalopathy is curable if thiamine is administered parenterally early in the course of the disease, but it may cause neurological deficits, or, in the worst case, death, if therapy is delayed (Shikata et al., 2000). A patient who presented with dysphagia and later developed a classical Wernickes encephalopathy prompted us to present his interesting case history for discussion. A 62-year-old man was admitted to our hospital with increasing dysphagia of 3 weeks duration, a swollen neck and a 10 kg weight loss. Physical examination revealed a normal heart rate of 72 beats/min. His blood pressure was 140/90 mmHg and there was no thyroid enlargement or palpable lymph nodes. His medical history consisted of hypertension and chronic bronchitis. He had elevated levels of plasma ASAT 2.65 m kat/l (aspartate aminotransferase; normal level: <0.80 m kat/l), ALAT 2.02 m kat/l (alanine aminotransferase; normal limit <0.80 m kat/l), amylase 0.88 m kat/l (normal range: 0.200.80 m kat/l), sedimentation rate 75 mm/h (normal level <22 mm/h), serum a 1-antitrypsin 1.93 g/l (normal range: 0.971.68 g/l), serum orosomucoid 1.61 g/l (normal range: 0.541.17 g/l), serum c-reactive protein 8.9 mg/l (normal limit: <3.0 mg/l), serum ceruloplasmin *Author to whom correspondence should be addressed. 0.53 g/l (normal range: 0.220.38 g/l), serum immunoglobulin IgA 6.30 g/l (normal range: 0.703.65 g/l) and plasma creatinine 106 m M (normal range: 63105 m M), while plasma sodium 134 mM (normal range: 136146 mM) was slightly decreased. Initially, the investigations concentrated on excluding malignancy of the oesophagus or the surrounding tissue. Chest X-ray showed evidence of emphysema. Endoscopy of the oesophagus and stomach was normal. A barium meal revealed some dysfunction of the oesophagus with slightly reduced motility; an ultrasound of the liver, bile ducts and pancreas showed signs of a fatty liver. The patient was examined by an ENT physician, who could not find any explanation for the dysphagia. Because of his inability to swallow, the patient initially received a glucose infusion intravenously and after a few days an intestinal feeding tube was placed into the descending duodenum for enteral nutrition. Five days after admission, the patient developed diplopia and staggering gait. The neurological signs consisted of gait ataxia, horizontal nystagmus, lateral rectus palsy, absence of deep tendon reflexes and positive Babinskis sign on the right side. It was not until then that it was known that he had been drinking ~225 cl of 40% alcohol (i.e. 720 g) per week for the last 8 months and consequently it was suspected that the patient was suffering from Wernickes encephalopathy. The patient received two injections of thiamine (50 mg/ml, 100 mg per dose) intravenously. The time period between the two doses was 16 h. The neurological symptoms and signs, including the dysphagia, completely disappeared 24 h after the first injection. Our patient developed signs compatible with progressive Wernickes encephalopathy. The unique finding in this patient was his dysphagia, which developed 4 weeks before any other neurological symptoms occurred. We believe that dysphagia is an extremely rare symptom in this disease, and has only been briefly mentioned in two Japanese articles (Sakakibara et al., 1997; Kikuchi et al., 2000). Our patient had difficulties in initiating swallowing, suggesting an oropharyngeal dysphagia. The oropharyngeal stage of deglutition is controlled by the n. glossopharyngeus, n. vagus and n. hypoglossus. The motor nuclei of these nerves are located in the floor of the fourth M. TRUEDSSON et al. ventricle, an area that may be affected in Wernickes encephalopathy (Charness, 1993), suggesting that his dysphagia was an early manifestation of Wernickes encephalopathy. Endoscopy, a barium meal and examination by an ENT physician did not provide any alternative explanation for the dysphagia. The swollen neck was initially considered to be due to subcutaneous emphysema, but chest X-ray showed no pneumothorax, nor any mediastinal tumour. When the neurological symptoms and signs developed a few days later, the differential diagnoses considered were hypophosphataemia, central pontine myelolinolysis, MillerFishers syndrome and Wernickes encephalopathy. Alcoholism and alcohol withdrawal in combination with carbohydrate infusion after starvation are known to contribute to hypophosphataemia (Subramanian and Khardori, 2000). The neurological symptoms and signs resemble Wernickes encephalopathy, including ophthalmoplegia, ataxia, areflexia, confusion, dysphagia and muscular weakness (Zurkirchen et al., 1994; Subramanian and Khardori, 2000), but the plasma phosphate concentration in our patient was normal (1.20 mM, reference value 0.701.30 mM). Central pontine myelinolysis is a disease predominantly observed in alcoholics but also non-alcoholics with liver diseases, malnutrition, cancer, congestive heart failure, adrenal insufficiency and renal disease (Ashrafian and Davey, 2001). The most common cause is rapid correction of hyponatraemia. The symptoms are weakness in extremities, diplopia, dysarthria, changes in corticospinal reflexes, dysphagia and confusion (Charness, 1993; Ashrafian and Davey, 2001). This diagnosis was excluded by the almost normal plasma sodium concentration (134 mM, reference value 136146 mM). MillerFishers syndrome, a variant of GuillainBarrs syndrome, is characterized by ophthalmoplegia, ataxia and areflexia (Fisher, 1956), but weakness and sensory disturbances of the limbs may also occur. The syndrome is associ (...truncated)