Neurol Sci (2008) 29:45–46 DOI 10.1007/s10072-008-0859-8
C A S E R E P O RT
Severe dysphagia as the presenting symptom of Wernicke-Korsakoff syndrome in a non-alcoholic man Ilias Karaiskos • Ioannis Katsarolis • Leonidas Stefanis
Received: 20 October 2007 / Accepted: 18 January 2008 © Springer-Verlag 2008
Abstract We present the case of a non-alcoholic man, who, following severe malnutrition, presented with dysphagia that necessitated gastrostomy tube placement. The patient subsequently developed encephalopathy, at which point thiamine deficiency was suspected and thiamine supplementation initiated. The encephalopathy and the dysphagia resolved, but the patient was left with a dense amnestic deficit consistent with Korsakoff syndrome. MRI at the time of the encephalopathy revealed lesions consistent with Wernicke-Korsakoff syndrome. This case represents a remarkable example of Wernicke-Korsakoff syndrome that for a prolonged time period had as its sole manifestation severe dysphagia. To our knowledge, there is only one similar case reported in the literature. This case serves to alert neurologists that isolated dysphagia may be the presenting symptom of this classic neurological syndrome even in the absence of alcoholism. Keywords Encephalopathy · Malnutrition · Amnesia · Ophthalmoplegia · Parenteral nutrition
I. Karaiskos · I. Katsarolis Fourth Department of Internal Medicine University of Athens Medical School Hospital “Attikon” Attiki, Greece L. Stefanis (쾷) Second Department of Neurology University of Athens Medical School Hospital “Attikon” 1, Rimini Street Haidari, Attiki 12464, Greece e-mail:
[email protected]
A 44-year-old Palestinian man, living in Greece for 3 years, was admitted after a fall. On admission, he was febrile; he acknowledged dysphagia of 10 days’ duration and weight loss. He had been working long hours in construction, and his fellow workers stated that he had been living on “coffee and cigarettes”, apparently in an effort to save money for his family, living in Romania. He had also been fasting due to Ramadan. There was no recent alcohol abuse. Chest CT revealed bilateral pleural effusions and a pericardial effusion. Endoscopy of oesophagus and stomach and barium meal were normal. The effusions were drained and classified as exudates. Intravenous antibiotics were administered and the patient became afebrile. Dysarthria was appreciated at this point and dysphagia worsened considerably, to the point that a rhinogastric and, subsequently, a gastrostomy feeding tube were placed. One month after admission, he appeared confused and disoriented. Brain MRI, of poor quality due to movement artefact, was read as normal. However, subsequent evaluation revealed symmetric high signal intensities in the medial thalami on T2 and FLAIR sequences (Fig. 1). Similar symmetric lesions also appeared in the periaqueductal grey matter and in the floor of the fourth ventricle. The patient was transferred to Attikon University Hospital for further investigation 5 days later. He was afebrile, but severely malnourished. He had his eyes open, was groaning and was non-communicative. He only withdrew partially to painful stimuli. Due to his condition, extraocular movements could not be assessed. CSF was entirely normal, including negative PCR for tuberculosis. Because of the presence of at least one brainstem sign (dysphagia) and the unexplained encephalopathy, Wernicke encephalopathy due to thiamine deficiency was suspected. IV thiamine (100 mg/day) was initiated. The
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Fig. 1 A transverse section of a FLAIR sequence of a brain MRI obtained at the time of onset of the encephalopathy. The bilateral, symmetric high signal intensity in the medial thalami is depicted by arrows
following day, the patient was alert, following simple commands. There were bilateral abducens nerve palsies, with coarse horizontal and vertical upbeat nystagmus. The patient’s condition continued to improve over the next 4 days, and he started eating and walking. Mild ataxia was noted, but this also was improving rapidly. Due to continued good oral intake, the gastrostomy tube was removed 8 days after initiation of thiamine supplementation. During this recovery period, the patient, although by now completely alert and conversant, manifested severe anterograde and retrograde memory problems. He insisted that he was in Romania, where he had resided in the past, despite being repeatedly told that he was in Greece. He had no knowledge of conversations with his family a few minutes after the fact. Formal testing revealed a sMMS score of 14. A repeat MRI was normal, without high-intensity lesions on FLAIR, however volumetric studies of the third ventricle or grey matter structures were not performed. The patient was discharged to the care of his family two months after his first admission. Immediately prior to his discharge, his mental status was unchanged. This case represents a remarkable example of Wernicke-Korsakoff syndrome that for a prolonged time period had as its sole manifestation severe dysphagia. The classic triad of ophthalmoplegia, ataxia and
Neurol Sci (2008) 29:45–46
encephalopathy developed much later. Dysphagia was obviously part of the syndrome, as it promptly resolved following thiamine supplementation. The presence of pleural and pericardial effusions represented a “red herring” that led to the assumption of a systemic infectious process. These effusions were likely the result of aspiration secondary to dysphagia. Although initially unappreciated, brain MRI findings were typical of the WernickeKorsakoff syndrome [1]. To our knowledge, this is the second reported case with dysphagia as the presenting symptom of WernickeKorsakoff syndrome. Truedsson et al. reported the case of an alcoholic man who presented with dysphagia and developed the typical syndrome only weeks later [2]. Our case demonstrates that Wernicke-Korsakoff syndrome may present in a similar atypical fashion due to severe malnutrition in the absence of alcoholism. In their classic monograph, Victor et al. [3] did not report dysphagia as a clinical symptom of the syndrome. Subsequent studies have identified dysphagia within the clinical spectrum of Wernicke-Korsakoff syndrome [4, 5], but not, as in our case, as a primary manifestation. Dysphagia in WernickeKorsakoff may be due to lesions of medullary regions around the floor of the fourth ventricle, which were noted on pathology in certain cases by Victor et al. [3], and on MRI imaging in our case. Dysphagia may be a particularly devastating manifestation of this syndrome, as it may lead to further lack of oral intake and propagation of the disease process, especially if enteral or parenteral nutrition is established and no provision is made for thiamine supplementation.
References 1. Cirignotta F, Manconi M, Mondini S et al (2000) WernickeKorsakoff encephalopathy and polyneuropathy after gastroplasty for morbid obesity: report of a case. Arch Neurol 57:1356–1359 2. Truedsson M, Ohlsson B, Sjoberg K (2002) Wernicke’s encephalopathy presenting with severe dysphagia: a case report. Alcohol Alcoholism 37:295–296 3. Victor M, Adams RD, Collins GH (1989) The WernickeKorsakoff syndrome, 2nd Edn. FA Davis Co., Philadelphia 4. Sakakibara R, Hattori T, Yasuda K et al (1997) Micturitional disturbance in Wernicke’s encephalopathy. Neurourol Urodyn 16:111–115 5. Kikuchi A, Chida K, Misu T et al (2000) A case of WernickeKorsakoff syndrome with dramatic improvement in consciousness immediately after intravenous infusion of thiamine. No To Shinkei 52:59–63
