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Wernicke's encephalopathy caused by pyloric stenosis after endoscopic submucosal dissection
At the Focal Point
Commentary FL is a non-Hodgkin lymphoma that is characteristically indolent, with a median survival of about 10 years. Indeed, many patients may not require treatment for prolonged periods of time. FL involves the small intestine most commonly and, as in this case, it may cluster around the second part of the duodenum and the ampulla of Vater. Obviously, it is important to differentiate FL from other types of lymphomas, and, of course, from benign lesions such as lymphangioma. For the latter we rely on routine histology, and for the former, immunohistochemical and molecular analysis. Whether magnification endoscopy can provide a view that is pathognomonic and can supplant these other expensive and elaborate techniques will need the test of time and many more patient evaluations. The white nature of these lesions can be explained by the lymphoid tissue and lymphatic vessels, but whether white is even a color or not is controversial and depends on whether one is answering the question from the viewpoint of a scientist or artist. Even the word white has many connotations, some good (eg, ‘‘white as the driven snow’’), some bad (the crime was ‘‘whitewashed’’). In these 2 cases, one white lesion was good (lymphangioma) and the other not good, or certainly not as good (FL). Magnification endoscopy will not tell us whether white is a color, but perhaps it can reveal to us why it is there. Lawrence J. Brandt, MD Associate Editor for Focal Points
Wernicke’s encephalopathy caused by pyloric stenosis after endoscopic submucosal dissection
A 78-year-old man underwent GI endoscopy as part of a health checkup. He had a history of diabetes mellitus, hypertension, hyperlipidemia, and myocardial infarction, and did not consume alcohol. Endoscopy showed a superficial elevated lesion that occupied three fourths of the py-
lorus circumferentially (A). The distal side of the lesion extended into the duodenum (B). Histologic analysis of the biopsy specimen revealed papillary adenocarcinoma of the stomach. We performed an endoscopic submucosal dissection (ESD), completely resecting the lesion without
1170 GASTROINTESTINAL ENDOSCOPY Volume 69, No. 6 : 2009
www.giejournal.org
At the Focal Point
complications. One week later, endoscopy revealed an ulcer extending three fourths of the way around the pylorus (C). The histopathologic diagnosis was papillary adenocarcinoma limited to the mucosa with carcinoma-free margins. Two months after ESD, anorexia developed and pyloric stenosis was documented; balloon dilatation was performed. Six months after ESD, the patient was hospitalized because of staggering gait, profound disorientation, indifference, and inattentiveness. Laboratory testing revealed anemia (hemoglobin 10.6 g/dL [normal 14.0-16.0 g/dL]), a serum thiamine value of 8 ng/mL (20-50 ng/ mL), and normal serum vitamin B12 and folate levels. The diagnosis was Wernicke’s encephalopathy. Endoscopy revealed a scarred pylorus stenosis (D). Encephalopathy was cured by administration of thiamine.
DISCLOSURE All authors disclosed no financial relationships relevant to this publication. Kyosuke Tanaka, MD, Department of Endoscopic Medicine, Mie University Hospital, Masatoshi Aoki, MD, Yasuhiko Hamada, MD, Ryo Kosaka, MD, Tomohiro Noda, MD, Department of Gastroenterology, Mie University Graduate School of Medicine, Ichiro Imoto, MD, Department of Endoscopic Medicine, Mie University Hospital, Yoshiyuki Takei, MD, Department of Gastroenterology, Mie University Graduate School of Medicine, Tsu, Japan doi:10.1016/j.gie.2008.11.023
Commentary Thiamine was discovered in 1910 by Umetro Suzuki when studying how rice bran cured beriberi. He did not know the active agent was an amine, and named it aberic acid for its therapeutic effect. Later named aneurin, short for antineuritic vitamin, its chemical composition, synthesis, and naming were reported in 1935 by Robert R. Williams, a Bell Telephone Company researcher who performed vitamin research in his spare time and also invented a process for making submarine insulation. Thiamine (vitamin B1) is a key player in the metabolism of carbohydrates and is integral to efficient energy metabolism, especially in cells with high metabolic requirements such as those in the central nervous system. Thiamine deficiency can occur as quickly as within a week of absent thiamine intake, and within a month a full-blown deficiency state can exist. A syndrome of acute mental confusion, ataxia, and ophthalmoplegia was first described by the Polish neurologist Dr Carl Wernicke, in 1881. All 3 parts of the triad are only present in about one third of cases, in part explaining why the majority of cases are unrecognized for long periods after symptom onset. A late manifestation of Wernicke’s encephalopathy with retrograde and/or anterograde amnesia and confabulation was subsequently described by Sergei Korsakoff and now is referred to by the eponym Wernicke-Korsakoff syndrome. Most often seen in alcoholics, the syndrome has been recognized to occur in many and varied disorders associated with malnutrition, after bariatric surgery, in patients on long-term hemodialysis, and those with hyperemesis gravidarum or AIDS. Pyloric stenosis is a unique precipitant, but perhaps anorexia with diminished intake was the primary culprit and the stenosis just the final insult in the etiologic sequencing. Think of thiamine deficiency whenever a patient has confusion, nystagmus, hypothermia, and gait disturbance, and in the emergency department, when you think a confused alcoholic might have hypoglycemia, be careful not to administer a bolus of concentrated glucose solution lest you precipitate acute thiamine deficiency with profound vasodilation and shock; administer intravenous thiamine first. Do not forget to eat your fortified breads, cereals, pastas, and whole grains, so you can think straight, walk tall, and see clearly. Lawrence J. Brandt, MD Associate Editor for Focal Points
www.giejournal.org
Volume 69, No. 6 : 2009 GASTROINTESTINAL ENDOSCOPY 1171
Commentary FL is a non-Hodgkin lymphoma that is characteristically indolent, with a median survival of about 10 years. Indeed, many patients may not require treatment for prolonged periods of time. FL involves the small intestine most commonly and, as in this case, it may cluster around the second part of the duodenum and the ampulla of Vater. Obviously, it is important to differentiate FL from other types of lymphomas, and, of course, from benign lesions such as lymphangioma. For the latter we rely on routine histology, and for the former, immunohistochemical and molecular analysis. Whether magnification endoscopy can provide a view that is pathognomonic and can supplant these other expensive and elaborate techniques will need the test of time and many more patient evaluations. The white nature of these lesions can be explained by the lymphoid tissue and lymphatic vessels, but whether white is even a color or not is controversial and depends on whether one is answering the question from the viewpoint of a scientist or artist. Even the word white has many connotations, some good (eg, ‘‘white as the driven snow’’), some bad (the crime was ‘‘whitewashed’’). In these 2 cases, one white lesion was good (lymphangioma) and the other not good, or certainly not as good (FL). Magnification endoscopy will not tell us whether white is a color, but perhaps it can reveal to us why it is there. Lawrence J. Brandt, MD Associate Editor for Focal Points
Wernicke’s encephalopathy caused by pyloric stenosis after endoscopic submucosal dissection
A 78-year-old man underwent GI endoscopy as part of a health checkup. He had a history of diabetes mellitus, hypertension, hyperlipidemia, and myocardial infarction, and did not consume alcohol. Endoscopy showed a superficial elevated lesion that occupied three fourths of the py-
lorus circumferentially (A). The distal side of the lesion extended into the duodenum (B). Histologic analysis of the biopsy specimen revealed papillary adenocarcinoma of the stomach. We performed an endoscopic submucosal dissection (ESD), completely resecting the lesion without
1170 GASTROINTESTINAL ENDOSCOPY Volume 69, No. 6 : 2009
www.giejournal.org
At the Focal Point
complications. One week later, endoscopy revealed an ulcer extending three fourths of the way around the pylorus (C). The histopathologic diagnosis was papillary adenocarcinoma limited to the mucosa with carcinoma-free margins. Two months after ESD, anorexia developed and pyloric stenosis was documented; balloon dilatation was performed. Six months after ESD, the patient was hospitalized because of staggering gait, profound disorientation, indifference, and inattentiveness. Laboratory testing revealed anemia (hemoglobin 10.6 g/dL [normal 14.0-16.0 g/dL]), a serum thiamine value of 8 ng/mL (20-50 ng/ mL), and normal serum vitamin B12 and folate levels. The diagnosis was Wernicke’s encephalopathy. Endoscopy revealed a scarred pylorus stenosis (D). Encephalopathy was cured by administration of thiamine.
DISCLOSURE All authors disclosed no financial relationships relevant to this publication. Kyosuke Tanaka, MD, Department of Endoscopic Medicine, Mie University Hospital, Masatoshi Aoki, MD, Yasuhiko Hamada, MD, Ryo Kosaka, MD, Tomohiro Noda, MD, Department of Gastroenterology, Mie University Graduate School of Medicine, Ichiro Imoto, MD, Department of Endoscopic Medicine, Mie University Hospital, Yoshiyuki Takei, MD, Department of Gastroenterology, Mie University Graduate School of Medicine, Tsu, Japan doi:10.1016/j.gie.2008.11.023
Commentary Thiamine was discovered in 1910 by Umetro Suzuki when studying how rice bran cured beriberi. He did not know the active agent was an amine, and named it aberic acid for its therapeutic effect. Later named aneurin, short for antineuritic vitamin, its chemical composition, synthesis, and naming were reported in 1935 by Robert R. Williams, a Bell Telephone Company researcher who performed vitamin research in his spare time and also invented a process for making submarine insulation. Thiamine (vitamin B1) is a key player in the metabolism of carbohydrates and is integral to efficient energy metabolism, especially in cells with high metabolic requirements such as those in the central nervous system. Thiamine deficiency can occur as quickly as within a week of absent thiamine intake, and within a month a full-blown deficiency state can exist. A syndrome of acute mental confusion, ataxia, and ophthalmoplegia was first described by the Polish neurologist Dr Carl Wernicke, in 1881. All 3 parts of the triad are only present in about one third of cases, in part explaining why the majority of cases are unrecognized for long periods after symptom onset. A late manifestation of Wernicke’s encephalopathy with retrograde and/or anterograde amnesia and confabulation was subsequently described by Sergei Korsakoff and now is referred to by the eponym Wernicke-Korsakoff syndrome. Most often seen in alcoholics, the syndrome has been recognized to occur in many and varied disorders associated with malnutrition, after bariatric surgery, in patients on long-term hemodialysis, and those with hyperemesis gravidarum or AIDS. Pyloric stenosis is a unique precipitant, but perhaps anorexia with diminished intake was the primary culprit and the stenosis just the final insult in the etiologic sequencing. Think of thiamine deficiency whenever a patient has confusion, nystagmus, hypothermia, and gait disturbance, and in the emergency department, when you think a confused alcoholic might have hypoglycemia, be careful not to administer a bolus of concentrated glucose solution lest you precipitate acute thiamine deficiency with profound vasodilation and shock; administer intravenous thiamine first. Do not forget to eat your fortified breads, cereals, pastas, and whole grains, so you can think straight, walk tall, and see clearly. Lawrence J. Brandt, MD Associate Editor for Focal Points
www.giejournal.org
Volume 69, No. 6 : 2009 GASTROINTESTINAL ENDOSCOPY 1171