Eur opean Rev iew for Med ical and Pharmacol ogical Sci ences 2012; 16(4 Suppl): 44-47 A case of vomiting in an anorexic achalasic patient

E. SCARPELLINI, S. CAFAROTTI* ,§ , A. CESARIO* ,§ , F. LOCOCO §, S. MARGARITORA §, M. GABRIELLI, A. TORTORA, A. GASBARRINI, P. GRANONE*

Internal Medicine Department, School of Medicine, Catholic University of the Sacred Heart, Rome, Italy and *Division of General Thoracic Surgery, School of Medicine, §IRCSS San Raffaele Pisana, Rome, Italy

Abstract. – Wernicke’s encephalopathy is a Case Presentation neurological disorder caused by thiamine (vita - A 26 year-old woman was referred to the min B1) deficiency characterized by vertigo, Emergency Department of our Hospital for the ataxia, and mental confusion. Wernicke’s en - acute worsening of a long-lasting dyspepsia with cephalopathy has a causative association with alcoholism but recently there has been an in - recurrent vomiting, advanced dehydration with creased prevalence also in other clinical condi - defeated nutritional status associated with tions. In literature potentially fatal Wernicke’s en - anorexia nervosa. The patient was previously cephalopathy onset in an advanced achalasia treated by anti-psychotic drugs both with a con - has been previously reported only once. We de - tinuative psychological support without any clin - scribe for the first time an improvement of acha - ical improvement. lasic symptoms in a young patient affected by During the diagnostic evaluation endoscopy end-stage achalasia and anorexia nervosa (com - ing from ineffective Heller-Dor myotomy) after vi - revealed an abnormal dilatation of the esophagus tamin B1 supplementation. This case report sug - with a tight stenosis at the distal third of the or - gest a potential positive impact of B1 supple - gan. No pathological intraluminal lesions were mentation on end-stage achalasic patients and observed. The endoscopic pattern was deemed to requires systematic studies to confirm this ob - be indicative of achalasia 1. Barium swallow gas - servation. tric examination confirmed the abnormal oe - Key Words: sophageal and gastric transit and showed the dis - Achalasia, Anorexia nervosa, Wernicke syndrome, tension of the oesophagus with complete aperi - Thiamine. stalsis. In addition a tight cardial stenosis was confirmed with the typical “rat’s tail” radiologi - cal features (Figure 1A). So far the patient was candidate for surgery. Before the surgical proce - Introduction dure an abdominal CT-scan was performed. The esophagus appeared extremely enlarged in all its Vomiting is one of the more common upper intra-thoracic course and in particular in the dis - gastrointestinal symptoms in the clinical practice tal pre-cardial tract (up to 8 cm from the cardia). either of central, peripheral or metabolic origin. At the level of the diaphragmatic hiatus a grind - We report of a young woman initially referring to ing “halt of caliber” was clearly detected with - our hospitals’ Emergency Department for upper out any pathological external lesions. Due to the gastro-intestinal symptoms impairment in history defeated conditions of the patient an oe - of anorexia nervosa syndrome not responding to sophageal manometric investigation was not un - the pharmacotherapy ; it was subsequently made derwent. All the routine laboratory’s tests were radiologic diagnosis of subsiding severe achala - within the normal range. sic condition ineffectively treated by surgery with According with the poor clinical status a total further worsening of the symptoms. A Wer - oesophagectomy, considered as the gold standard nicke’s syndrome was finally diagnosed by bio - for this advanced stage of achalasia, was consid - chemical and MRI imaging and successfully ered not feasible. Thus an extramucosal miotomy treated by thiamine supplementation with the of the distal esophagus (up to 4 cm from the car - consensual unexpected achalasic dismotility pat - dia) with Heller-Dor fundoplicatio was per - tern improvement . formed. No complications occurred after the pro -

44 Corresponding Author: Antonio Gasbarrini, MD; e-mail: [email protected] A case of vomiting in an anorexic achalasic patient

A B

Figure 1. Transit before (A) and after (B) the extramucosal myotomy sec. Heller-Dor and thiamine supplementation. cedure until the fifth post-operative day when the ing and neurological signs was indicative of a patient showed a progressive decay of the mental Wernicke’s syndrome (WE) diagnosis, as lately status associated with dyplopia, nystagmus, dys - also confirmed by detection of low plasma thi - metria, ataxia, deep asthenia and a loss of short- amine concentration [5 mg/dl high performance term memory over than vomiting not responding liquid chromatography (HPLC) ]2,3 . Intramuscular to metoclopramide and/or ondansetron intra - administration of thiamine chlorohydrate (300 venous administration. Respiratory (hypoxic en - mg daily for 2 weeks) together with intravenous cephalopathy) and metabolic causes were initial - nutritional support (1700 Kcal/day) were prompt - ly excluded while the neurologic status rapidly ly started with a significant clinical improvement impaired turning from a mild confusion to coma. since the second treatment day. The patient Occult post-operative septic status was also ex - restarted the oral feeding irrespective of severe cluded according to the laboratory tests and a achalasic condition , and a significantly improved new abdominal CT scan. The brain CT scan ex - oesophago-gastric motility pattern was also ob - cluded intracranial bleeding or stroke with the served at the radiographic study (Figure 1B). evidence of a bilateral thalamic hypodensity. Af - ter neurologic counseling brain MRI showed hy - per-intensity of both thalamic system and either Discussion the mamillary bodies as well as the white peri- aquaeductal matter on long TR and FLAIR im - Thiamine is crucial for several biochemical ages (Figure 2). The combination of MRI imag - processes such as intermediate carbohydrate and

45 E. Scarpellini, S. Cafarotti, A. Cesario, F. Lococo, S. Margaritora, et al.

ready been described in a patient with anorexia nervosa after laparoscopic cardiomyotomy for achalasia 6,7 . The association between achalasia and anorex - ia nervosa and/or misdiagnosis of the two disor - ders have been already described 8. Dysphagia is the initial and main clinical feature of achalasia. Other aspecific symptoms, such as vomiting and weight loss are also common. There is an usual elapse before the disease is diagnosed 6 through - out achalasia can be easily confounded with anorexia nervosa. Nevertheless differential diag - nosis between achalasia and anorexia nervosa is not always obvious as long oesophageal motor disorders are common in patients with a primary diagnosis of anorexia nervosa 9. For example pa - tients with eating disorders frequently have gas - tric emptying abnormalities causing bloating, postprandial fullness, and vomiting. These symp - toms usually improve with refeeding but some - times prokinetics administration may be neces - sary. In addition wilful avoidance of food and spontaneous or self-induced vomiting have been Figure 2. Brain MRI long TR and FLAIR images show - reported in patients with echolalia 10,11 . Thus , ing hyper-intensity of both thalamic system and either the mamillary bodies as well as the white peri-aqueductal many gastrointestinal diseases may present like 12 matter. eating disorders. Rosenzweig et al consider this misdiagnosis to be related to delay in obtaining appropriate investigations or misinterpretation of lipid metabolism over than amino acids and glu - their results. Other authors suggest that a careful cose-derived neurotransmitters synthesis. Any clinical history can localize gastrointestinal condition of unbalanced nutritional status lasting motility disorders together with appropriate diag - for 2-3 weeks can lead to thiamine depletion and nostic tests use 13 according to the recognition of brain lesions usually located in vulnerable re - two main groups of symptoms. First, dysphagia, gions with high thiamine content and turnover odynophagia, heartburn, and reflux have oe - (e.g. diencephalic and brainstem areas )2. WE is a sophageal origins and occur in achalasia. In this medical emergency thus thiamine supportive case the appropriate tests are barium-swallow en - therapy should be immediately initiated in order doscopy and oesophageal manometry and/or to prevent irreversible brain damages invariably scintigraphy. The second group of symptoms in - leading to death (acute mortality rate of about cludes nausea, vomiting, anorexia, bloating, and 20%) or to the chronic form of the encephalopa - abdominal pain, all symptoms of motility disor - thy (Korsakoff ’s syndrome) in up to 85% of sur - ders of the stomach and small intestine . vivors 2. The presumptive diagnosis of WE can be So far evidences from the literature do not in - confirmed by assessing the thiamine status by the clude a clear causal relationship between achala - direct measurement in whole blood or by per - sia, anorexia, malnutrition and consequent vita - forming the erythrocyte transketolase activation min deficiency. test although these measurements are limited by The clinical case above reported support for low specificity and technical difficulty 3,4 . MRI is the first time the possible relationship between currently considered the most valuable method to Wernicke syndrome due to chronic vitamin defi - confirm the WE diagnosis 2. Gastrointestinal ciency and “nervosa/achalasic” anorexia as com - causes of WE include: peptic ulcer, acute pancre - mon cause of vomiting in achalasic patients. atitis, oesophageal metastasis, and gastric or oe - Paradoxically an impaired achalasia may be both sophageal carcinoma 5. A combination of ophthal - a “clinical sign ” of vitamin deficiency due to an moplegia, nystagmus, and ataxia, considered to organic and nervosa anorexia and a cause of it reflect a Wernicke-Korsakoff syndrome, has al - turning all together in these pathophysiologic vi -

46 A case of vomiting in an anorexic achalasic patient cious circle. In fact the non-functional recovery 5) HANDLER CE, P ERKIN GD . Anorexia nervosa and after surgical oesophageal myotomy of the dys - Wernicke’s encephalopathy: an underdiagnosed phagia above reported asked for further and sys - association. Lancet 1982; 2: 771-772. tematic investigation about metabolic conditions 6) DUANE PD, M AGEE TM, A LEXANDER MS, H EATLEY RV, LOSOWSKY MS . Oesophageal achalasia in adoles - possibly underlying the dyspepsia. cent women mistaken for anorexia nervosa. Br In conclusion, we can be consequence and Med J 1992; 305: 43. cause of advanced status achalasia and anorexia 7) STACHER G, K ISS A, W IESNAGROTZKI S, B ERGMANN H, nervosa. Vitamin B1 supplementation could rep - HÖBART J, S CHNEIDER C. Oesophageal and gastric resent an “add-on” future source for the treat - motility disorders in patients categorised as hav - ment of advanced achalasia together with the ing primary anorexia nervosa. Gut 1986; 27: surgery staying the gold standard if this observa - 1120-1126. tion will be supported by systematic studies. 8) KENNEDY R, H UNT S, A HMAD J, M ENEZES C, C LEMENTS WB, K ENNEDY JA . Wernicke’s encephalopathy after laparoscopic cardiomyotomy for achalasia. J Par - enter Enteral Nutr 2007; 31: 324-325. References 9) DÄBRITZ J, D OMAGK D, M ONNINGER M, F OELL D. Achalasia mistaken as eating disorders: report of 1) SPECHLER SJ, C ASTELL DO . Classification of oe - two children and review of the literature. Eur J sophageal motility abnormalities. Gut 2001; 49: Gastroenterol Hepatol 2010; 22: 775-778. 145-151. 10) MCCLAIN CJ, H UMPHRIES LL, H ILL KK . Gastrointestinal 2) SECHI GP, S ERRA A. Wernicke’s encephalopathy: new and nutritional aspects of eating disorders. J Am clinical settings and recent advances in diagnosis Coll Nutr 1993; 12: 466-474. and management. Lancet Neurol 2007; 6: 442- 455. 11) STACHER G, W IESNAGROTZKI S, K ISS A. Symptoms of 3) TALWAR D, D AVIDSON H, C OONEY J, JO’R EILLY D. Vita - achalasia in young women mistaken as indicating min B1 Status assessed by direct measurement primary anorexia nervosa. Dysphagia 1990; 5: of thiamin pyrophosphate in erythrocytes or whole 216-219. blood by HPLC: comparison with erythrocyte 12) PRIOR AJ . Oesophageal achalasia mistaken for transketolase activation assay. Clin Chem 2000; anorexia nervosa. Br Med J 1992; 305: 833-834. 46: 704-710. 13) ABELL TL, W ERKMAN RF. Gastrointestinal motility 4) EBELS EJ . How common is Wernicke-Korsakoff syn - disorders. Am Fam Physician 1996; 53: 895- drome? Lancet 1978; 2: 781-782. 902.

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