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Show ]. Clin. Neuro-ophth.1lmol. 1: 195-198, 198!. Wernicke's Disease Complicating Surgical Therapy for Morbid Obesity JOHN F. ROTHROCK, M.D. MICHAEL S. SMITH, M.D. Abstract A case of Wernicke's disease complicating surgical therapy for morbid obesity is presented. Features emphasized include the occurrence of the condition in a nonalcoholic patient and the persistence of neurological signs and symptoms despite prompt therapy. Prophylactic treatment in situations of potential thiamine depletion is essential if iatrogenic Wernicke's disease is to be avoided. Wernicke's disease is a common neurological disorder resulting from thiamine deficiency. It is chiefly characterized by nystagmus, ophthalmoplegia, gait ataxia, and mental confusion, occurring singly or in any combination. Despite a general awareness within the medical community of the disease and its relationship to thiamine depletion, contain misconceptions regarding the condition persist. First, although the disease is certainly prevelant in the alcoholic population, especially in its chronic form, it is definitely not limited to that population and may occur in a wide variety of other clinical settings. The diagnosis may not be entertained in the nonalcoholic patient with acute Wernicke's disease, and consequent delays in appropriate therapy can prove disasterous. Second, once the disease manifests itself neurologICally, even prompt initiation of thiamine therapy does not invariably result in total remission of the associated signs and symptoms; incapacitating neurological sequelae frequently occur. The following case report demonstrates the above features and further underscores a need for greater physician awareness of the dangers inherent in iatrogenic thiamine depletion. Case Report A 33-year-old female underwent a gastric ~tapiing procedure for morbid obesity. She was dOing well, on a liquid diet of tea, coffee, and water only, From the Department of Neurology, University of Arizona Health Sciences Center, Tucson, Arizona. September 1981 until 1 month postoperatively, when she developed persistent vomiting. This continued over 3 weeks and she was consequently readmitted. After 6 days of receiving intravenous 5% dextrose in water and 0.2% saline she underwent laparotomy and lysIs of an adhesion which was felt to cause her gastric outlet obstruction. Twenty-four hours after surgery, she began to complain of "unsteadiness" and diplopia. The neurology service was consulted the following day. On examination, the patient was awake and fully oriented, but somewhat lethargic; there was no significant impairment of retentive memory. Mild bilateral ptosis was noted, and there was a nght esotropia in the primary position (Fig. 1.1). VolJtional gaze right revealed no abduction and minImal adduction (Fig. Ib). Volitional gaze left revealed minimal abduction and adduction (Fig. Ie). No horizontal nystagmus was present, either spontaneously or with attempted lateral gaze. Gaze upwards was moderately restricted with associated marked vertical nystagmus (fast phase directed superiorly) (Fig. Id). Gaze down was mildly restricted and associated with less pronounced vertical nystagmus (fast phase directed inferiorly). The remainder of the cranial nerves, including pupils and fundi, were normal. There was mild diffuse arm weakness bilaterally, but the legs were strong. Deep tendon reflexes were moderately depressed throughout, and plantar responses were flexor. Heel-to-shin and finger-to-nose performance was fair, but the patient's gait was wide-based, shuffling and unsteady, with staggering to both sides and falling. Lumbar puncture yielded no abnormalities. Ten milligrams of intravenous edrophonium chloride were administered, without significant subjective or objective change. One hundred milligrams of thiamine hydrochloride was then given intravenously and intramuscularly. At reexamination 9 hours later, lateral gaze, though obviously still impaired, was much improved; prominent bilateral horizontal jerk nystagmus had developed in addi- •tion to persistent vertical nystagmus. After 10 days of thiamine therapy, no restriction of gaze was apparent. The patient was quite alert and no longer exhibited ptosis. She continued to display obvious 195 Wernicke's Disease (a) (b) (e) (d) Figure 1. Extraocular movements in the patient presented prior to initial administration of thiamine. (a) Patient with eyes in primary position. Bilateral ptosis is evident. (b) Patient attempting gaze right. (c) Patient attempting gaze left. (d) Patient attempting upward gaze. vertical nystagmus in the primary position, exacerbated by volitional vertical gaze. A fine horizontal jerk nystagmus persisted with lateral gaze bilaterally. Her gait, although improved, remained quite ataxic, and she complained of frequent oscillopsia. A CT scan done 2 months after the onset of symptoms was normal. After 6 months, she continues to require a cane for ambulation. Discussion Vitamin Bl (thiamine) is an essential water-soluble vitamin which, in its phosphorylated form, acts as a coenzyme in intermediary carbohydrate metabolism, In some as yet unknown way, its deficiency in Wernicke's disease leads to neuronal dysfunction and even frank tissue destruction in peculiarly specific areas of the central nervous system (Fig. 2). In our society, Wernicke's diseasp is primarily f"tlfld in the alcoholic population as a cons€'quence 190 of deficient essential vitamin intake and alcohol's contribution of carbohydrate calories. I Nonalcoholic patients, however, are not immune to thiamine deficiency and Wernicke's disease. One of Wernicke's first case descriptions involved a young woman with pyloric stenosis and intractable vomiting secondary to sulfuric acid poisoning.2 Since then, the disease has been described in association with a variety of conditions, including hyperemesis gravidarum, gastric carcinoma and chronic gastritis, 4.;, and chronic hemodialysis,6 Nadel and Burger reported two cases occurring secondary to prolonged intravenous therapy,7 and it has been noted as a consequence of starvation therapy for obesity,8 Even more pertinent to the present example, Wernicke's disease following the gastric-stapling procedure for obesity has been reported elsewhere.9 In the past, investigators have maintained that the thiamine requirement, normally about 1 mg daily in the healthy adult, diminishes with de- Journal of Clinical Neuro-ophthalmology Rothrock, Smith (aJ creased caloric intake; I" hypothetically, they felt, the starving individual was able to satisfy a reduced tissue need for thiamine from endogenous stores as the body turned from carbohydrate metabolism to fat and amino acids for its calories. Drenick et aI., however, have presented urinary excretion data from starvation diet studies suggesting that endogenous thiamine stores do not suffice despite decreased carbohydrate metabolism.H The clinical occurrence of Wernicke's disease in starving patients obviously supports his conclusion. Moreover, endogenous stores are soon exhausted. Ziporin et al. have shown that total body thiamine depletion can occur within 18 days following dietary restriction in healthy adults. 1O Administration of intravenous or oral glucose without thiamine supplementation, an unfortunately common occurrence in many thiamine-deficient patients who are hospitalized, will further hasten depletion by using up remaining stores. I) An acute Wernicke's syndrome may result. Rapid improvement in gaze paresis following administration of parenteral thiamine is practically a sine qua non for the diagnosis of acute Wernicke's disease; failure to improve should cast great doubt upon the diagnosis.) Although our patient's oculomotor problems did improve rapidly and dramatically with therapy, she continued to have a significant neurological deficit at least 6 months later. In their extensive series, Victor et al. found September 1981 (b) Figure 2. Postmort('m sp('ciml'ns from oth('r pJti('nts showing dr('as typically involv('d in W('rnid.("s dis('Js('. (d) Ar('as of petl'chial h('morrhag(' dr(' ('vid('nt in th(' parJv('ntricular (III) r('gion. Th(' pontin(' t('gm('ntum and th(' floor of V('ntric1e IV. ( b) Hl'morrhJg(' within the mJmillary body, the most common area of involvement. 197 Wernicke's Disease that recovery from oculomotor palsies began early, often within hours following initial thiamine administration, and that recovery tended to be complete, except for a residual fine horizontal nystagmus (in 60% of their patients).1 Recovery from ataxia was relatively delayed, and in 62% recovery was incomplete or absent entirely. Recovery from acute mental signs tended to begin within 2 weeks; however, in many of their patients, the global confusional state observed initially gave way to a chronic impairment of retentive memory and learning (i.e., Korsakoff's psychosis). Of a total of 245 patients studied, 26 died during the acute phase of the iIIness. 1 As our case clearly demonstrates, thiamine deficiency, regardless of its cause, can lead to significant and long-lasting deficit. Once neurological signs and symptoms are present, prompt administration of thiamine does not preclude the patient's suffering significant, and even permanent, morbidity. It is therefore mandatory that physicians identify situations of potential thiamine deficiency before depletion occurs and accordingly begin appropriate replacement. The best treatment for Wernicke's disease is prophylaxis; a basic awareness and understanding of the condition will aid in preventing further iatrogenic cases. References 1. Victor, M., Adams, R., Collins, G.: The WernickeKorsakoff syndrome: A clinical and pathological study of 245 patients, 82 with post-mortem examinations. In Contemporary Neurology Series (7th ed.). F. A Davis, Philadelphia, 1971, pp. 1-206. 2. Wernicke, C: Lehrbuck der Gehirnkrankheiten fur Aerzte und Studirende. Vol. 2. Theodore Fischer, Kassel, 1881, pp. 229-242. 3. Sheehan, H.: Discussion of the neurological compli- 1915 cations of pregnancy. Proc. R. Soc. Med. 32: 584588, 1939. 4. Neuburger, K.: Uber die nichtalkoholische Wernickesche Krankheit, inbesondere uber ihr Vorkommen beim Krebsleiden. Virchows Arch. Pathol. Anat. 298: 68-86, 1937. 5. Neuburger, K.: Wernickesche Krankheit bei chronischer Gastritis. Ein Beitrag zu den Beziehungen zwischen Magen und Gehirn. Z. Ges. Neurol. Psychiatric 160: 208-225, 1937. 6. Lopez, R., and Collins, G.: Wernicke's encephalopathy. A complication of chronic hemodialysis. Arch. Neurol. 18: 248-259, 1968. 7. Nadel, A, and Burger, P.: Wernicke encephalopathy following prolonged intravenous therapy. ].AM.A. 235: 2403-2405, 1976. 8. Drenick, E., Joven, C, et al.: Occurrence of acute Wernicke's encephalopathy during prolonged starvation for the treatment of obesity. N. Engl.]. Med. 274: 937-939, 1966. 9. Cox, T., Corbett, Let a!.: Upbeat nystagmus changing to downbeat nystagmus with convergence. Neurology (in press). 10. Ziporin, Y, Nunes, W., et a!.: Thiamine requirement in the adult human as measured by urinary excretion of thiamine metabolites. ]. Nutr. 85: 297-304, 1965. 11. Phillips, G., Victor, M., Adams, R., et al.: A study of the nutritional defect in Wernicke's syndrome: the effect of a purified diet, thiamine and other vitamins on the clinical manifestations. ]. Clin. Invest. 31: 859-871, 1952. Acknowledgment Special thanks are due Dr. Peter C Johnson, Associate Professor of Pathology, Chief of Neuropathology, for his help in compiling this manuscript. Wite for reprints to: John F. Rothrock, M.D., Department of Neurology, Arizona Health Sciences Center, 1501 N. Campbell Avenue, Tucson, Arizona 85724. Journal of Clinical Neuro-ophthalmology |
