Wernicke encephalopathy: late-stage symptoms
Abstract
Introduction: Wernicke's encephalopathy is a rare acute/subacute neurological disorder, commonly caused by prolonged thiamine deficiency in patients who chronically consume alcohol. According to the Caine classification criteria, the clinical diagnosis of this encephalopathy involves at least two of the following four signs: malnutrition, oculomotor dysfunction, ataxia, and changes in mental status. This case report highlights rare clinical signs in the late stage of the disease, as well as the consequences of possible local hypoperfusion of the brainstem in the form of an ischemic vascular event.
Case presentation: A 39-year-old female patient (previously treated at a regional general hospital) was admitted to the Department of emergency neurology at the University clinical center of Vojvodina with a history of a series of epileptic seizures, altered consciousness, oculomotor signs, opisthotonus, and cognitive dysfunction, following years of alcohol consumption and nutritional deficiency. The diagnosis was confirmed by typical neuroimaging findings and specific laboratory tests. Opisthotonus/hypertonia was an unexpected clinical manifestation of Wernicke's encephalopathy, as well as the occurrence of an ischemic stroke. Empirical administration of high-dose thiamine, along with additional supportive intensive therapy, did not yield satisfactory outcomes.
Conclusion: Wernicke's encephalopathy represents a clinical diagnosis based on physical and neurological examination, with neuroimaging. Early recognition of both common and unusual symptoms, particularly in the late stage of the disease, could potentially reduce morbidity and mortality. It is essential to administer thiamine before glucose infusion to all patients with an undetermined cause of altered consciousness.
References
Donnino MW, Vega J, Miller J, Walsh M. Myths and misconceptions of Wernicke's encephalopathy: what every emergency physician should know. Ann Emerg Med 2007; 50(6): 715-21.
Sechi G, Serra A. Wernicke’s encephalopathy: new clinical settings and recent advances in diagnosis and management. Lancet Neurol 2007; 6: 442-55.
Zuccoli G, Santa Cruz D, Bertolini M, Rovira A, Gallucci M, Carollo C, et al. MR imaging findings in 56 patients with Wernicke encephalopathy: nonalcoholics may differ from alcoholics. Am J Neuroradiol 2009; 30(1): 171-6.
Caine D, Halliday GM, Kril JJ, Harper CG. Operational criteria for the classification of chronic alcoholics: identification of Wernicke's encephalopathy. J Neurol Neurosurg Psychiatry 1997; 62(1): 51-60.
Koguchi K, Nakatsuji Y, Abe K, Sakoda S. Wernicke's encephalopathy after glucose infusion. Neurology 2004; 62(3): 512.
Murata T, Fujito T, Kimura H, Omori M, Itoh H, Wada Y. Serial MRI and 1H-MRS of Wernicke's encephalopathy: report of a case with remarkable cerebellar lesions on MRI. Psychiatry Research: Neuroimaging 2001; 108(1): 49-55.
Schabelman E, Kuo D. Glucose before thiamine for Wernicke encephalopathy: a literature review. J Emerg Med 2012; 42(4): 488-94.
Zhang SX, Weilersbacher GS, Henderson SW, Corso T, Olney JW, Langlais PJ. Excitotoxic cytopathology, progression, and reversibility of thiamine deficiency-induced diencephalic lesions. J Neuropathol Exp Neurol 1995; 54: 255-67.
Takakusaki K, Chiba R, Nozu T, Okumura T. Brainstem control of locomotion and muscle tone with special reference to the role of the mesopontine tegmentum and medullary reticulospinal systems. J Neural Transm 2016; 123(7): 695-729.
Vetreno RP, Ramos RL, Anzalone S, Savage LM. Brain and behavioral pathology in an animal model of Wernicke's encephalopathy and Wernicke-Korsakoff Syndrome. Brain Res 2012; 1436: 178-92.
Sullivan EV, Pfefferbaum A. Neuroimaging of the Wernicke-Korsakoff syndrome. Alcohol and Alcoholism 2009; 44(2): 155-65.
Sinha S, Kataria A, Kolla BP, Thusius N, Loukianova LL. Wernicke Encephalopathy-Clinical Pearls. Mayo Clin Proc 2019; 94(6): 1065-72.
Harper C, Butterworth R. Nutritional and metabolic disorders. In: Graham D, Lantos PL, eds. Greenfield’s Neuropatholog. Vol 1. 6th ed. London, UK: Hodder Arnold;1997. p. 601-52.
Karhunen PJ, Erkinjuntti T, Laippala P. Moderate alcohol consumption and loss of cerebellar Purkinje cells. BMJ 1994; 308(6945): 1663-7.
Oka M, Terae S, Kobayashi R, Kudoh K, Chu B, Kaneko K, et al. Diffusion‐weighted MR findings in a reversible case of acute Wernicke encephalopathy. Acta Neurologica Scandinavica 2001; 104(3): 178-81.
Sullivan EV, Pfefferbaum A. Magnetic resonance relaxometry reveals central pontine abnormalities in clinically asymptomatic alcoholic men. Alcohol Clin Exp Res 2001; 25: 1206-212.
