Coexisting cytotoxic and vasogenic edema in Wernicke encephalopathy

Abstract

Wernicke encephalopathy (WE) is caused by a thiamine deficiency and typically results in symmetrical lesions in areas of the brain including the paraventricular regions of the thalamus and hypothalamus, the mammillary bodies, the periaqueductal region of the midbrain, the floor of the fourth ventricle and the superior cerebellar vermis. We observed a case of WE that exhibited simultaneous cytotoxic and vasogenic edema. Although cytotoxic edema and vasogenic edema are typical findings of Wernicke encephalopathy, it is very rare for both pathogenic findings to be observed together. A 52-year-old woman with a history of total colectomy was admitted for management of epigastric discomfort, nausea and vomiting 7 days in duration. The patient suffered from recurrent paralytic ileus due to systemic sclerosis. Three months prior to admission, the patient underwent a total colectomy due to colon obstruction. The patient had no prior history of hypertension, diabetes, or cerebrovascular diseases. She did not have a history of smoking or alcohol consumption (Fig. 1). Despite administration of antiemetics, vomiting persisted. The patient was treated with intravenous glucose and electrolyte solution without vitamin supplementation. After 15 days of hospitalization, the patient was referred to the Neurology Department for evaluation of convulsive movement, diplopia, and ataxia. On examination, the patient was alert, but disoriented. There was complete ophthalmoplegia with coarse spontaneous nystagmus. The upper and lower limbs were symmetrically hypotonic and weak. The patient was unable to maintain a sitting position or standing position without support due to truncal ataxia, and deep tendon reflexes were slightly depressed. Laboratory studies revealed anemia, leukocytosis and elevated liver enzymes. The serum thiamine level was below the normal range (14.5 lg/L, normal 28–85). Magnetic resonance imaging (MRI) performed on the referral day showed high signal intensity lesions in both medial thalami, the mammillary bodies, the periaqueductal region, the tectum of the midbrain and the medullary tegmentum in T2-weighted and fluid-attenuated inversion recovery (FLAIR) MR imaging. Diffusion-weighted images (DWI) showed more extensive symmetrical high signal intensities in the aforementioned areas, and also in both the central and precentral sulcus. Apparent diffusion coefficient (ADC) mapping showed low intensity areas in both cortical sulcal lesions, the most medial part of the thalami, the mammillary bodies, and the dorsal and upper part of the midbrain tectum, and simultaneous high or iso intensities in the rest of the medial thalamus, midbrain tectum and medullary tegmentum. The patient was given 100 mg of thiamine per day intravenously. Seven days after thiamine supplementation, her ocular motor function had improved, and 21 days after thiamine treatment began, the patient was able to walk without titubation. Many case investigations using DWI to examine patients with Wernicke encephalopathy have S. Kim S.-H. Kim J.-S. Kim (&) Department of Neurology, College of Medicine, Seoul St. Mary’s Hospital, The Catholic University of Korea, 505, Banpo-dong, Seocho-gu, Seoul 137-701, South Korea e-mail: neuronet@catholic.ac.kr