Neurol Sci DOI 10.1007/s10072-014-1639-2
LETTER TO THE EDITOR
Coexisting cytotoxic and vasogenic edema in Wernicke encephalopathy Seungyoo Kim • Seong-Hoon Kim • Joong-Seok Kim
Received: 28 August 2013 / Accepted: 15 January 2014 Ó Springer-Verlag Italia 2014
Keywords Wernicke encephalopathy Cytotoxic Vasogenic Edema
Dear Editor, 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. 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: [email protected]
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
123
Neurol Sci
Fig. 1 Magnetic resonance images from the day of onset of neurologic symptoms. a Extensive symmetrical high signal intensity lesions in the central and precentral sulcus, the medial thalami, the mammillary bodies, the periaqueductal region, the tectum of midbrain and the medullary tegmentum in axial diffusion-weighted images. b Axial apparent diffusion coefficient (ADC) mapping showed low
intensity areas in both cortical sulcul lesions, the most medial part of thalami, the mammillary bodies and the most dorsal and upper part of the midbrain tectum. At the same time, the ADC map showed slightly high or iso-signal intensity in the rest of the medial thalamus, midbrain tectum and medullary tegmentum
demonstrated typical neuroimaging findings of variable stages of cytotoxic or vasogenic edema [1]. Some studies have suggested that cytotoxic edema is converted to vasogenic edema as the disease progresses. Thiamine deficiency can firstly disturb thiamine-related cellular energy metabolism and osmotic gradient, and can result in cellular injuries recognized as cytotoxic edema. As this process continues, blood–brain barrier breakdown and intravascular fluid penetration to the cerebral parenchymal extracellular space may induce vasogenic edema [1]; however, this simple pathophysiologic sequence of WE has rarely been reported in humans [2]. Moreover, there have been observations indicating that vasogenic edema is not a secondary process of cytotoxic edema [3, 4], and some studies suggested that these two different pathologies occur independently [5, 6]. To our knowledge, there have been relatively few published reports of WE demonstrating concomitant vasogenic and cytotoxic edema. The presented case adds to this small number of recorded observations.
Conflict of interest disclose.
123
The authors have no conflicts of interest to
References 1. Jung YC, Chanraud S, Sullivan EV (2012) Neuroimaging of Wernicke’s encephalopathy and Korsakoff’s syndrome. Neuropsychol Rev 22(2):170–180 2. Ueda Y, Utsunomiya H, Fujii A, Kanaumi T, Yasumoto S (2007) Wernicke encephalopathy in a chronic peritoneal dialysis patient– correlation between diffusion MR and pathological findings. No To Hattats 39(3):210–213 3. Liu YT, Fuh JL, Lirng JF, Li AF, Ho DM, Wang SJ (2006) Correlation of magnetic resonance images with neuropathology in acute Wernicke’s encephalopathy. Clin Neurol Neurosurg 108(7):682–687 4. Hong KS, Kang DW, Cho YJ, Hwang YJ, Hur G (2002) Diffusionweighted magnetic resonance imaging in Wernicke’s encephalopathy. Acta Neurol Scand 105(2):132–134 5. Calingasan NY, Baker H, Sheu KF, Gibson GE (1995) Blood-brain barrier abnormalities in vulnerable brain regions during thiamine deficiency. Exp Neurol 134(1):64–72 6. Harata N, Iwasaki Y (1995) Evidence for early blood–brain barrier breakdown in experimental thiamine deficiency in the mouse. Metab Brain Dis 10(2):159–174
LETTER TO THE EDITOR
Coexisting cytotoxic and vasogenic edema in Wernicke encephalopathy Seungyoo Kim • Seong-Hoon Kim • Joong-Seok Kim
Received: 28 August 2013 / Accepted: 15 January 2014 Ó Springer-Verlag Italia 2014
Keywords Wernicke encephalopathy Cytotoxic Vasogenic Edema
Dear Editor, 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. 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: [email protected]
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
123
Neurol Sci
Fig. 1 Magnetic resonance images from the day of onset of neurologic symptoms. a Extensive symmetrical high signal intensity lesions in the central and precentral sulcus, the medial thalami, the mammillary bodies, the periaqueductal region, the tectum of midbrain and the medullary tegmentum in axial diffusion-weighted images. b Axial apparent diffusion coefficient (ADC) mapping showed low
intensity areas in both cortical sulcul lesions, the most medial part of thalami, the mammillary bodies and the most dorsal and upper part of the midbrain tectum. At the same time, the ADC map showed slightly high or iso-signal intensity in the rest of the medial thalamus, midbrain tectum and medullary tegmentum
demonstrated typical neuroimaging findings of variable stages of cytotoxic or vasogenic edema [1]. Some studies have suggested that cytotoxic edema is converted to vasogenic edema as the disease progresses. Thiamine deficiency can firstly disturb thiamine-related cellular energy metabolism and osmotic gradient, and can result in cellular injuries recognized as cytotoxic edema. As this process continues, blood–brain barrier breakdown and intravascular fluid penetration to the cerebral parenchymal extracellular space may induce vasogenic edema [1]; however, this simple pathophysiologic sequence of WE has rarely been reported in humans [2]. Moreover, there have been observations indicating that vasogenic edema is not a secondary process of cytotoxic edema [3, 4], and some studies suggested that these two different pathologies occur independently [5, 6]. To our knowledge, there have been relatively few published reports of WE demonstrating concomitant vasogenic and cytotoxic edema. The presented case adds to this small number of recorded observations.
Conflict of interest disclose.
123
The authors have no conflicts of interest to
References 1. Jung YC, Chanraud S, Sullivan EV (2012) Neuroimaging of Wernicke’s encephalopathy and Korsakoff’s syndrome. Neuropsychol Rev 22(2):170–180 2. Ueda Y, Utsunomiya H, Fujii A, Kanaumi T, Yasumoto S (2007) Wernicke encephalopathy in a chronic peritoneal dialysis patient– correlation between diffusion MR and pathological findings. No To Hattats 39(3):210–213 3. Liu YT, Fuh JL, Lirng JF, Li AF, Ho DM, Wang SJ (2006) Correlation of magnetic resonance images with neuropathology in acute Wernicke’s encephalopathy. Clin Neurol Neurosurg 108(7):682–687 4. Hong KS, Kang DW, Cho YJ, Hwang YJ, Hur G (2002) Diffusionweighted magnetic resonance imaging in Wernicke’s encephalopathy. Acta Neurol Scand 105(2):132–134 5. Calingasan NY, Baker H, Sheu KF, Gibson GE (1995) Blood-brain barrier abnormalities in vulnerable brain regions during thiamine deficiency. Exp Neurol 134(1):64–72 6. Harata N, Iwasaki Y (1995) Evidence for early blood–brain barrier breakdown in experimental thiamine deficiency in the mouse. Metab Brain Dis 10(2):159–174
