The Neuroradiology Journal 19: 603-605, 2006
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Magnetic Resonance Imaging in a Case of Wernicke’s Encephalopathy with Hyperemesis Gravidarum A Case Report ZHU XIANGYU, WU LEBIN, WANG GUANGBIN, LIN XIANGTAO, WANG TAO Medical school, University of Shandong; Shandong Medical Imaging Research Institute; Jinan, China
Key words: Wernicke's encephalopathy, magnetic resonance, thiamine deficency
SUMMARY – We describe a 29-year-old pregnant woman presenting with neurological signs and diagnosed to have Wernicke’s encephalopathy. The patient had a full recovery after prompt intravenous thiamine therapy. The clinical, pathological and MR features of Wernicke’s encephalopathy are discussed.
A 29-year-old pregnant woman with missed abortion was admitted because of progressive ophthalmoplegia and mental confusion over five days. She presented progressive confusion, with drowsiness, spatial and temporal disorientation, abasia, ataxia, nystagmus, hypermetria on finger-nose testing, tetraparesis with diffuse hypotonia, increased deep tendon reflexes and bilateral Babinski sign. Blood analysis was normal except for increased BUN (3.02 mg/dl; normal 10±50 mg/dl), Cr(209.2 mmol/kg; normal 71~106 mmol/kg),CRP(4.85 mg/dl; normal 0~8.20 mmol/kg) and decrease of potassium (2.30 mmol/kg; normal 135±144 mmol/kg). Lumbar puncture revealed no abnormality of the cerebrospinal fluid. EEG showed much higher pathological wave amplitude with 30~70 muv in the prefrontal region. B-type ultrasonography of the abdomen showed fetal death. The patient underwent MR scan on the day following admission. Both fast spin echo and fluid-attenuated inversion-recovery (FLAIR) sequences were performed on the brain. MRI showed symmetrical areas of increased signal on FLAIR images (figure 1 A,B) and T2weighted images (figure 1 C) in the periaqueductal grey matter, thalamus and mamillary bodies and the periphery of the third ventricle. The lesions gave a mildly low signal on T1-
weighted images (figure 1 D). The diagnosis of Wernicke’s encephalopathy was made and intravenous thiamine therapy, 100 mg daily, was started in addition to general parenteral nutrition as a bolus, and the same daily dose was administred with the TPN. After four days, hyperlactataemia fully subsided and her neurological condition started to improve. Eight days after the beginning of the treatment, the patient was fully alert, but both the nystagmus and the signs of cerebellar dysfunction were still present, although reduced. Two weeks later the patient was discharged home free of symptoms. Discussion Wernicke’s encephalopathy (WE) is an uncommon disorder caused by deficiency of vitamin B1 or thiamine, an essential coenzyme in intermediate carbohydrate metabolism. In its absence, glucose metabolism is impaired, causing pyruvate and lactate accumulation and adenosine triphosphate stores depletion 1. Although WE was initially considered a consequence of alcohol-related malnutrition, WE can occur in all conditions associated with a reduced thiamine intake and/or absorption, 603
Magnetic Resonance Imaging in a Case of Wernicke’s Encephalopathy with Hyperemesis Gravidarum. A Case Report
A
Zhu Xiangyu
B
Figure 1 A,B) Axial fluid-attenuated inversion-recovery (FLAIR) sequences on the day after admission show symmetrical highsignal areas in the periaqueductal grey matter, medial thalamus and the periphery of the third ventricle.
Figure 1 C Axial T2-weighted (TR 4000, TE 95) spin echo images showed high-signal areas in the periaqueductal grey matter, and medial thalamus, and the periphery of the third ventricle. Spin echo images showed the lesions gave mildly low signal in the periaqueduct.
604
Figure 1 D Axial T1-weighted (TR 500, TE 7.7) image shows a mildly low signal in the grey matter, medial thalamus and the periphery of the third ventricle.
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including prolonged starvation, hyperemesis gravidarum 2, anorexia nervosa, Wipple’s disease, malignancies and extensive inflammatory disorder of the gastrointestinal tract. Macroscopic pathological findings in acute Wernicke’s encephalopathy include symmetrical discoloration, shrinkage, congestion and microhemorrhages in some grey matter structures, including the periaqueductal grey matter, the mamillary bodies and medial thalamus. Macrohemorrhage is encountered in about 5% of cases and is thought to be a late change in patients with rapid depletion of thiamine, such as those receiving parenteral nutrition without vitamin supplements 3. Wernicke’s encephalopathy is clinically characterized by the triad of ophthalmoplegia, ataxia, nystagmus, disturbances of consciousness and mental symptoms ranging from confusion to coma, each finding being variably present. The disease is caused by malnutrition or malabsorption, for the pregnant woman, which was mainly caused by persistent vomiting during the course of pregnancy 4. Clinical features are promptly reversed by high dose chemotherapy of intravenous thiamine. Early diagnosis is essential in order to avoid irreversible neuronal changes associated with a persisting shortage of thiamine. MRI may be the only imaging technique able to detect the cerebral lesions, whose type and distribution are characteristic of Wernicke’s encephalopathy, whereas CT is positive only in exceptional cases 5. MRI demonstration of symmetri-
The Neuroradiology Journal 19: 603-605, 2006
cal damage to the mesencephalic tegmentum, mamillary bodies and medial thalamus is fundamental for diagnosis and treatment of this reversible but potentially fatal condition. MRI several days after admission showed a reduction of the areas of signal change in the periaqueductal grey matter, the dorsal nuclei of the thalamus and mamillary bodies, with mild ex vacuo dilatation of the third ventricle. The latter are better shown by MRI, as areas of increased signal on proton-density and T2weighted images. Although not entirely specific, the distribution of the lesions in the mesencephalic tegmentum, mamillary bodies and thalamus enables a straightforward diagnosis of acute Wernicke’s encephalopathy. Due to the possibility that cerebrospinal fluid may mask high-signal lesions on T2-weighted images, proton-density and possibly fluid-attenuated inversion-recovery (FLAIR) sequences are indicated to show the typical lesion distribution. After intravenous Gd-DTPA (0.1 mmol/kg), the periaqueductal grey matter and mamillary bodies and thalamus lesions enhanced moderately, enhancement may be absent on MRI in the early phase 6. Occurrence of Wernicke’s encephalopathy in the context of severe malnutrition unrelated to alcohol consumption is well known and awareness of the condition may be lifesaving, especially in patients in poor general health. The quick onset of thiamine therapy led to a regression of symptoms and prevented permanent brain lesions modifying the prognosis.
References 1 Brody BA: The Wernicke-Korsakoff syndrome. Neuropathology and pathogenetic basis. Int J Neuroradiol 2: 216-230, 1996. 2 Omer SM, Al-Kawi MZ, Al-Watban J et Al: Acute Wernicke’s encephalopathy associated with hyperemesis gravidarum: magnetic resonance imaging findings. J Neuroimaging 5: 251-253, 1995. 3 Mascalchi M, Simonelli P, Tessa C et Al: Do acute lesions of Wernicke’s encephalopathy show contrast enhancement? Report of three cases and review of the literature. Neuroradiology 41: 249-54, 1999. 4 Vortmeyer AO, Hagel C, Laas R: Haemorrhagic thiamine deficient encephalopathy following prolonged parenteral nutrition. J Neurol Neurosurg Psychiatry 55: 826-829, 1992. 5 L. Pagnan, G. Berlot, R.S. Pozzi-Mucelli: Magnetic resonance imaging in a case of Wernicke’s encephalopathy. Eur Radiol 8: 977-980, 1998.
6 Harter SB, Nokes SR: Gadolinium-enhanced MR findings in a pediatric case of Wernicke encephalopathy. Am J Neuroradiol 16: 700-702, 1995.
Zhu Xiangyu, M.D. Medical School, University of Shandong 250012 Jinan, China E-mail: [email protected] Phone: 8613210507380 Correspondence to: Wu Lebin Shandong Medical Imaging Research Institute 250021 Jinan China E-mail: [email protected]
605
www. centauro. it
Magnetic Resonance Imaging in a Case of Wernicke’s Encephalopathy with Hyperemesis Gravidarum A Case Report ZHU XIANGYU, WU LEBIN, WANG GUANGBIN, LIN XIANGTAO, WANG TAO Medical school, University of Shandong; Shandong Medical Imaging Research Institute; Jinan, China
Key words: Wernicke's encephalopathy, magnetic resonance, thiamine deficency
SUMMARY – We describe a 29-year-old pregnant woman presenting with neurological signs and diagnosed to have Wernicke’s encephalopathy. The patient had a full recovery after prompt intravenous thiamine therapy. The clinical, pathological and MR features of Wernicke’s encephalopathy are discussed.
A 29-year-old pregnant woman with missed abortion was admitted because of progressive ophthalmoplegia and mental confusion over five days. She presented progressive confusion, with drowsiness, spatial and temporal disorientation, abasia, ataxia, nystagmus, hypermetria on finger-nose testing, tetraparesis with diffuse hypotonia, increased deep tendon reflexes and bilateral Babinski sign. Blood analysis was normal except for increased BUN (3.02 mg/dl; normal 10±50 mg/dl), Cr(209.2 mmol/kg; normal 71~106 mmol/kg),CRP(4.85 mg/dl; normal 0~8.20 mmol/kg) and decrease of potassium (2.30 mmol/kg; normal 135±144 mmol/kg). Lumbar puncture revealed no abnormality of the cerebrospinal fluid. EEG showed much higher pathological wave amplitude with 30~70 muv in the prefrontal region. B-type ultrasonography of the abdomen showed fetal death. The patient underwent MR scan on the day following admission. Both fast spin echo and fluid-attenuated inversion-recovery (FLAIR) sequences were performed on the brain. MRI showed symmetrical areas of increased signal on FLAIR images (figure 1 A,B) and T2weighted images (figure 1 C) in the periaqueductal grey matter, thalamus and mamillary bodies and the periphery of the third ventricle. The lesions gave a mildly low signal on T1-
weighted images (figure 1 D). The diagnosis of Wernicke’s encephalopathy was made and intravenous thiamine therapy, 100 mg daily, was started in addition to general parenteral nutrition as a bolus, and the same daily dose was administred with the TPN. After four days, hyperlactataemia fully subsided and her neurological condition started to improve. Eight days after the beginning of the treatment, the patient was fully alert, but both the nystagmus and the signs of cerebellar dysfunction were still present, although reduced. Two weeks later the patient was discharged home free of symptoms. Discussion Wernicke’s encephalopathy (WE) is an uncommon disorder caused by deficiency of vitamin B1 or thiamine, an essential coenzyme in intermediate carbohydrate metabolism. In its absence, glucose metabolism is impaired, causing pyruvate and lactate accumulation and adenosine triphosphate stores depletion 1. Although WE was initially considered a consequence of alcohol-related malnutrition, WE can occur in all conditions associated with a reduced thiamine intake and/or absorption, 603
Magnetic Resonance Imaging in a Case of Wernicke’s Encephalopathy with Hyperemesis Gravidarum. A Case Report
A
Zhu Xiangyu
B
Figure 1 A,B) Axial fluid-attenuated inversion-recovery (FLAIR) sequences on the day after admission show symmetrical highsignal areas in the periaqueductal grey matter, medial thalamus and the periphery of the third ventricle.
Figure 1 C Axial T2-weighted (TR 4000, TE 95) spin echo images showed high-signal areas in the periaqueductal grey matter, and medial thalamus, and the periphery of the third ventricle. Spin echo images showed the lesions gave mildly low signal in the periaqueduct.
604
Figure 1 D Axial T1-weighted (TR 500, TE 7.7) image shows a mildly low signal in the grey matter, medial thalamus and the periphery of the third ventricle.
www. centauro. it
including prolonged starvation, hyperemesis gravidarum 2, anorexia nervosa, Wipple’s disease, malignancies and extensive inflammatory disorder of the gastrointestinal tract. Macroscopic pathological findings in acute Wernicke’s encephalopathy include symmetrical discoloration, shrinkage, congestion and microhemorrhages in some grey matter structures, including the periaqueductal grey matter, the mamillary bodies and medial thalamus. Macrohemorrhage is encountered in about 5% of cases and is thought to be a late change in patients with rapid depletion of thiamine, such as those receiving parenteral nutrition without vitamin supplements 3. Wernicke’s encephalopathy is clinically characterized by the triad of ophthalmoplegia, ataxia, nystagmus, disturbances of consciousness and mental symptoms ranging from confusion to coma, each finding being variably present. The disease is caused by malnutrition or malabsorption, for the pregnant woman, which was mainly caused by persistent vomiting during the course of pregnancy 4. Clinical features are promptly reversed by high dose chemotherapy of intravenous thiamine. Early diagnosis is essential in order to avoid irreversible neuronal changes associated with a persisting shortage of thiamine. MRI may be the only imaging technique able to detect the cerebral lesions, whose type and distribution are characteristic of Wernicke’s encephalopathy, whereas CT is positive only in exceptional cases 5. MRI demonstration of symmetri-
The Neuroradiology Journal 19: 603-605, 2006
cal damage to the mesencephalic tegmentum, mamillary bodies and medial thalamus is fundamental for diagnosis and treatment of this reversible but potentially fatal condition. MRI several days after admission showed a reduction of the areas of signal change in the periaqueductal grey matter, the dorsal nuclei of the thalamus and mamillary bodies, with mild ex vacuo dilatation of the third ventricle. The latter are better shown by MRI, as areas of increased signal on proton-density and T2weighted images. Although not entirely specific, the distribution of the lesions in the mesencephalic tegmentum, mamillary bodies and thalamus enables a straightforward diagnosis of acute Wernicke’s encephalopathy. Due to the possibility that cerebrospinal fluid may mask high-signal lesions on T2-weighted images, proton-density and possibly fluid-attenuated inversion-recovery (FLAIR) sequences are indicated to show the typical lesion distribution. After intravenous Gd-DTPA (0.1 mmol/kg), the periaqueductal grey matter and mamillary bodies and thalamus lesions enhanced moderately, enhancement may be absent on MRI in the early phase 6. Occurrence of Wernicke’s encephalopathy in the context of severe malnutrition unrelated to alcohol consumption is well known and awareness of the condition may be lifesaving, especially in patients in poor general health. The quick onset of thiamine therapy led to a regression of symptoms and prevented permanent brain lesions modifying the prognosis.
References 1 Brody BA: The Wernicke-Korsakoff syndrome. Neuropathology and pathogenetic basis. Int J Neuroradiol 2: 216-230, 1996. 2 Omer SM, Al-Kawi MZ, Al-Watban J et Al: Acute Wernicke’s encephalopathy associated with hyperemesis gravidarum: magnetic resonance imaging findings. J Neuroimaging 5: 251-253, 1995. 3 Mascalchi M, Simonelli P, Tessa C et Al: Do acute lesions of Wernicke’s encephalopathy show contrast enhancement? Report of three cases and review of the literature. Neuroradiology 41: 249-54, 1999. 4 Vortmeyer AO, Hagel C, Laas R: Haemorrhagic thiamine deficient encephalopathy following prolonged parenteral nutrition. J Neurol Neurosurg Psychiatry 55: 826-829, 1992. 5 L. Pagnan, G. Berlot, R.S. Pozzi-Mucelli: Magnetic resonance imaging in a case of Wernicke’s encephalopathy. Eur Radiol 8: 977-980, 1998.
6 Harter SB, Nokes SR: Gadolinium-enhanced MR findings in a pediatric case of Wernicke encephalopathy. Am J Neuroradiol 16: 700-702, 1995.
Zhu Xiangyu, M.D. Medical School, University of Shandong 250012 Jinan, China E-mail: [email protected] Phone: 8613210507380 Correspondence to: Wu Lebin Shandong Medical Imaging Research Institute 250021 Jinan China E-mail: [email protected]
605
![[Hyperemesis gravidarum with severe electrolyte disorders: report of a case].](/assets/img/hold.png)
