ABSTRACT
PURPOSE: Clinical features of Wernicke's encephalopathy (WE) confirmed strictly through the low blood vitamin B1 (VB1) levels are limited. This study aimed to analyse magnetic resonance imaging (MRI) findings, and clinical characteristics, in patients with WE who have confirmed low blood VB1 levels. METHODS: Clinical and laboratory records of 12 consecutive patients with WE admitted to our hospital during the past 11 years were reviewed. The WE diagnosis was confirmed based on low blood VB1 levels and the presence of at least one of the classical triad. RESULTS: Ophthalmoplegia and nystagmus were recorded in 75% and 50% of the patients, respectively. Eleven of 12 patients presented with consciousness disturbance/memory loss. All patients experienced gait disturbances. Eight of the 12 patients exhibited MRI abnormalities at typical sites (the dorsal midbrain [n = 7], medial thalamus [n = 6], mammillary bodies [n = 5], and dorsal pons [n = 5]). Of the 12 patients, six showed abnormalities at atypical sites (the splenium of the corpus callosum [n = 4], fornix [n = 3], cerebral cortex [n = 2], cerebellar vermis [n = 2], and dorsal medulla [n = 1]). Patients with positive MRI abnormalities had significantly lower blood VB1 levels than those without abnormalities (9.5 vs. 16.0 ng/mL). CONCLUSIONS: In cases of confirmed WE with low blood VB1 levels, the corpus callosum, fornix, and cerebral cortex were more frequently involved than in previous studies. MRI abnormalities at both typical and atypical sites were correlated with low blood VB1 levels in WE, suggesting that lower blood VB1 levels are associated with more severe brain damage in patients with WE.
Subject(s)
Magnetic Resonance Imaging , Thiamine , Wernicke Encephalopathy , Humans , Wernicke Encephalopathy/blood , Wernicke Encephalopathy/diagnostic imaging , Female , Male , Middle Aged , Aged , Thiamine/blood , Adult , Brain/diagnostic imaging , Retrospective StudiesABSTRACT
[Objective] To investigate association between Wernicke encephalopathy (WE) and brain MRI. [Subjects] 26 patients (7 females, mean age 63.9 ± 12.7 years) with WE admitted to our department between May 2008 and September 2022. [Methods] Wernicke's encephalopathy in patients with MRI lesions was defined as "MRI-positive group" (MPG), and those without MRI lesions as "MRI-negative group" (MNG). The following parameters were assessed between the two groups: age, sex, alcoholism, neurological symptoms, vitamin B1, lymphocyte, total cholesterol, albumin, and outcome at discharge. [Results] There were 17 patients in MPG. Compared to MNG, MPG had lower rates of alcohol abuse (10.0% vs 77.8%, P = 0.025), lower vitamin B1 (median 10.0â |ng/ml vs 29.0â |ng/ml, P < 0.001), and more vitamin B1 treatment dose (median 1900â |mg vs 600â |mg, P = 0.016). [Conclusion] Alcoholic WE may be overlooked if the focus is solely on brain MRI findings.
Subject(s)
Alcoholism , Brain , Magnetic Resonance Imaging , Thiamine , Wernicke Encephalopathy , Humans , Wernicke Encephalopathy/diagnostic imaging , Wernicke Encephalopathy/etiology , Female , Male , Middle Aged , Thiamine/administration & dosage , Aged , Brain/diagnostic imaging , Brain/pathology , Alcoholism/complications , Alcoholism/diagnostic imagingABSTRACT
BACKGROUND: Wernicke's encephalopathy (WE) is an acute neurological syndrome resulting from thiamine (vitamin B1) deficiency. It has been recognized increasingly in non-alcoholic patients, such as in the condition of malnutrition. Recent literature has shed light on uncommon symptoms and neuroimaging findings. CASE REPORT: We reported a case of a 44-year-old male who initially presented with bilateral hearing loss, and exhibited abnormality in the splenium of the corpus callosum on magnetic resonance imaging (MRI) diffusion-weighted imaging sequence. On the following day the patient developed new symptoms, including unstable walking, double vision and hallucination. The subsequent brain MRI demonstrated lesions involving periaqueductal grey matter and bilateral medial thalamus, indicating the diagnosis of WE. Empirical treatment with intravenous thiamine resulted in complete clinical and radiological resolution. CONCLUSION: To the best of our knowledge, the current case is the first report of WE in literature with uncommon but reversible manifestations. This case warns us to maintain a heightened level of suspicion for WE in malnourished patients with neurological deficits, despite the possibility of atypical presentations encompassing bilateral hearing disturbances and unusual neuroradiological results. Early diagnosis and timely administration of thiamine in WE are likely to lead to a favorable outcome and full recovery.
Subject(s)
Corpus Callosum , Hearing Loss, Bilateral , Wernicke Encephalopathy , Humans , Male , Wernicke Encephalopathy/diagnostic imaging , Wernicke Encephalopathy/complications , Wernicke Encephalopathy/diagnosis , Wernicke Encephalopathy/drug therapy , Adult , Corpus Callosum/diagnostic imaging , Corpus Callosum/pathology , Hearing Loss, Bilateral/etiology , Thiamine/therapeutic use , Thiamine/administration & dosage , Vitamin B Complex/administration & dosage , Vitamin B Complex/therapeutic use , Magnetic Resonance Imaging , Diffusion Magnetic Resonance ImagingSubject(s)
Marchiafava-Bignami Disease , Wernicke Encephalopathy , Female , Humans , Male , Middle Aged , Hand/physiopathology , Magnetic Resonance Imaging , Marchiafava-Bignami Disease/diagnostic imaging , Marchiafava-Bignami Disease/complications , Wernicke Encephalopathy/diagnostic imaging , Wernicke Encephalopathy/diagnosisABSTRACT
Wernicke encephalopathy is an emergent neurological disorder caused by vitamin B1 (thiamine) deficiency. Here, we present a case of Wernicke encephalopathy in a male patient in his 70s with normal serum thiamine levels and MRI findings on admission. He had a history of heavy alcohol consumption and a gradual decrease in food intake. On arrival at the hospital, his consciousness was impaired which persisted even after glucose replacement. Moreover, horizontal nystagmus and cerebellar ataxia were observed. Head CT scan and MRI revealed no abnormal findings. Further, his serum thiamine level was within the normal range. The patient was clinically diagnosed with Wernicke encephalopathy, and high-dose thiamine therapy was started. Then, his symptoms improved immediately. Thus, in case of clinical suspicion, treatment for Wernicke encephalopathy must be initiated promptly even in patients with normal serum thiamine levels.
Subject(s)
Beriberi , Korsakoff Syndrome , Thiamine Deficiency , Wernicke Encephalopathy , Humans , Male , Beriberi/complications , Korsakoff Syndrome/etiology , Magnetic Resonance Imaging , Thiamine , Thiamine Deficiency/complications , Thiamine Deficiency/diagnosis , Thiamine Deficiency/drug therapy , Wernicke Encephalopathy/diagnostic imaging , Wernicke Encephalopathy/drug therapy , AgedABSTRACT
BACKGROUND: Wernicke encephalopathy (WE) is a devastating acute or subacute neurological disorder caused by thiamine deficiency. Wernicke encephalopathy is characterized by the triad of ocular signs, cerebellar dysfunction, and confusion. Visual loss and hearing loss are less common findings in WE. Here, we report a case of Wernicke encephalopathy in a nonalcoholic liver cirrhosis patient who presented with acute bilateral deafness and bilateral blindness. CASE PRESENTATION: A 60-year-old Chinese man presented with a history of bilateral blindness and bilateral hypoacousia for 3 days. He had a history of liver cirrhosis and chronic hepatitis C virus infection and did not have a habit of alcohol consumption. Ophthalmologic and otologic examinations showed no obvious abnormalities. MRI findings revealed symmetric fluid-attenuated inversion recovery (FLAIR) hyperintensities in the bilateral medial dorsal thalamus, periventricular region around the third ventricle and tectum, and dorsal medulla oblongata. One day after hospitalization, the patient developed a mild coma. Based on the laboratory and neuroimaging findings, we diagnosed the patient with Wernicke encephalopathy. He soon regained consciousness after administration of thiamine. Both his visual acuity and his hearing function improved gradually. CONCLUSIONS: We suggest that Wernicke encephalopathy can present with bilateral blindness and bilateral deafness.
Subject(s)
Hepatitis C, Chronic , Thiamine Deficiency , Wernicke Encephalopathy , Male , Humans , Middle Aged , Wernicke Encephalopathy/diagnosis , Wernicke Encephalopathy/diagnostic imaging , Hearing Loss, Bilateral , Hepatitis C, Chronic/complications , Thiamine Deficiency/complications , Thiamine Deficiency/diagnosis , Blindness/complications , Vision Disorders/complications , Magnetic Resonance Imaging , Hearing , Liver Cirrhosis/complicationsABSTRACT
BACKGROUND AND PURPOSE: Wernicke's encephalopathy (WE) is a severe acute disorder related to thiamine deficiency. This study was aimed at revealing the relationship between clinical and imaging findings and WE recovery. METHODS: We retrospectively reviewed 34 cases of WE diagnosed between 2003 and 2020 (median age: 57 years, 14 females) at two academic institutions. WE cases were divided into two groups with symptomatic recovery within 4 weeks (group 1) or later (group 2). The lesion sites were divided into typical and atypical sites (total sites defined as when either typical or atypical sites were involved). Clinical and MRI features were compared between them as appropriate. RESULTS: WE patients were divided into group 1 (19 cases, median age: 57 years, 10 females) and group 2 (15 cases, median age: 57 years, four females). Regarding clinical features, only cerebellar ataxia was more often observed in group 1 than in group 2. Regarding MRI features, signal abnormality on T2-weighted image (WI)/fluid-attenuated inversion recovery (FLAIR) was more often observed in atypical sites between groups 1 and 2 (1/19 vs. 7/15; p = .01). There were significant differences between groups 1 and 2 regarding the presence of both vasogenic edema and cytotoxic edema in total sites (4/11 vs. 11/15, p = .005; 1/19 vs. 6/15, p = .03), with a significant difference in the presence of vasogenic edema in typical sites (4/19 vs. 10/15, p = .01). CONCLUSION: The early recovered group showed a lower incidence of T2WI/FLAIR abnormality in atypical sites and diffusion signal abnormality in total or typical sites with a lower incidence of cerebellar ataxia.
Subject(s)
Cerebellar Ataxia , Wernicke Encephalopathy , Female , Humans , Middle Aged , Wernicke Encephalopathy/diagnostic imaging , Wernicke Encephalopathy/etiology , Cerebellar Ataxia/complications , Retrospective Studies , Magnetic Resonance Imaging/adverse effects , Prognosis , Edema/complicationsABSTRACT
In sub-Saharan Africa, the COVID-19 pandemic has caused severe malnutrition in elderly populations with the appearance of vitamin deficiencies, in particular thiamine responsible for Gayet Wernicke's encephalopathy (EGW). We present a series of six (6) patients hospitalized in the Neurology Department of the CHU Ignace Deen for the management of a brain syndrome with vigilance disorders after recovery from COVID-19, including oculomotor disorders, motor incoordination on a course of severe weight loss. The six patients underwent an evaluation of malnutrition by determining the WHO body mass index, the Detsky index, the serum albumin assay, the thiamine assay and a neuroradiological assessment (MRI) and an electroencephalogram (EEG) examination although this does not seem necessary for diagnosis. Study of nutritional status: weight loss greater than 5%, patients in Desky group B and C, plasma albumin<30 g/l, lowered thiamine and MRI neuroradiological data: by the existence of hypersignals in certain regions of the neocortex, certain gray nuclei, the mammillary bodies the thalamic nuclei close to the wall of the 3rd ventricle and the regions bordering the 4th ventricle sign Gayet Wernicke's encephalopathy syndrome. This study shows a stereotyped clinical, biological, neuroradiological and evolutionary profile of Gayet Wernicke's encephalopathy in elderly subjects recovered from Covid-19 with proven malnutrition. These results are useful for the therapeutic and prognostic discussion.
Subject(s)
Brain Diseases , COVID-19 , Malnutrition , Wernicke Encephalopathy , Aged , Humans , Wernicke Encephalopathy/diagnosis , Wernicke Encephalopathy/diagnostic imaging , COVID-19/complications , Pandemics , Guinea , Thiamine/therapeutic use , Malnutrition/complicationsABSTRACT
BACKGROUND: Wernicke-Korsakoff syndrome is a neuropsychiatric disorder caused by thiamine deficiency composed of two related disorders accounting for an acute presentation and chronic progression. Hyperemesis gravidarum presents a significant risk factor for Wernicke-Korsakoff syndrome as symptoms may rapidly progress in the setting of pregnancy. We present the first-reported case of hyperemesis-gravidarum-associated Wernicke encephalopathy in a patient in the first half of pregnancy in which a missed diagnosis led to septic shock, fetal demise, and eventual profound Korsakoff syndrome. CASE PRESENTATION: We present the case of a 33-year-old primigravid African American woman at 15 weeks gestational age who initially presented at a community emergency department with nausea and vomiting that ultimately progressed to severe hyperemesis-gravidarum-associated Wernicke-Korsakoff syndrome, fetal demise, and septic shock. The patient received a total of 6 weeks of high-dose parenteral thiamine. Magnetic resonance imaging of the head and formal neuropsychological assessment following treatment plateau confirmed the diagnosis of Wernicke-Korsakoff syndrome. CONCLUSIONS: The multisystem complications seen in severe thiamine deficiency can delay timely administration of high-dose thiamine, particularly in pregnancy, in which the classic triad of Wernicke-Korsakoff syndrome may not raise clinical suspicion due to rapid progression of neurological sequelae in this population. We advise a low threshold for parenteral thiamine repletion in pregnant women with persistent vomiting as hyperemesis gravidarum-induced severe thiamine deficiency can result in Wernicke-Korsakoff syndrome, sepsis, and fetal demise.
Subject(s)
Hyperemesis Gravidarum , Korsakoff Syndrome , Shock, Septic , Thiamine Deficiency , Wernicke Encephalopathy , Female , Pregnancy , Humans , Adult , Hyperemesis Gravidarum/complications , Hyperemesis Gravidarum/diagnosis , Hyperemesis Gravidarum/therapy , Shock, Septic/complications , Korsakoff Syndrome/complications , Korsakoff Syndrome/diagnosis , Thiamine Deficiency/complications , Thiamine Deficiency/drug therapy , Thiamine Deficiency/diagnosis , Wernicke Encephalopathy/diagnostic imaging , Wernicke Encephalopathy/drug therapy , Thiamine/therapeutic use , Fetal DeathABSTRACT
OBJECTIVE: Wernicke encephalopathy (WE) is a neuropsychiatric syndrome caused by thiamine deficiency. Despite its low sensitivity, brain magnetic resonance imaging (MRI) is the most useful diagnostic technique. Our aim was to investigate whether the timing of the imaging study, and thiamine replacement can influence brain MRI findings in these patients. METHODS: Retrospective observational study of hospitalized patients between January/2008 and December/2020 with a clinical diagnosis of WE. Data from clinical presentation, diagnostic features, therapeutic approach, and outcomes were collected. RESULTS: We identified 41 patients (55 ± 13.3 years) with WE. Brain MRI was performed in 36 patients, and one third had T2/FLAIR hyperintensities suggestive of WE. We found an association between a history of poor diet and periventricular hyperintensities (p = 0.023), especially on the ventral surface of the thalamus and the periaqueductal region. It was found that the odds of having a typical imaging of WE decreased by 5.3% for each additional unit (100 mg) of thiamine administered (p = 0.046) (95% CI [0.89, 0.99]). On the other hand, the number of days from clinical presentation was not found to be a viable predictor (p = 0.254) (95% CI [0.88, 1.03]) Recovery was positively correlated with the total dose of thiamine received until discharge (p = 0.020). CONCLUSIONS: MRI hyperintensities seem to be dependent on the timing of thiamine correction and, particularly, on the thiamine dosage prescribed at admission. Nevertheless, thiamine replacement should not be delayed, as its timely prescription is associated with a better prognosis at discharge.
Subject(s)
Korsakoff Syndrome , Thiamine Deficiency , Wernicke Encephalopathy , Humans , Wernicke Encephalopathy/diagnostic imaging , Tertiary Care Centers , Thiamine Deficiency/complications , Thiamine Deficiency/diagnostic imaging , Thiamine/therapeutic use , Magnetic Resonance ImagingABSTRACT
A 35-year-old Japanese man had been treated for alcoholism until 6 months before coming to our hospital, after which he discontinued treatment for alcoholism. He noticed dizziness from two weeks ago. He visited our hospital because his dizziness was worsened and he noticed diplopia from two days ago. Physical examination revealed bilateral abducens nerve palsy, decreased limb tendon reflex, and ataxia. His blood vitamin B1 level was 16 ng/ml (normal range 24-66 ng/ml). FLAIR images on brain MRI showed high signal intensity lesions in the bilateral abducens nuclei and mammillary body. We diagnosed him as Wernicke encephalopathy (WE) with lesions in the bilateral abducens nuclei. Treatment with thiamine rapidly resulted in improvement of his neurological symptoms and MRI findings. He was discharged from our department on the 10th hospitalization day. Previous reports have shown that abducens nerve palsy and horizontal gaze evoked nystagmus may occur in the early state of WE. This case report highlights the importance to comprehend the atypical MRI findings of WE to treat a patient at the early stage.
Subject(s)
Abducens Nerve Diseases , Alcoholism , Wernicke Encephalopathy , Humans , Male , Adult , Wernicke Encephalopathy/diagnosis , Wernicke Encephalopathy/diagnostic imaging , Alcoholism/complications , Dizziness , Thiamine , Abducens Nerve Diseases/etiology , Magnetic Resonance Imaging , VertigoABSTRACT
Wernicke's encephalopathy (WE) and paraneoplastic limbic encephalitis (PLE) can both present with acute-to-subacute memory impairment and cognitive dysfunction. Both can lead to significant morbidity and mortality without rapid identification and treatment. Often patients with WE may not have the typical clinical triad of ophthalmoplegia, gait ataxia, and altered mental status. Furthermore, both WE and PLE may share similar MRI findings. Here, we present a case of a patient with a history of seronegative PLE presenting with acute-to-subacute cognitive changes and gait imbalance. Initially, it was felt to be a relapse of PLE but upon further history and testing may potentially have represented WE in the setting of a recent dietary change.