Fig 2 - uploaded by Edith V Sullivan
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Three contiguous FLAIR images (5 mm thick with a 2.5 mm skip) of the acute WE case in Fig. 1. Note the hyperintense signal in the mammillary bodies and colliculi (left), periventricular gray matter (middle), and fornix and thalamus (right).
Source publication
Presented is the neuroradiological signature of acute Wernicke's encephalopathy (WE), derived from different types of magnetic resonance imaging (MRI) sequences. WE results from thiamine depletion, and its most typical antecedent is chronic alcohol dependence. Brain regions observed with in vivo MRI affected in acute WE include the mammillary bodie...
Citations
... PKC signalling regulates neurotransmitter release by modulating the activity of presynaptic proteins involved in synaptic vesicle exocytosis and neurotransmitter release. In AD, dysregulated PKC activity disrupts neurotransmitter release mechanisms, leading to impaired synaptic transmission [150]. For example, PKC phosphorylates synapsin proteins, which regulate synaptic vesicle trafficking and neurotransmitter release. ...
Protein kinase C (PKC) is a diverse enzyme family crucial for cell signalling in various organs. Its dysregulation is linked to numerous diseases, including cancer, cardiovascular disorders, and neurological problems. In the brain, PKC plays pivotal roles in synaptic plasticity, learning, memory, and neuronal survival. Specifically, PKC's involvement in Alzheimer's Disease (AD) path-ogenesis is of significant interest. The dysregulation of PKC signalling has been linked to neurolog-ical disorders, including AD. This review elucidates PKC's pivotal role in neurological health, particularly its implications in AD pathogenesis and chronic alcohol addiction. AD, characterised by neurodegeneration, implicates PKC dysregulation in synaptic dysfunction and cognitive decline. Conversely, chronic alcohol consumption elicits neural adaptations intertwined with PKC signalling , exacerbating addictive behaviours. By unravelling PKC's involvement in these afflictions, potential therapeutic avenues emerge, offering promise for ameliorating their debilitating effects. This review navigates the complex interplay between PKC, AD pathology, and alcohol addiction, illuminating pathways for future neurotherapeutic interventions.
... KS does not necessarily occur as a consequence of WE, but both are often considered in the literature as a single disorder, termed Wernicke-Korsakoff Syndrome (WKS) [7]. Neuroimaging techniques generally show signal hyperintensity in the mammillary bodies, periaqueductal gray ma er, thalamus, and colliculi, but lesions may also involve the pons, cerebellum, and hippocampus [8,9]. WKS is usually caused by excessive alcohol consumption; more rarely, it can instead be found following repeated episodes of vomiting accompanied by severe weight loss [10] and malabsorption syndrome, as in the case of acute pancreatitis. ...
Wernicke–Korsakoff Syndrome (WKS) is a severe neurological disorder resulting from thiamine deficiency, commonly associated with alcohol consumption but also stemming from dietary imbalances or other clinical conditions. Cognitive deficits, affecting memory and executive functions, pose a serious concern, with partial recovery often not complete. A 28-year-old woman underwent surgery for acute necrotizing hemorrhagic pancreatitis, leading to admission for post-acute intensive treatment due to prolonged bed rest syndrome. Clinical examinations revealed sensory–motor neuropathy, denervation in the active phase, mammillary body hyperintensity, and cognitive impairment. The patient exhibited poor orientation, lacked awareness of her clinical condition, and experienced impaired nonverbal memory, practical constructive issues, and planning difficulties—consistent with WKS. The patient received high-dose thiamine (300mg TDS), coupled with daily physiokinesitherapy and occupational therapy. A final neuropsychological evaluation three months later showed substantial remission of executive and memory difficulties, improved spatial–temporal orientation, and enhanced awareness. The complex case required timely multidisciplinary intervention for accurate diagnosis and effective rehabilitation. The patient experienced rapid clinical improvement and cognitive recovery with high-dose thiamine and physiotherapy.
... An MRI of the brain is an important tool to help physicians diagnose WE, particularly in cases of nonalcoholic WE, due to its high specificity. It exhibits a high specificity of 93% and a sensitivity of 53% [13]. Only one-half to two-thirds of patients show WE-related MRI findings [5], thus a negative brain MRI cannot definitively rule out the diagnosis of WE. ...
... Only one-half to two-thirds of patients show WE-related MRI findings [5], thus a negative brain MRI cannot definitively rule out the diagnosis of WE. The usual MRI findings include bilateral, symmetric T2-weighted, fluid-attenuated inversion recovery, diffusion-weighted imaging, or T1 post-contrast hyperintensities in the medial thalami, mammillary bodies, periaqueductal area, or tectal plate [13]. Cortical MRI lesions are indicative of permanent damage and serve as a poor prognostic sign. ...
Wernicke encephalopathy (WE) is a rare but life-threatening syndrome that is commonly associated with chronic alcoholism. It has also been found to be associated with malnutrition, prolonged parenteral nutrition, hemodialysis, hyperemesis gravidarum, gastroplasty, and AIDS. It usually presents as a clinical triad of confusion, ophthalmoplegia, and gait ataxia. Nystagmus is usually the most common and earliest ophthalmologic sign. We report a case of non-alcoholic WE in a patient who had prior bariatric surgery and was treated for malnutrition and sepsis, with nystagmus being the initial presentation. The MRI of the brain was normal. The diagnosis of WE was made clinically and was supported by the patient's symptomatic and clinical recovery following intravenous thiamine treatment. It is essential to highlight that a high level of suspicion is needed to diagnose non-alcoholic WE to allow the commencement of appropriate treatment and reduce morbidity and mortality rates related to this condition.
... The atrophy would also concern the thalamus, the mammillary bodies and the frontal lobes. In 2009, one study showed a graded effect of structural damage ranging from mild to moderate damage in patients with AUD to severe damage in patients with Korsakoff syndrome in the mammillary bodies, hippocampus, thalamus, cerebellum and pons [51]. Signi cant structural and metabolic damage have been shown in patients with Korsakoff syndrome, particularly in the Papez circuit and the frontocerebellar circuit. ...
Chronic alcohol use, either in the context of repeated binge drinking behavior or alcohol use disorder, induces brain and cognitive alterations. These brain and cognitive alterations can be reversible and a recovery, at least partial, is seen in general after weeks or months of abstinence. Cognitive deficits are highly frequent in patients with alcohol use disorder and are largely under-diagnosed and under-treated as for example for the Gayet-Wernicke encephalopathy that could be easily treated and or prevented (if suspected) by thiamine treatment. Cognitive deficits have an impact on the treatment and should be well identified and targeted in order to improve care of patients and increase the success rate in maintaining long term abstinence or reduced alcohol intake.
... Numerous magnetic resonance imaging (MRI) studies have typically shown symmetrical signal intensity alterations of the mammillary bodies, thalamus, fornices, midbrain and periaqueductalperiventricular grey matter area (Galvin et al., 2010;Sullivan and Pfefferbaum 2009, Nishimoto et al. 2017, Novo-Veleiro et al. 2021. Finding these alterations on MRI strongly supports a clinical diagnosis of Wernicke's encephalopathy (Guirguis et al. 2017). ...
... Current neuroimaging literature regarding Korsakoff syndrome clearly showed lesions to the thalamus, mammillary bodies and hippocampus (Sullivan and Pfefferbaum 2009, Segobin and Pitel 2021, Kopelman 2022. In a population of patients with alcohol use disorders, comparing those with or without Korsakoff syndrome, Segobin and Pitel (2021) showed significantly greater bilateral grey matter loss in the thalami and mammillary bodies within the group of patients with Korsakoff syndrome. ...
... The fornix and the cingulum, both of which have direct connections to the thalamus and hippocampus, have been observed to have increased white matter bundles. Thus, impaired microstructural integrity seems to be more severe in Korsakoff syndrome (Sullivan andPfefferbaum 2009, Segobin and. ...
Wernicke-Korsakoff syndrome is a complication of thiamine (vitamin B1) deficiency, common in patients with alcohol use disorders. Up to 80% of patients with Wernicke’s encephalopathy are undiagnosed and, thus remain untreated. This syndrome is classically described as a clinical triad consisting of confusion, ocular dysfunction (notably nystagmus and ophthalmoparesis) and ataxia. However, a minority of patients (16%) with Wernicke’s encephalopathy present with the complete triad. According to the European Federation of Neurological Societies, the clinical diagnosis of Wernicke’s encephalopathy in patients with alcohol use disorder requires two of the following signs: (1) dietary deficiencies, (2) ocular symptoms, (3) cerebellar dysfunction and (4) either an altered mental state or mild memory impairment. Wernicke encephalopathy is readily reversible if treated with adequate doses of parenteral thiamine, preferably within the first 48–72 h of the onset of symptoms. The overall safety of intravenous thiamine is very good.
When untreated, Wernicke’s encephalopathy can lead to coma or death, or progress to Korsakoff syndrome. Korsakoff syndrome is chronic and may be irreversible. It is characterized by cognitive and behavioral symptoms, including anterograde and retrograde memory impairments, executive dysfunction, confabulation, apathy, affective and social-cognitive impairments. Cognitive rehabilitation, including memory compensation techniques, as well as long-term thiamine supplementation, are currently recognized in the treatment of Korsakoff syndrome.
... The retrograde amnesia usually involves a temporally-graded memory loss, extending back 25 years or more [24,28,[81][82][83][84]. The specific neuroimaging findings characteristically associated with the Korsakoff syndrome have also been reviewed elsewhere [36] with characteristic atrophy on quantified structural MRI in the thalami and mammillary bodies, often associated with frontal lobe volume loss [85][86][87], and changes in glucose metabolism on FDG (fluorodeoxy-glucose) PET (positron emission tomography) in the thalamus and associated limbic circuitry [30]. ...
This paper begins with a short case report of florid, spontaneous confabulation in a 61-year-old man with an alcohol-induced Wernicke-Korsakoff syndrome. His confabulation extended across episodic and personal semantic memory, as well as orientation in time and place, as measured on Dalla Barba’s Confabulation Battery. Five other brief case summaries will then be presented, followed by a summary of the clinical, neurological, and background neuropsychological findings in three earlier series of Korsakoff patients. These observations will be considered in light of Wijnia’s recent and my own, earlier reviews of the Korsakoff syndrome. Taken together, they indicate the need for a multi-faceted approach (clinical, neurological, neuropsychological, and neuroimaging) to the assessment and diagnosis of the disorder.
... A systematic review by Caballeria et al (2020) highlighted studies that have looked into the bene ts of what is called the component method which is a rehabilitative strategy of breaking down complex tasks into smaller and easier to navigate components and small improvements in visuospatial skills were noted after one month of abstaining from alcohol. However, evidence in earlier studies had already indicated that impairment in this domain improved rapidly with abstinence (Sullivan 2008, Rosenbloom et al 2000. Horton et al (2015) investigated three different types of assessment (neurocognitive assessment, standardised self-reported questionnaires and functional assessment) of twenty participants in an abstinence based supported accommodation service in Glasgow. ...
Aims
Research into psychosocial and therapeutic interventions that people with a diagnosis of alcohol related brain damage (ARBD) access to further their recovery is scarce. The aim of this research project was to explore the potential benefits or limitations that such interventions can have in relation to their quality of life (QOL) as well as advancing this field of knowledge.
Methods
A search of the literature was completed to ascertain what is known of this topic. Semi-structured qualitative interviews were carried out with six participants with a diagnosis of ARBD residing in a supported residential setting. A grounded theory methodology was utilised to identify emerging themes.
Results
The themes of family input, television and technology, choice, decision making and goal-setting were identified as being key themes of the participant responses with the multifaceted roles of television being of particular interest.
Conclusion
The role of family can be helpful in many ways as well as providing a source of hope and motivation and this should be promoted whenever possible. Offering people with ARBD therapeutic opportunities should be encouraged as low motivation and indecision may be a presenting symptom. However, the multi-functioning role that television can play in the recovery process was most surprising and should be explored further by researchers.
... A systematic review by Caballeria et al (2020) highlighted studies that have looked into the bene ts of what is called the component method which is a rehabilitative strategy of breaking down complex tasks into smaller and easier to navigate components and small improvements in visuospatial skills were noted after one month of abstaining from alcohol. However, evidence in earlier studies had already indicated that impairment in this domain improved rapidly with abstinence (Sullivan 2008, Rosenbloom et al 2000. Horton et al (2015) investigated three different types of assessment (neurocognitive assessment, standardised self-reported questionnaires and functional assessment) of twenty participants in an abstinence based supported accommodation service in Glasgow. ...
Aims
Research into psychosocial and therapeutic interventions that people with a diagnosis of alcohol related brain damage (ARBD) access to further their recovery is scarce. The aim of this research project was to explore the potential benefits or limitations that such interventions can have for in relation to their quality of life (QOL) as well as advancing this field of knowledge.
Methods
A search of the literature was completed to ascertain what is known of this topic. Semi-structured qualitative interviews were carried out with six participants with a diagnosis of ARBD residing in a supported residential setting. A grounded theory methodology was utilised to identify emerging themes.
Results
The themes of family input, television and technology; choice, decision making and goal-setting were identified as being key themes of participant responses with the various roles of television being of particular interest.
Conclusion
The role of family can be helpful in many ways as well as providing a source of hope and motivation and should be promoted whenever possible. Offering people with ARBD therapeutic opportunities should be encouraged as low motivation and indecision may be present. However, the multi-functioning role that television can play in the recovery process was most surprising and should be explored further by researchers.
... The preservation of memories is limited only to those which are formed before ethanol-induced brain damage. Besides that, Korsakoff's amnesic patients also experience loss of muscle coordination (ataxia), violent behaviour, and confabulation [44,63]. ...
Recreational use of alcohol is a social norm in many communities worldwide. Alcohol use in moderation brings pleasure and may protect the cardiovascular system. However, excessive alcohol consumption or alcohol abuse are detrimental to one’s health. Three million deaths due to excessive alcohol consumption were reported by the World Health Organization. Emerging evidence also revealed the danger of moderate consumption, which includes the increased risk to cancer. Alcohol abuse and periods of withdrawal have been linked to depression and anxiety. Here, we present the effects of alcohol consumption (acute and chronic) on important brain structures—the frontal lobe, the temporal lobe, the limbic system, and the cerebellum. Apart from this, we also present the link between alcohol abuse and withdrawal and mood disorders in this review, thus drawing a link to oxidative stress. In addition, we also discuss the positive impacts of some pharmacotherapies used. Due to the ever-rising demands of life, the cycle between alcohol abuse, withdrawal, and mood disorders may be a never-ending cycle of destruction. Hence, through this review, we hope that we can emphasise the importance and urgency of managing this issue with the appropriate approaches.
... Other less well-known causes are HIV, SARS-CoV-2, and other viral infections [2][3][4][5], bacterial infections [6], end-stage cancer in palliative care [7], and hunger strike [8]. Although the diagnosis and treatment of patients with Wernicke-Korsakoff syndrome has been described for decades based on studies of biochemical processes [9,10], clinical presentation [10,11], pathological-anatomical [12] and neuroimaging findings [13], daily practice still shows challenges in diagnosing and treating these patients. The goal of this article is to provide a clinical framework for (timely) ...
... Although the mechanisms of the cognitive dysfunction are still not fully understood, loss of function in the Papez and frontocerebellar circuits [28,29] both including parts of the thalamus [30], may cause the impaired memory and executive functions that are the main characteristics of Korsakoff syndrome [11,31]. Some patients with Korsakoff syndrome suffer from additional damage in the cerebellum [13]. In our experience, this is accompanied by more serious disturbances in regulating behavior, such as perseveration and rigidity, and can increase caregiver burden. ...
... In magnetic resonance imaging (MRI) during the early Wernicke phase of the disorder, Sullivan and Pfefferbaum (2009) have shown an altered signal in various components of the limbic, circuits including the paraventricular regions of the thalamus, the hypothalamus, mammillary bodies, the periaqueductal region, the floor of the fourth ventricle and midline cerebellum [13,31]. The sensitivity of MRI in detecting Wernicke encephalopathy is only 53%, with a specificity of 93% [13]. ...
The purpose of this article is to improve recognition and treatment of Wernicke-Korsakoff syndrome. It is well known that Korsakoff syndrome is a chronic amnesia resulting from unrecognized or undertreated Wernicke encephalopathy and is caused by thiamine (vitamin B1) deficiency. The clinical presentation of thiamine deficiency includes loss of appetite, dizziness, tachycardia, and urinary bladder retention. These symptoms can be attributed to anticholinergic autonomic dysfunction, as well as confusion or delirium, which is part of the classic triad of Wernicke encephalopathy. Severe concomitant infections including sepsis of unknown origin are common during the Wernicke phase. These infections can be prodromal signs of severe thiamine deficiency, as has been shown in select case descriptions which present infections and lactic acidosis. The clinical symptoms of Wernicke delirium commonly arise within a few days before or during hospitalization and may occur as part of a refeeding syndrome. Wernicke encephalopathy is mostly related to alcohol addiction, but can also occur in other conditions, such as bariatric surgery, hyperemesis gravidarum, and anorexia nervosa. Alcohol related Wernicke encephalopathy may be identified by the presence of a delirium in malnourished alcoholic patients who have trouble walking. The onset of non-alcohol-related Wernicke encephalopathy is often characterized by vomiting, weight loss, and symptoms such as visual complaints due to optic neuropathy in thiamine deficiency. Regarding thiamine therapy, patients with hypomagnesemia may fail to respond to thiamine. This may especially be the case in the context of alcohol withdrawal or in adverse side effects of proton pump inhibitors combined with diuretics. Clinician awareness of the clinical significance of Wernicke delirium, urinary bladder retention, comorbid infections, refeeding syndrome, and hypomagnesemia may contribute to the recognition and treatment of the Wernicke-Korsakoff syndrome.
