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Korsakoff's psychosis (Wernicke-Korsakoff-Syndrome) following severe, long-term alcoholism is considered to be widely resistant to treatment. We report two cases with beneficial effects of treatment by a combination of clonidine and fluvoxamine.
BACKGROUND: Korsakoff syndrome presents with memory dysfunction with retrograde amnesia, anterograde amnesia, limited insight into dysfunction, and confabulation. The most common etiology of Korsakoff syndrome is thiamine deficiency secondary to alcoholism. There are limited case reports of structural lesions causing Korsakoff syndrome. CASE DESCRIPTION: A 46-year-old male with a long history of alcoholism presented with a history of confusion, amnesia, and confabulation with no localizing features on neurological examination. The patient showed no clinical change with intravenous thiamine. Computed tomography of the brain revealed a heterogenous, enhancing mass lesion centered within the third ventricle, with other lesions found throughout cortical and subcortical regions. The patient was given dexamethasone i.v. without noticeable clinical improvement but with marked radiological improvement with mass reduction. Stereotactic biopsy revealed a diagnosis of primary central nervous system (CNS) lymphoma. CONCLUSIONS: Most patients presenting with Korsakoff syndrome have thiamine deficiency; however, mass lesions can produce an identical clinical picture. This is the first case report of a patient with primary CNS lymphoma presenting as Korsakoff syndrome.
Alcoholic Korsakoff's syndrome (KS) is marked by remote memory impairment together with characteristic profound anterograde memory deficits. Despite previous studies of memory processes in KS, questions remain regarding the nature and severity of these impairments and identification of brain systems that underlie these different memory impairments. This study examined remote and anterograde memory function in 5 KS patients in comparison with 8 patients with Alzheimer's disease (AD) and 24 normal control subjects (NC). In addition, relationships between memory performance and regional brain volumes were examined in the KS group. Overall, the KS group showed severe impairment on both remote and anterograde memory measures, performing at the level of the AD group on most measures. Differences were observed on the pattern of temporal gradient for verbal recognition, with KS exhibiting a more steeply graded rate of decline over the most recent period examined. Severity of the remote memory deficit in KS was not associated with severity of anterograde memory deficit. Examination of brain structure-function relationships in the KS subjects revealed that photo naming of remote historical information was related to posterior cortical white matter volumes but not hippocampal volumes; sequencing was related to prefrontal but not hippocampal volumes. By contrast, a measure of anterograde memory for nonverbal visual material showed a relationship to hippocampal but not regional cortical white matter volumes. This set of dissociations, which parallels that observed in our earlier study of AD, is now documented in KS and provides further evidence that these separate cortical and limbic brain systems are principal neural substrates of the remote and anterograde memory and sequencing deficits in KS.
The Wernicke-Korsakoff syndrome is induced by thiamine deficiency. It occurs mainly in subjects with chronic alcoholism. It begins with an acute phase (Wernicke's encephalopathy) and changes to a chronic phase, which is characterized by a symptomatology varying from subclinical findings to a fully developed Korsakoff psychosis. We examined otoneurologically eight patients with Wernicke-Korsakoff syndrome. The examination included: clinical status, caloric stimulation, optokinetic nystagmus, pure tone audiogram (air- and bone conduction), speech audiogram, and brain-stem auditory evoked potentials. The typical findings of the chronic phase are: gaze nystagmus and reduced gain of the optokinetic nystagmus. In five of seven patients OK gain was reduced by more than two standard-deviations. The other examinations including brain-stem potentials did not reveal any specific findings. The symptomatology of the acute phase is well known: nystagmus (mostly lateral gaze nystagmus) and reduced by caloric responses. Early diagnosis of a Wernicke-Korsakoff syndrome is of great importance since its treatment is simple and efficient. The results of a thorough neuro-otologic examination are of considerable diagnostic value, particularly during the chronic phase of the disease.
Anterograde amnesia in Wernicke-Korsakoff syndrome is associated with diencephalic lesions, mainly in the anterior thalamic nuclei. Whether diencephalic and temporal lobe amnesias are distinct entities is still not clear. We investigated episodic memory for faces using functional MRI (fMRI) in eight controls and in a 34-year-old man with Wernicke-Korsakoff syndrome and diencephalic lesions but without medial temporal lobe (MTL) involvement at MRI. fMRI was performed with a 1.5 tesla unit. Three dual-choice tasks were employed: (i) face encoding (18 faces were randomly presented three times and subjects were asked to memorize the faces); (ii) face perception (subjects indicated which of two faces matched a third face); and (iii) face recognition (subjects indicated which of two faces belonged to the group they had been asked to memorize during encoding). All activation was greater in the right hemisphere. In controls both the encoding and recognition tasks activated two hippocampal regions (anterior and posterior). The anterior hippocampal region was more activated during recognition. Activation in the prefrontal cortex was greater during recognition. In the subject with Wernicke-Korsakoff syndrome, fMRI did not show hippocampal activation during either encoding or recognition. During recognition, although behavioural data showed defective retrieval, the prefrontal regions were activated as in controls, except for the ventrolateral prefrontal cortex. fMRI activation of the visual cortices and the behavioural score on the perception task indicated that the subject with Wernicke-Korsakoff syndrome perceived the faces, paid attention to the task and demonstrated accurate judgement. In the subject with Wernicke-Korsakoff syndrome, although the anatomical damage does not involve the MTL, the hippocampal memory encoding has been lost, possibly as a consequence of the hippocampal-anterior thalamic axis involvement. Anterograde amnesia could therefore be the expression of damage to an extended hippocampal system, and the distinction between temporal lobe and diencephalic amnesia has limited value. In the subject with Wernicke-Korsakoff syndrome, the preserved dorsolateral prefrontal cortex activation during incorrect recognition suggests that this region is more involved in either the orientation or attention at retrieval than in retrieval. The lack of activation of the prefrontal ventrolateral cortex confirms the role of this area in episodic memory formation.
Wernicke-Korsakoff syndrome, which is associated with high morbidity and mortality, is a more common neuropsychiatric sequela of alcohol misuse than is widely realized. It is easily prevented and treated with parenteral B-complex vitamins, although this treatment is widely under-utilized.
The Wernicke-Korsakoff syndrome is a rare neurological disorder which strikes primarily alcoholics and is caused, at least in part, by insufficient bioavailability of thiamin. Because of the low cost of preventing this disease by adding thiamin to alcoholic beverages relative to the cost of the long-term care of patients with the chronic phase of the disease, there has been a need to determine the feasibility of this particular preventive strategy. Recent data demonstrating normal levels of erythrocyte transketolase in patients presenting with Wernicke's encephalopathy has led some to question the link between thiamin deficiency, and the development of the syndrome, despite the fact that virtually all patients with Wernicke's encephalopathy will respond favorably to parenteral thiamin, and the evidence that rats deprived of thiamin develop lesions that are characteristic of the disease. Thiamin is nontoxic, is stable in alcoholic beverages, and is undetectable in beer to professional taste-testers at levels theoretically sufficient to guarantee adequate absorption. Although there is much inter-subject variability it appears that thiamin at therapeutic concentrations can be absorbed by the jejunum in the presence of alcohol. Evidence that patients with the Wernicke-Korsakoff syndrome have an altered thiamin-requiring isoenzyme and evidence that six patients with Wernicke's encephalopathy failed to respond to oral thiamin has caused some doubt as to whether the target population would be able to benefit from the measure. Other determinants of thiamin utilization, such as folate and magnesium status, also may figure in the etiology of the disease.(ABSTRACT TRUNCATED AT 250 WORDS)
Although alcoholic Korsakoff's syndrome has traditionally been considered an acute disorder related to a nutritional deficiency, recent evidence demonstrating that ethanol may be neurotoxic has raised the possibility that the perceptual, problem-solving, and memory deficits associated with this chronic neurological disorder may develop slowly over decades of alcohol abuse. A review of the recent cognitive literature provides only limited support for this "continuity hypothesis." Long-term alcoholics, as with patients with alcoholic Korsakoff's syndrome, are impaired on numerous visuoconceptual and learning tasks, but there is little or no evidence that the information-processing deficits underlying the two patient groups' anterograde memory problems are similar. Furthermore, experimental and clinical studies of retrograde amnesia have noted only mild remote memory impairments in non-Korsakoff alcoholics as well as clear indications that alcoholic Korsakoff patients' severe loss or access to remote memories occurs acutely with the onset of Wernicke's encephalopathy. It is concluded that while the continuity hypothesis has heuristic value, there is still insufficient evidence to place the Korsakoff patient, the detoxified long-term alcoholic, and the heavy social drinker at different points on a single continuum of cognitive impairment.
BACKGROUND: Investigations of the Korsakoff syndrome by researchers from different disciplines have proliferated in recent years, making it apposite to review the various findings. METHOD: This review is based on the author's knowledge of reports in the major clinical and neuropsychological journals, supplemented by Medline searches to update particular subtopics. RESULTS: The Korsakoff syndrome is defined as a disproportionate impairment in memory, relative to other aspects of cognitive function, resulting from a nutritional (thiamine) depletion. The initial manifestations of the disorder are variable, and a persistent memory impairment can result from a non-alcoholic aetiology, although this seems to happen much less commonly than in the past - presumably because of generally higher standards of nutrition. Although there is agreement on the underlying neuropathology, the critical lesion sites for memory disorder have been debated. Recent evidence suggests that the circuit involving the mammillary bodies, the mammillo-thalamic tract and the anterior thalamus, rather than the medial dorsal nucleus of the thalamus, is particularly critical in the formation of new memories. The relationship of these deficits to thiamine depletion remains a topic of current investigation, as does the purported role of neurotransmitter depletions in the cholinergic, glutamate/GABA and catecholamine and serotonergic systems. Neuro-imaging studies have confirmed autopsy findings of more widespread structural and metabolic abnormalities, particularly involving the frontal lobes. CONCLUSIONS: The relationship of these neuropathological, neurochemical, and metabolic abnormalities to cognitive functioning, with particular reference to specific aspects of memory processing, has been considered in some detail. Whereas structural and/or neurochemical abnormalities within the limbic/diencephalic circuits account for anterograde amnesia, some other factor, such as frontal lobe dysfunction, must underlie the severe retrograde memory loss which is characteristically found in this syndrome.
There are limited case reports of structural lesions causing Korsakoff syndrome. This report describes acute Korsakoff syndrome following localized, bilateral infarction of the mammillothalamic tracts (MTTs). Axial T2-weighted imaging revealed the lesions at the lateral wall level of the third ventricle and diffusion-weighted imaging confirmed that the left lesion was new and the right old. Korsakoff syndrome persisted 6 months after the onset. This case suggests that bilateral MTT dysfunction can lead to Korsakoff syndrome.
The Wernicke-Korsakoff syndrome is a neuropathological term which encompasses two clinical syndromes in thiamine-deficient alcoholics, Wernicke's encephalopathy and Korsakoff's psychosis. Wernicke's encephalopathy is characterised by eye and gait disorders and mental confusion, and can lead to the profound and permanent amnesia known as Korsakoff's psychosis. Despite this specific difference, both conditions appear to have identical neuropathology with haemorrhages and other lesions around the ventricular system. The memory deficit has been attributed to a number of brain lesions, including a recent suggestion that brain pathways utilising particular neurotransmitters are specifically damaged. To examine this, the number of chemically identified neurons in particular brain regions was quantified in patients with Wernicke's encephalopathy alone or in combination with Korsakoff's psychosis and compared with age- and sex-matched controls. Noradrenaline, a neurotransmitter thought to be involved in the process of selective attention, is localised in pathways projecting to the cortex. Our patients with either Wernicke's encephalopathy or additional Korsakoff's psychosis do not differ from controls in the distribution and number of these cells. Serotonin is another neurotransmitter that has been linked with alcohol dependency. Both patient groups have a profound loss of serotonin-containing neurons compared with controls. The loss of forebrain neurons containing acetylcholine in patients with Alzheimer's disease has implicated this neurotransmitter in the maintenance of memory functions. There was a large variation in the number of these forebrain neurons in thiamine-deficient alcoholics compared with controls. Cholinergic cell loss reflected the severity of cognitive dysfunction, but was not exclusive to patients with amnesia. Two thalamic nuclei are involved in relaying memories for storage and retrieval, the anterior and mediodorsal thalamic nuclei. While patients with Wernicke's encephalopathy often had neuronal loss in the mediodorsal nucleus, only patients with Korsakoff's psychosis had cell loss in both medial thalamic nuclei. The results suggest that cumulative lesions contribute to the amnesia seen in thiamine-deficient alcoholics, including deficits in serotonergic, cholinergic and medial thalamic pathways.
The Wernicke-Korsakoff syndrome occurs most frequently in alcoholic patients when they become thiamin deficient. First admissions to psychiatric units with the chronic component of this syndrome, Korsakoff's psychosis, peaked in Queensland in 1975-1976. The fall in hospital admission rates since this time could relate to a decline in per-capita alcohol consumption in Australia, or to more awareness of the thiamin needs of drinkers. Alternatively, the improvement may be illusory: although many cases of Wernicke's encephalopathy are being diagnosed, many of these patients are not receiving psychiatric assessment and treatment, perhaps because admission to psychiatric hospital beds is more difficult than it was formerly. Patients who are diagnosed as having Korsakoff's psychosis fare badly in the community, and have a greatly increased mortality rate than do such patients in hospital. Optimal care for such patients is necessarily costly of medical resources. Of available preventive measures, evidence is presented to support the fortification of beer with thiamin and the provision of community educational programmes. The fortification of flour with thiamin may have little impact on the thiamin-deficiency syndromes that arise in problem drinkers in Queensland.
29 patients with Korsakoff syndrome (KS) were examined by means of brainstem auditory evoked potentials. The interpeak-latencies were delayed cumulative with the most accentuation of IPL I-VI. BAEP are suitable for subdividing of KS. It is suggested that BAEP shall observed in alcoholics for detection of norm-deviations.
The Wernicke-Korsakoff syndrome (WKS) is a picture of oculomotor alterations, ataxia and confusion presented in chronic alcoholics. It has more rarely been described in non alcoholic patients with malnutrition. The case of a patient with ulcerous peptic disease of long evolution who consulted for a picture compatible with WKS following clinical manifestations of repeated vomiting secondary to complete pyloric stenosis is presented. The peculiarity of the picture and the convenience of prevention in malnourished patients receiving intravenous glucose sera is discussed.
BACKGROUND: Surgical exclusion of portions of the gastrointestinal tract is a predisposing risk factor for the development of Wernicke-Korsakoff syndrome. When this disease occurs, it is usually within weeks after the gastrointestinal surgery. However, it is not well known that Wernicke-Korsakoff syndrome may occur after a long latent interval following gastrectomy. SETTING: A research-oriented hospital. PATIENTS: Three patients without a history of alcoholism or dietary deprivation developed Wernicke-Korsakoff syndrome 2 to 20 years after undergoing gastrectomy. In these patients, minor changes in dietary habit led to the development of Wernicke-Korsakoff syndrome. CONCLUSIONS: In addition to a long-standing latent deficiency in thiamin levels due to defective absorption following gastrectomy or gastrojejunostomy, other minor factors that may influence the intake of thiamin and the need for thiamin in subjects who have undergone gastrectomy may cause a state of thiamin deficiency resulting in Wernicke-Korsakoff syndrome. Results from our study indicate that the following measures are mandatory: educating patients about proper dietary habits, carefully monitoring their thiamin intake, recognizing Wernicke-Korsakoff syndrome early, and treating it immediately with appropriate measures.
PURPOSE: The authors hereby describe a case of Wernicke-Korsakoff syndrome with accompanying ocular findings that is caused by hyperernesis gravidarum. METHODS: We experienced a 27-year-old female at 12 weeks of pregnancy, who visited our clinic because of weight loss, gait disturbance, decreased mentality and dizziness after prolonged vomiting for 2 months. Neurological examination demonstrated ataxia of gait and loss of orientation. Ophthalmologic examination showed decreased visual acuity, upbeat nystagmus, diplopia and retinal hemorrhage. RESULTS: We report a relatively rare case of Wernicke-Korsakoff syndrome with ophthalmic symptoms induced by hyperemesis gravidarum. CONCLUSIONS: If a pregnant women has symptoms of severe vomiting along with other ocular findings such as retinal hemorrhage or restricted extraocular movement, one must suspect the diagnosis of Wernicke-Korsakoff syndrome, and should start appropriate treatment immediately.
In order to support the concept that a lesion of the thalamus is sufficient to cause a Korsakoff syndrome, we are presenting 5 patients, all of whom developed the syndrome after sustaining a left (dominant) thalamic infarction. Two patients had pure thalamic strokes followed by a permanent Korsakoff syndrome. One of these patients was studied with neuropsychometric testing, as well as with a modern MRI scan. In 2 other patients, clinical and imaging data indicate that infarction was not limited to the thalamus. Another patient had bilateral thalamic infarcts but only a temporary Korsakoff syndrome. Neuropathological data are needed to elucidate the exact anatomical substrate of dominant thalamic Korsakoff syndrome.
Gastrectomy, alcoholism and malignant tumour are three predisponing risk factors for the development of Wernicke-Korsakoff syndrome. We described the clinical case of a patient with history of alcoholism that developed Wernicke-Korsakoff syndrome 30 years after undergoing gastrectomy. This patient had, in the last year, a diagnostic for prostatic adenocarcinoma and changes in dietary habits. We presented the clinical and neuropathological features of the Wernicke-Korsakoff syndrome. As well as some aspects in the treatment and prognosis.