Chronic Wernicke Research Articles

Over a Century of Study and Still Misunderstood: Recognizing the Spectrum of Acute and Chronic Wernicke–Korsakoff Syndrome

The aim of this study was to objectively evaluate the hypothesis that the neuropsychological presentation of Korsakoff’s syndrome, the chronic phase of Wernicke–Korsakoff syndrome (WKS), is invariably a severe, selective amnesia against a background of relatively preserved general intellectual functions in a consecutive clinical sample. An analysis of the neuropsychological profiles of nine cases with a recorded history of WKS was undertaken. All cases were adult males (ages 32 to 70) with a long history of alcohol use disorder. Eight cases were chosen retrospectively on a consecutive basis from patient referrals. One additional case was recruited prospectively. Conventional understanding and some current opinion of Korsakoff’s syndrome predicts anterograde memory to be consistently more impaired than other cognitive abilities, but this was not found in this case series. The Mean Wechsler Delayed Memory Index was not significantly different from the Wechsler Full-Scale IQ (FSIQ), p = 0.130. Regression of Delayed Memory on FSIQ produced a non-significant intercept, p = 0.213. The ‘hallmark’ criterion of anterograde memory score at least 20 points less than intelligence score was observed in four of eight cases with available data, equating to a ‘sensitivity’ of 50%. Three of eight cases with available data had an FSIQ less than the memory score. Contrary to a common view, general intellectual function was not consistently preserved in Korsakoff’s syndrome relative to memory function. This study illustrates one of the specific merits of case series, namely, to critique an established view. Clinicians and researchers should expand their diagnostic criteria for Korsakoff’s syndrome to include more variable cognitive phenotypes, including a potentially reversible dementia-like impairment of variable severity, and focus on potential treatment opportunities.

Imaging of semantic association judgements reveals areas of functional disruption and compensation remote from lesion in chronic Wernicke's aphasia

Imaging of semantic association judgements reveals areas of functional disruption and compensation remote from lesion in chronic Wernicke's aphasia

Arterial spin labelling shows functional depression of non-lesion tissue in chronic Wernicke's aphasia

Behavioural impairment post-stroke is a consequence of structural damage and altered functional network dynamics. Hypoperfusion of intact neural tissue is frequently observed in acute stroke, indicating reduced functional capacity of regions outside the lesion. However, cerebral blood flow (CBF) is rarely investigated in chronic stroke. This study investigated CBF in individuals with chronic Wernicke's aphasia (WA) and examined the relationship between lesion, CBF and neuropsychological impairment.Arterial spin labelling CBF imaging and structural MRIs were collected in 12 individuals with chronic WA and 13 age-matched control participants. Joint independent component analysis (jICA) investigated the relationship between structural lesion and hypoperfusion. Partial correlations explored the relationship between lesion, hypoperfusion and language measures.Joint ICA revealed significant differences between the control and WA groups reflecting a large area of structural lesion in the left posterior hemisphere and an associated area of hypoperfusion extending into grey matter surrounding the lesion. Small regions of remote cortical hypoperfusion were observed, ipsilateral and contralateral to the lesion. Significant correlations were observed between the neuropsychological measures (naming, repetition, reading and semantic association) and the jICA component of interest in the WA group. Additional ROI analyses found a relationship between perfusion surrounding the core lesion and the same neuropsychological measures.This study found that core language impairments in chronic WA are associated with a combination of structural lesion and abnormal perfusion in non-lesioned tissue. This indicates that post-stroke impairments are due to a wider disruption of neural function than observable on structural T1w MRI.

Impaired speech perception leads to poor semantic access: ERP evidence from individuals with pathologically or artificially impaired speech perception.

Impaired speech perception leads to poor semantic access: ERP evidence from individuals with pathologically or artificially impaired speech perception.

Classification of Mental Disorders of Late Age

Aging is accompanied by the rise of mental health problems and dementia. Their number by 2050 could reach 120 million people. The absens of classification of mental disorders of late age prevents from their correct diagnosis, forensic psychiatric assessment, promotes ageism. Kraepelin (1900) placed them in the category 'Interior body disease processes', and subdivided into 'arteriosclerotic', 'presenile psychosis of the age reverse development' and 'senile mental disorders'. Bleuler (1920) attributed them to 'Acquired psychosis with gross brain disorders. Organic syndrome'. Sternberg (1977) distinguished functional, vascular and atrophic forms. Shakhmatov (1996) believed that the classification of mental disorders of late age should basically be determined by the presence or absence of dementia. In ICD-9, ICD-10, DSM-5 there is no special section on mental disorders of aging. As a result there is a loss of such unique and specific syndromes of involution, as pseudoparalytic, frontal, paraphrenic disoders, acute and chronic Wernicke presbyophrenia, 'functional', delirium (involutional paranoid) and hallucinatory (Akboma dermatozoic delusion) psychosis of later age. ICD-10 consides a state of R54 'Old Age', but without pairing with category 'Mental disorders'. In the DSM-Y there is no section on mental disorders of old age, the only innovation is ontogenetic approach in the description of mental disorders, depending on different age periods and renaming of dementia into neurocognitive disorders, aimed at destigmatization of patients. The pilot version of the section 'Mental disorder' in the ICD-11 presents the category of 'Old age'. We believe that it is reasonable to classify 'Behavioral and mental disorders of late age' in this variant.

The anterior temporal lobes support residual comprehension in Wernicke’s aphasia

Wernicke's aphasia occurs after a stroke to classical language comprehension regions in the left temporoparietal cortex. Consequently, auditory-verbal comprehension is significantly impaired in Wernicke's aphasia but the capacity to comprehend visually presented materials (written words and pictures) is partially spared. This study used functional magnetic resonance imaging to investigate the neural basis of written word and picture semantic processing in Wernicke's aphasia, with the wider aim of examining how the semantic system is altered after damage to the classical comprehension regions. Twelve participants with chronic Wernicke's aphasia and 12 control participants performed semantic animate-inanimate judgements and a visual height judgement baseline task. Whole brain and region of interest analysis in Wernicke's aphasia and control participants found that semantic judgements were underpinned by activation in the ventral and anterior temporal lobes bilaterally. The Wernicke's aphasia group displayed an 'over-activation' in comparison with control participants, indicating that anterior temporal lobe regions become increasingly influential following reduction in posterior semantic resources. Semantic processing of written words in Wernicke's aphasia was additionally supported by recruitment of the right anterior superior temporal lobe, a region previously associated with recovery from auditory-verbal comprehension impairments. Overall, the results provide support for models in which the anterior temporal lobes are crucial for multimodal semantic processing and that these regions may be accessed without support from classic posterior comprehension regions.

Coverbal gestures in the recovery from severe fluent aphasia: A pilot study

This post hoc study investigated coverbal gesture patterns in two persons with chronic Wernicke's aphasia. They had both received therapy focusing on multimodal communication therapy, and their pre- and post-therapy verbal and gestural skills in face-to-face conversational interaction with their speech therapist were analysed by administering a partial barrier Referential Communication Task (RCT). The RCT sessions were reviewed in order to analyse: (a) participant coverbal gesture occurrence and types when in speaker role, (b) distribution of iconic gestures in the RCT communicative moves, (c) recognisable semantic content, and (d) the ways in which gestures were combined with empty or paraphasic speech. At post-therapy assessment only one participant showed improved communication skills in spite of his persistent language deficits. The improvement corresponded to changes on all gesturing measures, suggesting thereby that his communication relied more on gestural information. No measurable changes were observed for the non-responding participant—a finding indicating that the coverbal gesture measures used in this study might account for the different outcomes. These results point to the potential role of gestures in treatment aimed at fostering recovery from severe fluent aphasia. Moreover, this pattern of improvement runs contrary to a view of gestures used as a pure substitute for lexical items, in the communication of people with severe fluent aphasia.Learning outcomes: The readers will describe how to assess and interpret the patterns of coverbal gesturing in persons with fluent aphasia. They will also recognize the potential role of coverbal gestures in recovery from severe fluent aphasia.

Treatment-induced neuroplasticity following intensive naming therapy in a case of chronic Wernicke's aphasia

Background: Renewed interest in the effects of intensity on treatment has led to development of short-term, intensive treatment protocols, such as Constraint-Induced Language Therapy (CILT), in which participants with chronic aphasia begin to show statistically significant language improvements in as little as 2 weeks. Given its relatively short treatment cycle, CILT is also a good choice of treatment methodology for studying brain/behaviour plasticity in post-stroke aphasia. Aims: This study aimed to examine differences between two short, intensive treatment protocols in a participant with chronic Wernicke's aphasia both in terms of treatment outcomes and changes in patterns of BOLD signal activation. Methods & Procedures: The participant (ACL) participated in language testing and an fMRI overt speech confrontation-naming paradigm pre and post 2 weeks of CILT, post 2 weeks of unconstrained language therapy (PACE), and 6 months post-CILT. He named 48 black/white line drawings from each of four conditions: treated (CILT or PACE), untreated, or consistently correctly named pictures. Outcomes & Results: Naming treated pictures improved, even in the scanner, while naming untreated pictures did not. About one third of PACE and three-fourths of CILT gains were maintained. Rather than a distinct pattern of activation distinguishing treated from untreated or CILT from PACE pictures, ACL recruited a frontal network during naming of all pictures that included left middle and inferior frontal cortex, SMA and pre-SMA, and that varied in spatial extent and degree of activation according to accuracy and performance expectation. In post-hoc analyses of accuracy, this frontal network was most active during incorrect trials. At 6 months post-CILT, compared to controls, incorrect naming recruited a large and significant bilateral network including right Wernicke's area homologue. Conclusions: Results suggest that short, intensive therapy can improve naming and jumpstart language recovery in chronic aphasia, whether responses are constrained to the speech modality or not. Modulation of a left frontal network was associated with accuracy in naming and may represent compensatory adaptation to improve response selection, self-monitoring, and/or inhibition.

Size of Mamillary Bodies in Health and Disease: Useful Measurements in Neuroradiological Diagnosis of Wernicke's Encephalopathy

As the resolution of noninvasive neuroimaging techniques improves, small structures such as the mamillary bodies can be visualized and measured. The mamillary bodies are pathologically small in a number of neurological disorders, the most common and important is chronic Wernicke's encephalopathy (WE), or the Wernicke-Korsakoff syndrome (WKS), as it is often called. This disorder is caused by vitamin B1 deficiency (thiamin) and is seen most commonly in people who drink excessive amounts of alcohol. The disorder is easily preventable by using oral or parenteral thiamin. The aim of this study was to establish a range for the volume of the mamillary bodies in normal and in various disease states, particularly WE. Brains were taken from 2212 sequential autopsies performed at the New South Wales Institute of Forensic Medicine from 1996 through 1997. After fixation in 10% formalin, the brains were sectioned coronally and a block containing the mamillary bodies was dissected. The maximum vertical and transverse diameters of the mamillary bodies were measured using Mitutoyo vernier callipers and the volume calculated (V = 4/3 (a2b), where a and b are the vertical and transverse radii, respectively). There were 164 cases with significant pathological changes in the mamillary bodies. These included cases with WE (25), Alzheimer's discase (10), infarction (11), and trauma (55). All but two of the WE cases were chronic or acute on chronic. The mean volume of the mamillary bodies was reduced by 60% in cases with chronic WE and by 25% in cases with Alzheimer's disease. In normal cases, there was a significant age-related reduction in volume, and males had larger mamillary bodies than females. Cases with alcoholic cirrhosis of the liver had normal mamillary body volumes. There is a gross shrinkage of the mamillary bodies in cases of chronic WE, but clinicians need to consider other diagnoses, such as Alzheimer's disease, which can also result in shrinkage. These quantitative data will be helpful in the neuroradiological diagnosis of some of these disorders, particularly WE.

Mamillary body atrophy in Wernicke's encephalopathy: antemortem identification using magnetic resonance imaging.

We used magnetic resonance imaging to determine the volume of the mamillary bodies in 9 patients with chronic Wernicke's encephalopathy, 7 patients with presumed Alzheimer's disease, and 37 control patients. The mean mamillary body volume (+/- standard error) was 21.3 +/- 5.8 mm3 in Wernicke patients, 40.1 +/- 3.7 mm3 in Alzheimer patients, and 51.7 +/- 2.5 mm3 in control patients. Seven of nine (78%) patients with chronic Wernicke's encephalopathy had smaller mamillary bodies than 36 of 37 control patients and 7 of 7 Alzheimer patients. The decrease in mamillary body volume was related neither to patient age nor to degree of ventricular enlargement, and most likely reflects the mamillary body atrophy that is grossly apparent at autopsy in up to 81% of Wernicke patients. This technique provides a means of identifying the most specific macroscopic lesion of chronic Wernicke's encephalopathy.

The incidence of Wernicke's encephalopathy in Australia--a neuropathological study of 131 cases.

In a nine year necropsy study in Western Australia, the incidence of Wernicke's encephalopathy was 2.8%. The incidence appears to be increasing. Although Wernicke's encephalopathy is a nutritional disorder, the majority of cases occur in the alcoholic population. Only 20% of the 131 cases studied had been diagnosed clinically as Wernicke's encephalopathy. This large discrepancy between numbers of cases diagnosed clinically and pathologically suggests that chronic Wernicke's encephalopathy, which comprised 83% of the cases, may be the end result of repeated subclinical episodes of Wernicke's encephalopathy. Thus, Wernicke's encephalopathy could be considered a "progressive" disorder and as patients respond well to thiamine replacement therapy, early diagnosis is important. Alternatively, prevention by vitamin enrichment of alcoholic beverages may have to be considered in an attempt to minimise the social and economic impact of Wernicke's encephalopathy on Western society.

A disease in infants resembling chronic Wernicke's encephalopathy

Summary 1.Three cases are reported of childrensuccumbing to a disease process resembling Wernicke's encephalopathy. 2.There is no convincing evidence of a dietary inadequacy of thiamine or other vitamins, nor of a difficulty with intestinal absorption, nor of an abnormally high thiamine requirement in these cases. 3.Two of the patients were siblings of a consanguineous marriage. It is suggested that a congenital metabolic disorder, possibly involving the utilization of thiamine or in the enzymatic processes with which thiamine derivatives are concerned, may be present in these cases. Summary 1.Three cases are reported of childrensuccumbing to a disease process resembling Wernicke's encephalopathy. 2.There is no convincing evidence of a dietary inadequacy of thiamine or other vitamins, nor of a difficulty with intestinal absorption, nor of an abnormally high thiamine requirement in these cases. 3.Two of the patients were siblings of a consanguineous marriage. It is suggested that a congenital metabolic disorder, possibly involving the utilization of thiamine or in the enzymatic processes with which thiamine derivatives are concerned, may be present in these cases.