|Year : 2015 | Volume
| Issue : 1 | Page : 99-103
Neuropsychological functioning in Wernicke's encephalopathy
Sushree Sangita Behura1, Sarada Prasanna Swain2
1 Department of Clinical Psychology, Mental Health Institute, SCB Medical College, Cuttack, Odisha, India
2 Department of Psychiatry, Centre of Excellence, Mental Health Institute, SCB Medical College, Cuttack, Odisha, India
|Date of Web Publication||16-Jul-2015|
Sarada Prasanna Swain
Department of Psychiatry, Centre of Excellence, Mental Health Institute, SCB Medical College, Cuttack - 753 007, Odisha
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Context: Wernicke's encephalopathy (WE) is caused by thiamine (Vitamin B1) deficiency and most commonly found in chronic alcoholism and malnutrition. Clinically, the key features are mental status disturbances (global confusion), oculomotor abnormalities, and gait disturbances (ataxia). Apart from these clinical features, we can find deficits in neuropsychological functioning in patients with WE, which is more prominent after the improvement in the physical conditions. Neuropsychological functioning includes both basic cognitive processes (i.e., attention-concentration) as well as higher order cognitive processes (i.e., memory, executive functioning, reasoning), which is much vital for the maintenance of quality of life of an individual. However, unfortunately, in most of the cases, neuropsychological functioning is ignored by the clinicians. Materials and Methods: In this study four case reports of WE have been presented. The patients were taken from the outdoor department of Mental Health Institute, S.C.B. Medical College, Cuttack, Odisha. Neuropsychological functioning was measured by administration of PGIBBD and Quality of Life was measured by WHO-QOL BREF Odia Version. Discussion: As described in the literature, among the three cardinal signs ( global confusion, ataxia, and ocular sings), the first two were present in all cases, but nystagmus was present in only two cases.Memory dysfunction was so disabling that the persons were unable to maintain a good Quality of Life and occupational impairment was prominent. There are disturbances in recent, remote memory, immediate recall, delayed recall, and attention and concentration, ultimately creating both physical and mental disability. PGI-BBD findings also suggest the overall impairment in neuropsychological functioning other than memory, that is, executive functioning, visual acuity, and depth perception. Findings of WHO-QOL BREF suggest the impairment of four domains of QOL in all the cases, but the severity level varies from person to person. Conclusion: Like the three cardinal features, neuropsychological dysfunction in WE should be given importance, which is a most vital component for the maintenance of QOL. As a result, the disability produced by this condition can be well managed.
Keywords: Neuropsychological functioning, thiamine (Vitamin B 1 ), Wernicke′s encephalopathy
|How to cite this article:|
Behura SS, Swain SP. Neuropsychological functioning in Wernicke's encephalopathy. Ind Psychiatry J 2015;24:99-103
Wernicke's encephalopathy (WE) is caused by thiamine (Vitamin B 1 ) deficiency, deriving from malnutrition of various sources and most commonly found in patients with chronic alcoholism. WE is a clinically underdiagnosed, acute or subacute illness that can cause permanent memory disturbance or death if proper treatment is not given in time. Thiamine deficiency can also cause cerebellar degeneration (alcoholic cerebellar degeneration, nutritional cerebellar degeneration) and neuropathy (alcohol neuropathy, thiamine deficiency neuropathy). 
Wernicke's encephalopathy was first described in 1881 by the German Physician Carl Wernicke (1848-1905) as an acute illness affecting two men with chronic alcoholism and a young woman with protracted vomiting after drinking sulfuric acid in a suicide attempt. Their symptoms and signs were acute confusion, impairment of consciousness, paresis of ocular muscles, nystagmus, and unsteady or ataxic gait. All three died after a short course of illness. Neuropathology revealed punctate hemorrhages around the third and fourth ventricles and aqueduct. Wernicke named the disease "acute superior hemorrhagic polioencephalitis." , During 1887 through 1889, a Russian Physician Sergei S. Korsakoff (1853-1900) described a similar illness in a larger group of patients with acute confusion and peripheral neuropathy. Those who survived had protracted memory disturbances with great difficulty in memorizing recent events. He named the disease "polyneuritic psychosis." 
| Materials and methods|| |
This battery developed by Dr. Dwarka Pershad and Dr. S. K. Verma. Both are Ex-additional Professors of Clinical Psychology, PGIMER, Chandigarh, and Ex-consultants of Clinical Psychology, Government Medical College and Hospital, Chandigarh, India.
- Diagnostic and Statistical Manual of Mental Disorders, 5 th Edition Criteria of American Psychiatric Association (2013)
- WHO-QOL BREF- Odia Version: This is developed by Quality of life Research and Development Foundation (2008), consisting of 26 items.
- PGI battery of brain dysfunction (PGI-BBD).
PGI battery of brain dysfunction is proposed to be used to quantify cognitive dysfunction/impairment/decline/deficit (1) for clinical purposes, (2) to evaluate extent of decline or loss in cognitive area as a result of illness/accident/injury/natural calamities, etc., (3) to plan rehabilitatory strategy, (4) helping judiciary in taking decision about extent of decline for grant of compensation, (5) to plan training/treatment, (6) for the evaluation of outcome of the treatment/management.
The battery consists of five subtests and 19 variables:
- PGI-memory scale consists of 10 variables
- Battery of performance tests of intelligence consists of 2 variables
- Verbal adult intelligence scale (VAIS) consists of 5 variables
- Nahor Benson Test (NBT) consists of 1 variable
- Bender Visual Motor Gestalt Test consists of 1 variable.
A Hindu male aged 36 years, an auto driver by profession was addicted to alcohol since last 20 years was admitted in the hospital (Indoor of Psychiatry, Mental Health Institute, SCB Medical College, Cuttack, Odisha, India) with complaints of confusion in daily routine activities, irritability, aphasia, ataxia, urinating in the bed most of the time at night, unsteady gait, memory impairment (immediate and recent), forgetfulness, and difficulty in the initiation of sleep and loss of appetite. On examination, there is dysfunction in the Lever function test (↑AST, ↑ALT, ↑GGT, ↑Alkaline phosphatase with fatty changes in the liver). After improvement in the physical conditions, thiamine injection was administered in the doses of 200 mg/day around 1 month. After that, in order to assess the neurocognitive functioning of the patient, PGI-BBD was tried. However, due to the presence of aphasia, visuo-motor incoordination, unsteady gait, the patient did not cooperate for the test.
He was treated for 1 year with donepezil, thiamine, and benzodiazepines with follow-up in every month at the OPD. Neuropsychological evaluation was done after 1 year by the administration of PGI-BBD.
The findings of PGI-BBD are as follows: Impairment in recent and remote memory, immediate recall, delayed recall, attention and concentration, visual retention, recognition, retention for similar and dissimilar pairs. Impairment in memory scale indicates the involvement of temporal lobe, impaired performance IQ revealed the involvement of frontal lobe, which suggests the patient has difficulty in abstract reasoning and practical ability. Dysfunction in NBT suggested difficulty in visual acuity and depth perception. Visuo-motor in coordination and organicity were confirmed from Bender Gestalt Test. VAIS was not administered because of persistent motor aphasia of the patient, while there was no indication of sensory aphasia. Even after the adequate treatment of the patient, the improvement in the neurocognitive function was very slow. Computed tomoth graph y (CT) scan findings revealed lesion in the frontotemporal region.
A Hindu male, aged 40 years, an industrial worker in a steel plant, belongs to a tribal community of the state, where alcohol (Handia) was regularly consumed by almost all adult males and females in the evening time after returning from the work as their sociocultural customs. The person after returning from the work started consuming alcohol from his adolescent age, at the age of 16, before admitted to a tertiary care hospital (Mental Health Institute, SCB Medical College, Cuttack, Odisha, India). He had complaints of confusion in daily routine activities in social and occupational sphere (the confusion is such an extent that at times he was unable to reach at home in the evening after returning from the plant work). He had ataxic gait, diminished vision, forgetfulness, confabulation, neuropathic pain all over the body, agitated, episodic seizure once. For all the above symptoms, he was admitted in the hospital. On investigation, there was liver dysfunction (↑ALT, ↑GGT, ↑Serum alkaline phosphatase, and fatty changes in the liver), neuropathic pain in all four limbs. The neuropsychological assessment was tried after the improvement of his physical conditions (around 1 month). However, the patient did not cooperate at that time. After 2 months of discharge, neuropsychological assessment was done. PGI-BBD findings revealed impairment in memory, verbal and performance intelligence quotient, disturbances in visual acuity and depth perception, as well as disturbances in visuo-motor coordination, which indicates there may be the involvement of all the four lobes of the brain. On ophthalmological investigation, there was dysfunction of refractive error. However, there was no optic atrophy or cranial nerve palsy as examined by the ophthalmologist. CT scan findings are suggestive of cortical atrophy.
A Hindu male, aged 55 years, educated up to third standard belonging to lower socioeconomic status, who was working as a mechanic in a motor garage. He was addicted to alcohol from his adolescence age of 13 years. He was abdicated to beer (Local name-Aska 40), Whisky-40% of alcohol, and Rum-45% of alcohol and was taking very often with his friends. In the initial years of addiction, the frequency of consuming alcohol was 3-4 times/week. However, after 5 years his frequency had been increased to almost every day. He was admitted in the hospital with complaints of grandiose ideas, aggressive behavior, paranoid ideas, confusion, and ataxia. On examination, he had liver dysfunction (↑ALT, ↑AST, ↑ALK. Phos. and ↑ GGT). On CT scan, a hypodense lesion was seen in the left temporal lobe which was a small infarct and diffuse hypodensity in bilateral periventricular area suggesting ischemic changes was found, whereas lateral and third ventricle was normal. After improvement of his physical conditions, he was discharged from the hospital and reported after 2 months. After 2 months, there were complaints of forgetfulness in his daily routine and occupational activities, persistent paranoid ideas, and minor mistakes in his occupational work. There was no ataxia, but diminished vision was there which is because of refractive error, but not due to optic nerve atrophy (as examined by the ophthalmologist).
To assess the neurocognitive status, PGI-BBD was administered. The findings were as follows:
There were dysfunctions in the immediate, recent, remote memory, and recognition, whereas minimal impairment in attention and concentration, retention for dissimilar pairs, and visual retention. In verbal intelligence scale, there were minimal impairment in information, digit span, and arithmetic. Slight deviation in dysfunction rating score on NBT suggested the disturbances in visual acuity and depth perception. Organicity and disturbances in visuo-motor coordination were confirmed by Bender Gestalt test.
A Hindu male, aged 48 years, educated up to graduation, worked as a store keeper in Indian Army, was addicted to alcohol from his age 33. He was taking around 180-200 ml alcohol (Rum: Containing 45% of alcohol). Being the store keeper in the army, he had easily accessible to alcohol and was consumed very often almost every day at evening time. He was admitted in the drug de addiction center with complaints of global confusion, gait disturbance (ataxia), forgetfulness in daily activities, doing minor mistakes in daily routine activities, highly irritable, and restless. On examination, there was liver dysfunction (↑ALT, ↑AST, ↑ALK. Phos. and ↑ GGT and there was a fatty change in the liver). There was no abnormality detected on CT scan.
After treatment in the drug de addiction center, he was discharged. After 2 months to assess the Neurocognitive functions, PGI-BBD was administered and results are as follows:
There were great deviations in delayed recall, attention and concentration retention for dissimilar pairs, whereas little deviations in remote memory, mental balance, immediate recall, visual retention, and recognition. However, there was no impairment in recent memory. His intelligence was found to be decreased from the previous level. The results in NBT also revealed the disturbances in visual acuity and depth perception. No abnormality was detected in CT scan findings.
| Discussion|| |
Thiamine plays a vital role in the metabolism of carbohydrates. It is a cofactor for several essential enzymes, in deficiency of which cellular system begins to fail, resulting eventually cell death. Because these enzymes play an essential role in cerebral energy utilization, thiamine deficiency may propagate brain tissue injury by inhibiting metabolism in brain regions with higher metabolic demands and high thiamine turnover. 
In chronic alcoholics with high metabolic requirements and inadequate stores of thiamine, energy production drops and neuronal damage ensues. Increased cell death then feeds the localized vasogenic response.  Additionally, reduced production of succinate, which plays a role in gamma-aminobutyric acid metabolism and electrical stimulation of neurons, lead to further central nervous system injury.
In chronic alcoholics, there always occurs a substantial thiamine deficiency because it affects thiamine uptake and utilization. In long-term alcoholics, malnutrition can reduce intestinal thiamine absorption by 70%, decreasing serum levels of thiamine to between 30% and 98% below the lower level established for normal subjects. Thiamine acts as a coenzyme in the metabolism of glucose and lipids and as stores of water soluble vitamins are limited in the body, deficiency can manifest within 2-3 weeks of cessation of intake. ,
Chronic alcohol consumption in other ways induce thiamine deficiency through several potential mechanism: Genetic predisposition, replacement of vitamin-containing foods by the high calorific value of alcohol, impaired absorption of thiamine from the gut, impairment of storage by the liver, thiamine transport problems, other nutritional deficiencies, decreased phosphorylation to thiamine pyrophosphate, and excessive requirement for the metabolism of alcohol. 
In all cases of WE described above, global confusion and ataxic gait were present at the time of admission which was improved after subsequent interventions. As described in the literature, among the three cardinal signs, that is, global confusion, ataxia, and ocular sings, the first two were present in all cases, but nystagmus was present in only two cases.  The defect in the memory function which is the most vital component for maintenance of quality of life (QOL) was assessed by PGI-BBD after improvement of the physical conditions. The typical Korsakoff memory defect was clearly evident from the outset, being at the time of initial examinations. However, in all testable cases, the assessment of memory function was not possible after improvement of physical condition because of overlapping of symptoms of global confusion and memory dysfunction. It was very hard to determine at which point confusion of thought recedes and the memory defect becomes the most prominent sign.
In case 1 and 2, memory dysfunction was so disabling that the persons were unable to maintain a good QOL and occupational impairment was prominent. Administration of thiamine in early stage improves the ataxia and ophthalmoplegia, but confusion and neurologic dysfunction persisted for a long time, which was evident from the neuropsychological assessment. , Although confusion was disappeared in later phase, but the memory function and learning of new things appear to be impaired and dysfunction in these two spheres lasted for a long time.
The CT scan findings revealed lesions in the different parts of the brain, which was different in all the four cases. The findings suggested the lesions in the frontal and temporal lobe in two cases, cortical atrophy in one case and no abnormality was detected in the last case.
The dysfunction in memory, attention and concentration, delayed recall, intelligence were persisted for a long time even after treatment. Studies suggest that up to 80% of patients of WE, may not be diagnosed at early stages, which make estimates of morbidity rates unreliable.  The classical three components of WE presentation may not be associated with classical clinical triad up to 90% cases.
Other than the cardinal features of WE, there is a definite deficit in neuropsychological functioning in all the cases. Memory dysfunction is present in all the cases, which is confirmed from PGI Memory Scale. In most of the cases, there are disturbances in recent, remote memory, immediate recall, delayed recall, and attention and concentration. This memory dysfunction causes impairment in their social and occupational life and ultimately creating both physical and mental disability. Information processing (ability to learn new things) is also affected in most of the cases, which is one of the important components of daily living. PGI-BBD findings also suggest the overall impairment in neuropsychological functioning other than memory, that is, executive functioning, visual acuity, and depth perception. Dysfunction in neuropsychological functioning hampers the QOL of an individual. QOL is an important parameter that provides an insight into how a disorder impacts the life of those affected. QOL, a concept situated between social and clinical sciences, is a pertinent indicator to evaluate the subjective experience of the patient and to quantify the psychosocial burden of alcoholism. , Among various psychiatric conditions, alcohol-related disorders, that is, WE, significantly affect QOL, but this area has not been extensively studied. Findings of WHO-QOL BREF suggest the impairment of four domains of QOL in all the cases, but the severity level varies from person to person.
Because WE is a reversible condition, the diagnosis in early stage and subclinical stages is highly essential, mostly in all chronic alcoholics and administration of thiamine intramuscularly (200-300) mg per day for (3-5) days should be the part of community detoxification program in all communities, which will improve the patient's metabolic, memory functioning, and neurological status. 
| Conclusion|| |
Like the three cardinal features, memory dysfunction in WE should be given importance, which is a most vital component for the maintenance of QOL.
As a result, the disability produced by this condition can be well managed. By reviewing the different literatures and scientific articles from PubMed, Medscape, Cochraine, and IndMed, it has been concluded that around 70-80% of cases of WE, the three cardinal features were not present, which corroborates with the findings of our cases. The memory functioning, that is, including immediate, recent and remote is a vital component of WE has been concluded from our study. In addition, attention-concentration, visuo-spatial ability, executive functioning, and visuo-motor coordination have been impaired to different degrees in different cases according to the duration, frequency, amount, and nutritional status of the chronic alcoholics.
Wernicke's encephalopathy is a significantly disabling and potentially lethal condition that can be prevented if identified and treated early in the course. Those who are chronic alcoholics should be screened out at an early stage. Institutionalization, discharge, and follow-up arrangements for such individuals may be as important as thiamine replacement. Monitoring the level of thiamine in alcoholics should be an important component of all consultations either in the hospital or in DDC. In chronic alcoholics screening for neuropsychological functioning should be a routine in clinical practice, which is the sole component of QOL and daily living. In current practice, the QOL of alcohol-dependent patients is not measured systematically, even though this is relevant to the psychosocial context of intervention. , QOL should be extensively studied in order to evaluate the subjective experiences of the patient and to quantify the psychosocial burden of chronic alcoholism. Awareness in the society regarding alcoholism can be act as a protective factor.
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