Neuropsychiatric presentation of Wernicke–Korsakoff syndrome secondary to chronic alcohol dependence

Dear Sir,

Wernicke–Korsakoff syndrome (WKS) consists of two components Wernicke encephalopathy (WE) and Korsakoff syndrome (KS). The global prevalence of WKS ranges between 0 and 2%.[1] WE is an acute and rare neuropsychiatric condition which generally manifests in the form of a triad of ataxia, confusion, and ophthalmoplegia. The etiology of WE is very diverse, and main culprit is deficiency of vitamin B1 (Thiamine). The most common risk factor is chronic alcohol dependence which leads to reduced absorption as well as utilization of thiamine.[1] Other causes of WE include malnutrition, starvation, anorexia nervosa, schizophrenia, terminal malignancies, hyperemesis gravidarum, inflammatory bowel disease, AIDS, tuberculosis, and uremia.[1] WE is a reversible condition, and if not diagnosed and treated on time, it can lead to disabling and majorly irreversible condition known as KS.[1] KS is characterized by memory impairment, confabulation, and behavioral disturbances. Reporting a case of WKS can improve clinicians’ and resident doctors’ knowledge which may help in preventing the progression of disorder; can help in reduction of mortality and morbidity; and can help researchers and clinicians in better understanding the prevalence, risk factors, diagnostic approach, and treatment strategies. It can help to formulate better preventive strategies and more target-specific interventions.

A 35-year-old unmarried man was brought by his family members with complaints of talking irrelevantly, becoming irritable and trying to beat family members, smiling and crying without any apparent reason, sleep disturbance, reduced food intake, gesticulation, and not able to walk properly since last 2 months period with an increased severity since last 8 days. He was also exhibiting symptom of muttering to self since last 8 days period. He was first admitted in general medicine ward and later on was transferred to psychiatry ward secondary to bizarre behavior of masturbating in general medicine ward along with passing faeces in his pant. Detailed history revealed that he was consuming alcohol daily since last 15 years. He was consuming around 3 to 4 quarters country liquor per day, eye opener drink was present, and last drink was around 25 to 30 days back. There were no active withdrawal symptoms at the time of presentation. The past history of multiple uncomplicated alcohol withdrawal episodes were present in the form of hand tremors, sleep disturbance, palpitations, increased sweating, and restlessness. There was no history of complicated withdrawal episodes. On mental status examination, he was conscious but confused and confabulating regarding the current location and was neither able to correctly tell the name of city nor identify the location/hospital. He was saying that he came to police station for joining the duty sometimes at Mumbai and sometimes at Nagpur. In past, he had worked in those cities for brief periods. On asking about his muttering and masturbatory behavior, he told that he was hearing seducing voices of a few women which were not perceived by others. He was not able to communicate about his fecal incontinence. On neurological examination, he was having ataxic gait. He did not have any ophthalmic signs including ophthalmoplegia. On physical examination, he was hemodynamically stable. His blood investigations were within normal limits. Based on the clinical presentation, his provisional diagnosis was kept as Chronic Alcohol Dependence induced WKS. He was started on intravenous thiamine supplementation in the form of 300 mg thrice a day for 5 days. After 5 days, he was shifted on oral thiamine in a dose of 500 mg per day. He was also started on oral haloperidol 5 mg twice a day along with oral trihexyphenidyl 2 mg twice a day due to the psychotic/behavioral symptoms.

His magnetic resonance imaging (MRI) of the brain showed bilateral mammillary body atrophy on sagittal T1 image [Figure 1] and axial T2 FLAIR images showed prominent ventricles [Figure 2]. These MRI brain changes particularly that of mammillary body atrophy confirmed the diagnosis of WKS in index individual.

Figure 1.

Mammillary body atrophy on MRI brain sagittal T1 section

Figure 2.

Showing prominent ventricles on MRI brain axial T2 FLAIR image

Index individual gradually showed behavioral improvement as well as his gait also improved and he started walking properly over 1 week after starting the treatment.

In the present case, the index individual had 2 components of WE triad in the form of ataxia and confusion. He did not have ophthalmoplegia. According to the literature, the classic triad of WE can be found in only 10% cases.[2] He also had symptoms of KS in the form of confabulation and behavioral disturbances. His confabulating behavior also showed improvement on treatment. Although majorly irreversible, in some cases, KS is reversible.[3] According to the literature, around 68% cases of WE are either missed or misdiagnosed in individuals with alcohol dependence.[4] WE and KS can co-occur due to thiamine deficiency.[5] Thiamine deficiency leads to bilateral degeneration of projections or neurons in the Mammillary bodies which in turn leads to anterograde amnesia, confabulation, decreased motivation, and reckless behavior.[5] The main stay treatment of WKS is high dose thiamine which can be given in a dose of 500 mg to 1500 mg intravenous, three times a day for at least 3 days period.[4]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient and his brother have given their consent for patient's images and other clinical information to be reported in the journal. The patient and his brother understand that patient's name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Authors’ contributions

Concepts and design: ASG, SAT; Drafting and revision: ASG, SAT, ACJ, SBK, AAD; Final edition and approval of the revision to be published: ASG, SAT, ACJ, SBK, AAD.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.