We rediscovered a phenotype of AD known in the early 1900s as presbyophrenia, but then forgotten, and renamed as confabulation-misidentification phenotype. The phenotype includes diencephalic amnesia whose prototype is Korsakoff syndrome. The main features are anterograde and retrograde amnesia with marked disorientation and confabulation, executive impairments, reduced insight and attention deficits, misidentification, minor hallucination and other delusions, behavioral disturbances, and early anxiety. In this article, we summarize what we have discovered about the new phenotype and what is still missing to confirm this diencephalic variant of AD.

Personality research is of relevance because it provides insights into the psychological strengths and vulnerabilities of a person. Korsakoff\'s syndrome (KS) is a severe neuropsychiatric disorder following acute thiamine deficiency, usually as a consequence of alcohol-use disorders. Research on personality traits of KS patients is currently limited. The aim of the current exploratory study was therefore to examine the personality traits and problems of KS patients. We assessed self-reported and informant-reported personality traits and problems in institutionalized KS patients (n = 30). Results indicate that the psychotic personality organization was more frequently present in KS patients compared to a psychiatric reference population, leading to increased vulnerability for the development of severe psychiatric issues. Informants observed more premorbid and current cluster B (\"emotional\") and C (\"anxious\") personality behavior in comparison to the general population. Also, rigid- and socially avoidant behavior in KS patients was observed to increase over the years. They also observed personality problems such as emotional-, unpredictable-, dramatic-, anxious and fearful behavior in the present and in the past. KS patients reported themselves as healthy individuals, indicating a lack of self-awareness. We recommend training programs for the medical team focussed on psycho-education and supportive interventions for patients with complex personality problems concomitant to KS.

We report a child with steroid-dependent nephrotic syndrome presenting with excessive irritability, double vision and inability to walk for 5 days. On examination, the child was irritable with Glasgow coma sccale (GCS of 12/15, had bilateral convergent squint (R>L), vertical nystagmus, ataxia without any focal neurological deficits and normal fundus. MRI brain with venogram showed bilateral symmetric FLAIR hyperintensity in the medial thalamus and periaqueductal grey matter showing diffuse restriction with normal venogram. A possibility of Wernicke encephalopathy (WE) was considered and the child was started on thiamine supplementation, following which he had significant improvement in his symptoms. His irritability reduced with significant improvement in the range of eye movements and vertical nystagmus. At 3-month follow-up, the child is asymptomatic with normal gait. Although WE is uncommon in children with nephrotic syndrome, the possibility has to be kept in mind when a child presents with atypical neurological symptoms.

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BACKGROUND: While prospective memory (i.e., the ability to execute a future plan) has been extensively researched, little is known about negative prospective memory (i.e., the ability to remember not to execute a future plan) in Korsakoff\'s syndrome (KS). We thus evaluated both prospective memory and negative prospective memory in KS, as well as the relationship between both types of prospective memory and inhibition.
METHODS: Patients with KS and control participants were invited to press a button on the keyboard (i.e., prospective memory) or not to press a button when a cue word (e.g., negative prospective memory) was encountered.
RESULTS: The analysis demonstrated that patients with KS had more prospective memory errors (i.e., forgetting to click the keyboard when the instruction was to do so) than control participants. The analysis also demonstrated that patients with KS had more negative prospective memory errors (i.e., clicking the keyboard when the instruction was not to do so) than control participants. Similar levels of commissions and omissions were observed in patients with KS than in control participants. Interestingly, prospective and negative prospective performances were significantly correlated with performance on an inhibition assessment task.
CONCLUSIONS: Our study demonstrates that KS is characterized by not only difficulty in remembering to execute a future intention but also by difficulty in remembering not to execute a future intention. A decline in negative prospective memory in KS can be associated with difficulty in inhibiting no longer appropriate previously learned intentions.

Wernicke encephalopathy is an emergent neurological disorder caused by vitamin B1 (thiamine) deficiency. Here, we present a case of Wernicke encephalopathy in a male patient in his 70s with normal serum thiamine levels and MRI findings on admission. He had a history of heavy alcohol consumption and a gradual decrease in food intake. On arrival at the hospital, his consciousness was impaired which persisted even after glucose replacement. Moreover, horizontal nystagmus and cerebellar ataxia were observed. Head CT scan and MRI revealed no abnormal findings. Further, his serum thiamine level was within the normal range. The patient was clinically diagnosed with Wernicke encephalopathy, and high-dose thiamine therapy was started. Then, his symptoms improved immediately. Thus, in case of clinical suspicion, treatment for Wernicke encephalopathy must be initiated promptly even in patients with normal serum thiamine levels.

OBJECTIVE: To conduct a nationwide retrospective cohort study to assess trends and hospitalization-associated outcomes in patients with Wernicke encephalopathy.
METHODS: In this nationwide retrospective cohort study, we used in-hospital claims data of patients hospitalized with Wernicke encephalopathy in Switzerland from January 1, 2012, to December 31, 2020. We estimated incidence rates per 100,000 person-years among the overall Swiss population stratified by alcohol and non-alcohol-induced Wernicke encephalopathy. The primary outcome was all-cause in-hospital mortality. Secondary outcomes included progression to Korsakoff syndrome and 1-year hospital readmission. We estimated odds ratios (ORs) for binary outcomes.
RESULTS: It was found that 4098 of 4393 hospitalizations (93.3%) for Wernicke encephalopathy during the 8-year study were alcohol-related. Incidence rates for hospitalizations were 14-fold higher in alcohol-related compared with non-alcohol-related Wernicke encephalopathy (5.43 vs 0.39 per 100,000 person-years). The risk for in-hospital mortality was significantly lower in patients with alcohol-related vs non-alcohol-related Wernicke encephalopathy (3.2% vs 8.5%; adjusted OR, 0.38; 95% CI, 0.23 to 0.62). Patients with alcohol-related Wernicke encephalopathy had higher risk for development of Korsakoff syndrome (16.9% vs 1.7%; adjusted OR, 10.64; 95% CI, 4.37 to 25.92) and 1-year hospital readmission (31.6% vs 18.7%; adjusted OR, 1.4; 95% CI, 1.04 to 1.88).
CONCLUSIONS: In this Swiss nationwide cohort study, Wernicke encephalopathy was a rare but serious cause for hospitalization and mainly alcohol-related. Patients with alcohol-related Wernicke encephalopathy had lower risks of in-hospital mortality but were more likely to develop Korsakoff syndrome and be readmitted to the hospital.

BACKGROUND: Autoimmune encephalitis is a rare immune-related adverse event of PD-1 inhibitors, nivolumab and pembrolizumab. Autoimmune hypophysitis can also be seen with the use of these agents. The relationship between these two phenomena is currently unknown.
METHODS: We describe a 79-year-old man with anterior scalp melanoma who received adjuvant nivolumab therapy. Sixteen weeks after the completion of nivolumab therapy, the patient presented to the hospital with altered mental status, anterograde amnesia, and symptoms of nausea and vomiting. The patient\'s encephalopathy was associated with confabulations. Workup identified increased CSF protein without increased cellularity, along with decreased serum cortisol and ACTH levels. This was consistent with encephalitis and central adrenal insufficiency.
METHODS: The patient had a robust clinical response to steroids, with resolution of mental status changes and normalization of blood pressure. He continues to receive maintenance steroid therapy without any further symptoms six months later.
CONCLUSIONS: We report herein a unique case of encephalopathy in the setting of nivolumab use for the treatment of melanoma. The condition resembled Korsakoff psychosis seen in the setting of alcoholism and was associated with central adrenal insufficiency. A prompt response to steroids was both diagnostic and therapeutic in our case, suggesting the resolution of autoimmune phenomena related to nivolumab.

Thiamine (vitamin B1) deficiency is relatively common in patients with kidney disease. Wernicke\'s encephalopathy (WE) is caused by vitamin B1 deficiency. Our aim was to systematically review the signs and symptoms of WE in patients with kidney disease. We conducted a systematic literature review on WE in kidney disease and recorded clinical and radiographic characteristics, treatment and outcome. In total 323 manuscripts were reviewed, which yielded 46 cases diagnosed with acute and chronic kidney disease and WE published in 37 reports. Prodromal characteristics of WE were loss of appetite, vomiting, weight loss, abdominal pain, and diarrhea. Parenteral thiamine 500 mg 3 times per day often led to full recovery, while Korsakoff\'s syndrome was found in those receiving low doses. To prevent WE in kidney failure, we suggest administering high doses of parenteral thiamine in patients with kidney disease who present with severe malnutrition and (prodromal) signs of thiamine deficiency.

OBJECTIVE: Wernicke-Korsakoff syndrome (WKS) is a neurological disorder caused by the lack of thiamine (vitamin B1) and includes Wernicke encephalopathy and the later stage of Korsakoff amnesic syndrome. WKS could be secondary to malnutrition, including Hyperemesis Gravidarum (HEG), which is characterized by severe vomiting during pregnancy. We present a case of HEG resulting in suspected WKS where neuropsychological evaluation clarified cognitive status with complex cultural, language, and educational factors.
METHODS: Repeat evaluations of a 29-year-old West-African* woman with mental status change, decreased verbal output, agitation, and inability to walk in the context of HEG. Initially referred for inpatient neuropsychological evaluation at 21 weeks gestation. MRI demonstrated mild diffuse cerebral atrophy and symmetric foci of T2 FLAIR hyperintensities in periaqueductal gray matter, hypothalamus, bilateral thalami, perirolandic cortex, and bilateral mammillary bodies.
RESULTS: Initial inpatient testing with a face-to-face language congruent interpreter demonstrated notable multidomain impairment. Possible Intellectual Disability was considered by the referring team and test performances. Attempts at re-evaluation were limited by medical complications and prolonged hospitalization. Repeat inpatient evaluation 5 months later following birth of healthy child and initiation of stimulant medication, demonstrated profound memory impairment but otherwise improved cognitive capacity and engagement. Results aided in discharge planning including cognitive remediation with goal of improving functional independence.
CONCLUSIONS: Wernicke-Korsakoff syndrome due to Hyperemesis Gravidarum is an uncommon but known complication that can have profound acute and long-term consequences. This case demonstrates that repeat neuropsychological evaluations can clarify cognitive status in complex medical/cultural/language situations. *(country of origin and primary language not provided for patient privacy).