Fahr's disease with neuropsychiatric symptoms and intermittent course: a case report.

Fahr's disease is a rare neurodegenerative disorder with brain calcifications and neuropsychiatric symptoms. It can have variable phenotypic expression and intermittent symptomatology, making diagnosis challenging. In this report, we describe a young female patient presenting with symptoms of psychosis and confusion, which could be indicative of a delirium superimposed on the cerebral vulnerability associated with Fahr's disease. Notably, about two years prior, she experienced multiple episodes of tonic-clonic seizures that spontaneously resolved without pharmacological intervention. She had no previous psychiatric history. Following comprehensive investigations, other organic causes were ruled out, and Fahr's disease was diagnosed based on bilateral symmetrical brain calcifications seen on a head CT scan. Her treatment regimen encompassed antipsychotics and anticonvulsants. This case highlights the importance of considering Fahr's disease as a differential diagnosis in patients with new-onset neuropsychiatric symptoms. The case also explores the atypical early onset and intermittent nature of symptoms in the absence of a positive family history, highlighting the complexity of Fahr's disease. A multidisciplinary approach and regular follow-up are crucial for optimizing patient care and monitoring disease progression. Further research is needed to enhance our understanding of Fahr's disease and develop standardized treatment strategies for this rare condition.

EEG and acute confusional state at the emergency department.

OBJECTIVES: Acute confusional state (ACS) is a common cause of admission to the emergency department (ED). It can be related to numerous etiologies. Electroencephalography (EEG) can show specific abnormalities in cases of non-convulsive status epilepticus (NCSE), or metabolic or toxic encephalopathy. However, up to 80% of patients with a final diagnosis of NCSE have an ACS initially attributed to another cause. The exact place of EEG in the diagnostic work-up remains unclear. METHODS: Data of consecutive patients admitted to the ED for an ACS in a two-year period and who were referred for an EEG were collected. The initial working diagnosis was based on medical history, clinical, biological and imaging investigations allowing classification into four diagnostic categories. Comparison to the final diagnosis was performed after EEG recordings (and sometimes additional tests) were performed, which allowed the reclassification of some patients from one category to another. RESULTS: Seventy-five patients (mean age: 71.1 years) were included with the following suspected diagnoses: seizures for 8 (11%), encephalopathy for 14 (19%), other cause for 34 (45%) and unknown for 19 (25%). EEG was recorded after a mean of 1.5 days after symptom onset, and resulted in the reclassification of patients as follows: seizure for 15 (20%), encephalopathy for 15 (20%), other cause for 29 (39%) and unknown cause for 16 (21%). Moreover, ongoing epileptic activity (NCSE or seizure) and interictal epileptiform activity were found in eight (11%) patients initially diagnosed in another category. DISCUSSION: In our cohort, EEG was a key examination in the management strategy of ACS in 11% of patients admitted to the ED. It resulted in a diagnosis of epilepsy in these patients admitted with unusual confounding presentations.

A 52-Year-Old Woman with Weakness and Confusion.

A 52-Year-Old Woman with Weakness and ConfusionA 52-year-old woman presented for evaluation of abdominal pain, weakness, and confusion. How do you approach the evaluation, and what is the diagnosis?

Factors associated with disagreement between clinician app-based ultra-brief Confusion Assessment Method and reference standard delirium assessments.

BACKGROUND: Recently, the Ultra-Brief Confusion Assessment Method (UB-CAM), designed to help physicians and nurses to recognize delirium, showed high, but imperfect, accuracy compared with Research Reference Standard Delirium Assessments (RRSDAs). The aim of this study is to identify factors associated with disagreement between clinicians' app-based UB-CAM assessments and RRSDAs. METHODS: This is a secondary analysis of a prospective diagnostic test study. The study was conducted at two hospitals and included 527 inpatients (≥70 years old) and 289 clinicians (53 physicians, 236 nurses). Trained research associates performed RRSDAs and determined delirium presence using the CAM. Clinicians administered the UB-CAM using an iPad app. Disagreement factors considered were clinician, patient, and delirium characteristics. We report odds ratios and 95% confidence intervals. RESULTS: One thousand seven hundred and ninety-five clinician UB-CAM assessments paired with RRSDAs were administered. The prevalence of delirium was 17%. The rate of disagreement between clinician UB-CAM assessments and RRSDAs was 12%. Significant factors associated with disagreement between clinician UB-CAM assessments and RRSDAs (OR [95% CI]) included: presence of dementia (2.7 [1.8-4.1]), patient education high school or less (1.9 [1.3-2.9]), psychomotor retardation (2.5 [1.4-4.2]), and the presence of mild delirium or subsyndromal delirium (5.5 [3.5-8.7]). Significant risk factors for false negatives were patient age less than 80 (2.2 [1.1-4.3]) and mild delirium (3.5 [1.6-7.4]). Significant risk factors for false positives were presence of dementia (4.0 [2.3-7.0]), subsyndromal delirium (5.1 [2.9-9.1]), and patient education high school or less (2.0 [1.2-3.6]). Clinician characteristics were not significantly associated with disagreement. CONCLUSIONS: The strongest factors associated with disagreement between clinician UB-CAM screens and RRSDAs were the presence of dementia and subsyndromal delirium as risk factors for false positives, and mild delirium and younger age as a risk factor for false negatives. These disagreement factors contrast with previous studies of risk factors for incorrect clinician delirium screening, and better align screening results with patient outcomes.

The Confusion Assessment Method in action: Implementation of a protocol to increase delirium screening and diagnosis.

The use of the Confusion Assessment Method (CAM) for delirium assessment in real-life can be inconsistent. We examined the impact of a protocol on delirium screening and detection in hospitalized older adults using the CAM. We analyzed data from 32,338 admissions to a quaternary hospital between 2018 and 2022. We assessed the percentage of admissions screened for delirium, adherence to daily screening, positive screening, and overlap with ICD-10 coding. The percentage of admissions screened for delirium increased from 74% in 2018 to 98.7% in 2022. Adherence to daily screening was achieved in 24.5% of admissions, and the percentage of positive screenings fluctuated between 8.4% and 11.5%. Among the admissions with a delirium-related ICD-10 code, 32% had a positive screening, 62% were negative, and 6% remained unscreened. While implementing a protocol increased the proportion of admissions screened for delirium, adherence to daily screening and consistency of positive delirium screenings remain areas for improvement.

Motorist disorientation syndrome; clinical features and vestibular findings.

BACKGROUND: Motorist Disorientation Syndrome (MDS) is a term used to describe patients who primarily experience symptoms of dizziness/disorientation whilst in a motor car [21]. There is uncertainty about the relevance of vestibular dysfunction and whether this disorder could instead be a visually induced dizziness (VV) or part of a functional disorder similar to Persistent postural perceptual dizziness (PPPD). OBJECTIVE: We present the largest case-series to date of patients whose main complaint is of illusions of movement of self/vehicle when driving, characterising features of this group. METHODS: 18 subjects underwent detailed clinical assessment including validated questionnaires. A subset of patients underwent vestibular function testing. RESULTS: Mean onset age was 42 years, with no gender preponderance. Mean symptom duration was 6.39 years and was significantly longer in women. 50% reported moderate or severe handicap. Vestibular abnormalities were found in 60% of subjects tested. There was no significant difference in VSS total score between those with MDS and vestibular migraine (p = 0.154) with both having higher scores than healthy controls (p = 0.002, 0.000 respectively). CONCLUSIONS: MDS represents consistent symptoms, with high symptom burden, comparable to vestibular migraine. Vestibular deficits were not a consistent feature and similarities to VV and PPPD exist.

[Recognizing acute confusional syndrome and neurocognitive disorders in the emergency department]. / Reconnaître un syndrome confusionnel aigu et des troubles neurocognitifs aux urgences.

In the heart of the emergency room, when the nurse takes charge of the patient, he/she must be able to distinguish between an acute confusional syndrome and psychobehavioral symptoms related to neurocognitive disorders. Indeed, early identification of the confusional syndrome is essential to accelerate the implementation of non-drug measures by the nurse in order to reduce its duration and the induced complications.

Confusion assessment method accurately screens for hepatic encephalopathy and predicts short-term mortality in hospitalized patients with cirrhosis.

Hepatic encephalopathy (HE), a subtype of delirium, is common in cirrhosis and associated with poor outcomes. Yet, objective bedside screening tools for HE are lacking. We examined the relationship between an established screening tool for delirium, Confusion Assessment Method for the Intensive Care Unit (CAM-ICU) and short-term outcomes while comparing its performance with previously established measures of cognitive function such as West Haven criteria (WHC). Prospectively enrolled adults with cirrhosis who completed the CAM-ICU from 6/2014-6/2018 were followed for 90 days. Blinded provider-assigned West Haven Criteria (WHC) and other measures of cognitive function were collected. Logistic regression was used to test associations between CAM-ICU status and outcomes. Mortality prediction by CAM-ICU status was assessed using Area under the Receiver Operating Characteristics curves (AUROC). Of 469 participants, 11% were CAM-ICU( +), 55% were male and 94% were White. Most patients were Childs-Pugh class C (59%). CAM-ICU had excellent agreement with WHC (Kappa = 0.79). CAM-ICU( +) participants had similar demographic features to those CAM-ICU(-), but had higher MELD (25 vs. 19, p < 0.0001), were more often admitted to the ICU (28% vs. 7%, p < 0.0001), and were more likely to be admitted for HE and infection. CAM-ICU( +) participants had higher mortality (inpatient:37% vs. 3%, 30-day:51% vs. 11%, 90-day:63% vs. 23%, p < 0.001). CAM-ICU status predicted mortality with AUROC of 0.85, 0.82 and 0.77 for inpatient, 30-day and 90-day mortality, respectively. CAM-ICU easily screens for delirium/HE, has excellent agreement with WHC, and identifies a hospitalized cirrhosis cohort with high short-term mortality.

Pellagra as a differential diagnosis in the confused patient on the acute medical unit.

A man in his 80s was admitted via the acute medical take after presenting with increased confusion and features of alcohol withdrawal. He had a several-month history of a worsening pruritic rash surrounding his neck, arms and legs in addition to new, profuse diarrhoea. In view of the background of known chronic alcoholism and the coexisting symptoms of rash, confusion and diarrhoea, pellagra was diagnosed via a multidisciplinary approach. Oral nicotinamide supplementation was commenced and his symptoms responded rapidly. The bias and challenge of reaching a unified diagnosis in the context of a multisystem condition are exemplified in this case report.

Progressive MRI findings of West Nile virus encephalitis in a patient with diabetes mellitus.

A man in his 70s was admitted to our hospital with complaints of fatigue, loss of appetite and fever. His neurological examination was normal. He had a medical history of diabetes mellitus for 25 years. Urine analysis showed many leucocytes. Empirical antibiotic treatment was started for urinary system infection. Three days later, his mental status worsened with confusion and disorientation. MRI of the brain was normal. Two days later, the patient was intubated because of respiratory insufficiency. MRI showed restricted diffusion in bilateral thalamic nuclei. Encephalitis and ischaemia were considered in the differential diagnosis. Cerebrospinal fluid IgM antibody for West Nile virus was positive. Sixteen days later, cranial nerve reflexes were lost. MRI showed restricted diffusion and increased T2 signal intensity in the dorsal medulla and increased T2 signal intensity without diffusion restriction in bilateral substantia nigra and dentate nuclei. He died of cardiac arrest 40 days after hospitalisation.

Detection of unrecognized spatial disorientation: A theoretical perspective.

BACKGROUND: Spatial disorientation (SD) is a problem that pilots often encounter during a flight. One reason for this problem is that among the three types of SD, there is no validated method to detect the Type I (unrecognized) SD. OBJECTIVE: In this pursuit, initially we reviewed the problems and the evaluation methods of associated with SD. Subsequently, we discussed the advantages and disadvantages of the subjective questionnaire evaluation method and the behavior evaluation method. METHODS: On the basis of these analyses, we proposed a method to detect the unrecognized SD that improved the assessment of SD to a significant extent. We developed a new direction to study the unrecognized SD based on the subjective report and the center of pressure (CoP). RESULTS: The proposed evaluation method can assist the pilots to understand the feelings and physical changes, when exposed to unrecognized SD. CONCLUSION: We hope that this evaluation method can provide a strong support in developing a countermeasure against the unrecognized SD and fundamentally solve the severe flight accidents arising due to them.

Physiological Assessment of Delirium Severity: The Electroencephalographic Confusion Assessment Method Severity Score (E-CAM-S).

OBJECTIVES: Delirium is a common and frequently underdiagnosed complication in acutely hospitalized patients, and its severity is associated with worse clinical outcomes. We propose a physiologically based method to quantify delirium severity as a tool that can help close this diagnostic gap: the Electroencephalographic Confusion Assessment Method Severity Score (E-CAM-S). DESIGN: Retrospective cohort study. SETTING: Single-center tertiary academic medical center. PATIENTS: Three-hundred seventy-three adult patients undergoing electroencephalography to evaluate altered mental status between August 2015 and December 2019. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We developed the E-CAM-S based on a learning-to-rank machine learning model of forehead electroencephalography signals. Clinical delirium severity was assessed using the Confusion Assessment Method Severity (CAM-S). We compared associations of E-CAM-S and CAM-S with hospital length of stay and inhospital mortality. E-CAM-S correlated with clinical CAM-S (R = 0.67; p < 0.0001). For the overall cohort, E-CAM-S and CAM-S were similar in their strength of association with hospital length of stay (correlation = 0.31 vs 0.41, respectively; p = 0.082) and inhospital mortality (area under the curve = 0.77 vs 0.81; p = 0.310). Even when restricted to noncomatose patients, E-CAM-S remained statistically similar to CAM-S in its association with length of stay (correlation = 0.37 vs 0.42, respectively; p = 0.188) and inhospital mortality (area under the curve = 0.83 vs 0.74; p = 0.112). In addition to previously appreciated spectral features, the machine learning framework identified variability in multiple measures over time as important features in electroencephalography-based prediction of delirium severity. CONCLUSIONS: The E-CAM-S is an automated, physiologic measure of delirium severity that predicts clinical outcomes with a level of performance comparable to conventional interview-based clinical assessment.

A 66-Year-Old Man with Fever and Confusion.

A 66-Year-Old Man with Fever and ConfusionA 66-year-old man with Parkinson's disease and hypertension presented to the ED with fever and confusion after returning to the U.S. from Cambodia. He was febrile with diaphoresis and rigors. What was the diagnosis?

Evaluation of the 3-Minute Diagnostic Confusion Assessment Method for Identification of Postoperative Delirium in Older Patients.

Importance: Delirium is a common postoperative complication in older patients that often goes undetected and might lead to worse outcomes. The 3-Minute Diagnostic Confusion Assessment Method (3D-CAM) might be a practical tool for routine clinical diagnosis of delirium. Objective: To assess the 3D-CAM for detecting postoperative delirium compared with the long-form CAM used for research purposes. Design, Setting, and Participants: This cohort study of older patients enrolled in ongoing clinical trials between 2015 and 2018 was conducted at a single tertiary US hospital. Included participants were aged 60 years or older undergoing major elective surgical procedures that required at least a 2-day hospital stay. Data were analyzed between February and April 2019. Exposures: Surgical procedures of at least 2 hours in length requiring general anesthesia with planned extubation. Main Outcomes and Measures: Patients were concurrently assessed for delirium using the 3D-CAM assessment and the long-form CAM, scored based on a standardized cognitive assessment. Agreement between these 2 methods was tested using Cohen κ with repeated measures, a generalized linear mixed-effects model, and Bland-Altman analysis. Results: Sixteen raters conducted 471 concurrent CAM and 3D-CAM interviews including 299 patients (mean [SD] age, 69 [6.5] years), the majority of whom were men (152 [50.8%]), were White (263 [88.0%]), and had noncardiac operations (211 [70.6%]). Both instruments had good intraclass correlation (0.84 for the CAM and 0.98 for the 3D-CAM). Cohen κ demonstrated good overall agreement between the CAM and 3D-CAM (κ = 0.71; 95% CI, 0.58 to 0.83). According to the mixed-effects model, there was statistically significant disagreement between the 3D-CAM and CAM (estimated difference in fixed effect, -0.68; 95% CI, -1.32 to -0.05; P = .04). Bland-Altman analysis showed the probability of a delirium diagnosis with the 3D-CAM was more than twice the probability of a delirium diagnosis with the CAM (probability ratio, 2.78; 95% CI, 2.44 to 3.23). Conclusions and Relevance: The 3D-CAM instrument demonstrated agreement with the long-form CAM and might provide a pragmatic and sensitive clinical tool for detecting postoperative delirium, with the caveat that the 3D-CAM might overdiagnose delirium.

[What's all that confusion about ? Delirium in the elderly]. / C'est quoi ce délire ? - État confusionnel aigu chez la personne âgée.

Delirium is common in community-dwelling as well as in hospitalized older persons aged 75 years and older. Often underdiagnosed, delirium is associated with increased morbidity and mortality. Screening (with CAM and 3D-CAM) and identification of older people at increased risk for delirium is essential to enhance non-pharmacological preventative measures and monitor their evolution to allowing an early diagnosis. Screening instruments are currently available, such as the CAM and 3D-CAM. Pharmacological treatment is proposed only in situations where the patients endanger him-herself or other persons. In patients without previously known cognitive impairment, a cognitive assessment is mandated within the next 6 to 12 months period following the delirium episode.

L'état confusionnel aigu (ECA) est fréquent chez le patient âgé de plus de 75 ans, tant à domicile qu'en soins aigus. Souvent sous-diagnostiqué, il s'accompagne d'une morbi-mortalité accrue. L'identification des patients à risque d'ECA, tout comme un dépistage rapide avec des échelles validées (par exemple, Confusion Assessment Method (CAM) ou 3D-CAM), sont primordiaux afin de mettre en place une prévention active essentiellement non pharmacologique. Le traitement pharmacologique devrait être réservé aux situations dans lesquelles le patient se met en danger ou met en danger d'autres personnes. Un ECA n'est pas anodin et nécessite d'être signalé pour les prises en charge ultérieures. En l'absence de troubles neurocognitifs antérieurs connus, un bilan cognitif est recommandé dans les 6 à 12 mois suivant l'ECA.

[Confusional syndrome in the emergency room: from diagnosis to management]. / Le syndrome confusionnel, une urgence diagnostique et thérapeutique.

Delirium is an emergency and can have serious consequences. On the arrival at the emergency room of an elderly person, it should be systematically checked for confusional syndrome. If it is confirmed, a systematic and rapid etiological assessment carried out in the emergency room allows the identification of predisposing and precipitating factors. Therapeutic management is urgent, and includes treatment of the causes in the first instance.

Evolving symptom networks in relation to HIV-positive duration among people living with HIV: A network analysis.

OBJECTIVES: To explore and visualize the relationships among multiple symptoms in people living with HIV (PLWH) and compare centrality indices and the density of symptom networks among groups of individuals with different HIV-positive durations. METHODS: We conducted a secondary analysis of data from the HIV-related Symptoms Monitoring Survey conducted in China. Networks were constructed among 27 symptoms. Centrality properties, including strength and closeness, and network density were used to describe relationships among symptoms in 5 different HIV-positive duration groups. RESULTS: The findings showed that PLWH with longer HIV-positive durations did not have more severe symptoms; instead, their symptom networks were denser than those of their newly HIV-diagnosed counterparts (F = 27.073, P < 0.001). Fatigue was the most severe and central symptom in PLWH with an HIV-positive duration <10 years (rS = 7.79-10.09, rB = 18-44, rC = 0.01). Confusion was the most central symptom across the 3 centrality indices (rS = 11.81, rB = 14.00, rC = 0.02) in PLWH who had HIV-positive durations >10 years. CONCLUSION: This study demonstrates a need to include an assessment of PLWH symptom networks as an essential component of HIV care. We recommended evaluating cognitive function and cognitive training as essential components of HIV care for long-term survivors, even in younger populations (aged ≤50 years).