A neural circuit for spatial orientation derived from brain lesions.

There is disagreement regarding the major components of the brain network supporting spatial cognition. To address this issue, we applied a lesion mapping approach to the clinical phenomenon of topographical disorientation. Topographical disorientation is the inability to maintain accurate knowledge about the physical environment and use it for navigation. A review of published topographical disorientation cases identified 65 different lesion sites. Our lesion mapping analysis yielded a topographical disorientation brain map encompassing the classic regions of the navigation network: medial parietal, medial temporal, and temporo-parietal cortices. We also identified a ventromedial region of the prefrontal cortex, which has been absent from prior descriptions of this network. Moreover, we revealed that the regions mapped are correlated with the Default Mode Network sub-network C. Taken together, this study provides causal evidence for the distribution of the spatial cognitive system, demarking the major components and identifying novel regions.

Functional brain networks in Developmental Topographical Disorientation.

Despite a decade-long study on Developmental Topographical Disorientation, the underlying mechanism behind this neurological condition remains unknown. This lifelong selective inability in orientation, which causes these individuals to get lost even in familiar surroundings, is present in the absence of any other neurological disorder or acquired brain damage. Herein, we report an analysis of the functional brain network of individuals with Developmental Topographical Disorientation ($n = 19$) compared against that of healthy controls ($n = 21$), all of whom underwent resting-state functional magnetic resonance imaging, to identify if and how their underlying functional brain network is altered. While the established resting-state networks (RSNs) are confirmed in both groups, there is, on average, a greater connectivity and connectivity strength, in addition to increased global and local efficiency in the overall functional network of the Developmental Topographical Disorientation group. In particular, there is an enhanced connectivity between some RSNs facilitated through indirect functional paths. We identify a handful of nodes that encode part of these differences. Overall, our findings provide strong evidence that the brain networks of individuals suffering from Developmental Topographical Disorientation are modified by compensatory mechanisms, which might open the door for new diagnostic tools.

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.

Acute confusional state in HaNDL syndrome.

OBJECTIVE: To assess epidemiological, clinical and neuroimaging features of acute confusional state in the Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) syndrome. BACKGROUND: HaNDL is an increasingly recognized syndrome in which migraine-like headache episodes accompanied by hemiparaesthesia and/or hemiparesis and/or dysphasia are associated to CSF lymphocytic pleocytosis. The International Classification of Headache Disorders (ICHD-3) includes HaNDL syndrome in group 7 "headache attributed to non-vascular intracranial disorder" code 7.3.5, and lists the HaNDL-associated signs/symptoms that may be found less frequently. Confusional state is not mentioned in the 7.3.5-ICHD-3 "notes" or "comments" section as part of the HaNDL neurological spectrum. Moreover, the acute confusional state pathogenesis in HaNDL syndrome remains still uncertain and debated. METHODS: Here, we report a 32-year-old male who complained episodes of migraine-like headache and left hemiparaesthesia complicated by confusional state which led to discovering CSF lymphocytosis. Since other workup to determine the cause of his symptoms was otherwise negative, he was diagnosed as having HaNDL syndrome. We also ascertained and reviewed all available reports of HaNDL to assess the significance of confusional state in this syndrome. RESULTS: The search yielded 159 HaNDL cases among single reports and small/large series. Out of 159 patients who fulfilled the inclusion criteria for HaNDL according to the current ICHD at the time of diagnosis, 41 (25.7%) presented with acute confusional state. Among 41 HaNDL patients with confusional state, 16 (66.6%) out of 24 who underwent spinal tap had increased opening pressure. CONCLUSION: We propose that a mention of acute confusional state may be included in the "comments" section of "7.3.5-syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL)," when ICHD-3 diagnostic criteria will be updated. Moreover, we speculate that intracranial hypertension may play a role in the pathogenesis of the acute confusional state associated to HaNDL syndrome. Larger case series are needed to evaluate this hypothesis.

Non-convulsive status epilepticus possibly induced by a rapid correction of severe hyperkalemia: a case report and literature review.

BACKGROUND: Patients with chronic kidney disease frequently develop neurological complications including confusion and altered consciousness. Non-convulsive status epilepticus, which is characterized by a change in behavior and/or mental process accompanied by epileptiform discharges on electroencephalogram in the absence of convulsive seizures, is one of the overlooked causes of altered consciousness. The incidence and precise pathophysiological mechanism of non-convulsive status epilepticus in patients with kidney disease, and especially in patients with electrolyte disturbances, remains unknown. We recently treated an older patient with chronic kidney disease and severe hyperkalemia in whom non-convulsive status epilepticus developed following a correction of severe hyperkalemia. CASE PRESENTATION: An 82-year-old male was admitted to our hospital at midnight because of weakness of all four limbs (Day 1). He underwent urgent hemodialysis for severe hyperkalemia (9.84 mEq/L) and his serum potassium concentration decreased to 4.97 mEq/L. He regained full consciousness and his limb weakness improved on the morning of Day 2, but he became confused in the evening. Electroencephalogram revealed repeated low-voltage ictal discharges in the right occipital region and a diagnosis of non-convulsive status epilepticus was made. Following medication with fosphenytoin and phenytoin, the patient became fully alert and orientated on Day 8. CONCLUSION: We speculate that a rapid correction of hyperkalemia was the possible cause of non-convulsive status epilepticus development. To our knowledge, this is the first report of non-convulsive status epilepticus from a potassium abnormality. We described a case of this condition in detail and summarized 78 previous case reports of non-convulsive status epilepticus with kidney disease or electrolyte disturbances.

[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.

Hyperacute hyponatremia mimicking acute ischemic stroke.

We present a case of hyperacute hyponatremia with stroke like symptoms on presentation. Symptoms included confusion, left-sided facial droop, right-sided hemiparesis, dysarthria and aphasia, with an NIH stroke score of 5. Sodium level at the time of presentation was 119 mmol/L which dropped acutely from 138 mmol/L seven hours prior. Symptoms improved after treatment with 3% saline and no evidence of stroke, intracranial hemorrhage or space-occupying lesion was seen on imaging. The most likely cause of the hyponatremia was increased free water consumption and ADH surge. The patient remained symptom free after discharge with resolution of hyponatremia. Acute hyponatremia can cause focal neurological complaints and deficits, mimicking acute ischemic stroke. We advise clinicians to be aware of this entity when considering interventions for possible acute ischemic stroke and evaluating a patient with focal neurological deficits.

A man in his forties with acute confusion. / En mann i 40-årene med akutt forvirring.

Rapid and thorough assessment of acute neuropsychiatric symptoms is essential for effective treatment. Here we describe a patient with a distinctive but relatively rare disease.

Prospective Validation of the Preschool Confusion Assessment Method for the ICU to Screen for Delirium in Infants Less Than 6 Months Old.

OBJECTIVES: ICU delirium is a predictor of greater morbidity and higher mortality in the pediatric population. The diagnostic obstacles and validity of delirium monitoring among neonates and young infants have yet to be fully delineated. We sought to validate the Preschool Confusion Assessment Method for the ICU in neonates and young infants and determine delirium prevalence in this young population. DESIGN: Prospective cohort study to validate the Preschool Confusion Assessment Method for the ICU for the assessment of ICU delirium in neonates and young infants compared with the reference standard, Child and Adolescent Psychiatry. SETTING: Tertiary medical center PICU, including medical, surgical, and cardiac patients. PARTICIPANTS: Infants less than 6 months old admitted to the PICU regardless of admission diagnosis. MEASUREMENTS AND MAIN RESULTS: We enrolled 49 patients with a median age of 1.8 months (interquartile range, 0.7-4.1 mo), 82% requiring mechanical ventilation. Enrolled patients were assessed for delirium in blinded-fashion by the research team using the Preschool Confusion Assessment Method for the ICU and independently assessed by the psychiatry reference rater using Diagnostic and Statistical Manual of Mental Disorders-5 criteria. A total of 189 paired assessments were completed, and the Preschool Confusion Assessment Method for the ICU performed with a sensitivity of 95% (95% CI, 89-100%), specificity of 81% (68-90%), "negative and positive predictive values" of 97% (94-100%) and 69% (55-79%), respectively, compared with the reference rater. Delirium prevalence was 47%, with higher rates of 61% observed among neonates (< 1 mo old) and 39% among infants 1-6 months old. CONCLUSIONS: The Preschool Confusion Assessment Method for the ICU is a valid screening tool for delirium monitoring in infants less than 6 months old. Delirium screening was feasible in this population despite evolving neurocognition and arousal architecture. ICU delirium was prevalent among infants. The consequence of acute brain dysfunction during crucial neurocognitive development remains unclear. Future studies are necessary to determine the long-term impact of ICU delirium and strategies to reduce associated harm in critically ill infants.

West Nile Virus Neuroinvasive Disease Accelerating Probable Dementia With Lewy Bodies.

We describe a case of dementia with Lewy bodies immediately following encephalitis due to West Nile virus (WNV). The patient had rapid eye movement-sleep behavior disorder and constipation before the onset of encephalitis, which suggests that he would have ultimately developed dementia with Lewy bodies even without WNV infection. Our case illustrates the interactions between α-synuclein and WNV, as observed in mouse models, wherein synuclein expression augments after WNV infection and protects neurons against the virus.

Neurological Manifestations in COVID-19 Infection: A Systematic Review and Meta-Analysis.

BACKGROUND: Growing evidence showed that coronavirus disease 2019 (COVID-19) infection may present with neurological manifestations. This review aimed to determine the neurological manifestations and complications in COVID-19. METHODS: We conducted a systematic review and meta-analysis that included cohort and case series/reports involving a population of patients confirmed with COVID-19 infection and their neurologic manifestations. We searched the following electronic databases until April 18, 2020: PubMed, Embase, Scopus, and World Health Organization database (PROSPERO registration number: CRD42020180658). RESULTS: From 403 articles identified, 49 studies involving a total of 6,335 confirmed COVID-19 cases were included. The random-effects modeling analysis for each neurological symptom showed the following proportional point estimates with 95% confidence intervals: "headache" (0.12; 0.10-0.14; I2 = 77%), "dizziness" (0.08; 0.05-0.12; I2 = 82%), "headache and dizziness" (0.09; 0.06-0.13; I2 = 0%), "nausea" (0.07; 0.04-0.11; I2 = 79%), "vomiting" (0.05; 0.03-0.08; I2 = 74%), "nausea and vomiting" (0.06; 0.03-0.11; I2 = 83%), "confusion" (0.05; 0.02-0.14; I2 = 86%), and "myalgia" (0.21; 0.18-0.25; I2 = 85%). The most common neurological complication associated with COVID-19 infection was vascular disorders (n = 23); other associated conditions were encephalopathy (n = 3), encephalitis (n = 1), oculomotor nerve palsy (n = 1), isolated sudden-onset anosmia (n = 1), Guillain-Barré syndrome (n = 1), and Miller-Fisher syndrome (n = 2). Most patients with neurological complications survived (n = 14); a considerable number of patients died (n = 7); and the rest had unclear outcomes (n = 12). CONCLUSION: This review revealed that neurologic involvement may manifest in COVID-19 infection. What has initially been thought of as a primarily respiratory illness has evolved into a wide-ranging multi-organ disease.

Need for Standardization of Measurement of Time to Reorientation as a Predictor of Cognitive Adverse Effects of Electroconvulsive Therapy.

ABSTRACT: Testing for time to reorientation (TRO) after electroconvulsive therapy (ECT) has been one of the most predictive measures for cognitive adverse effects of ECT. However, measuring TRO varies between clinical trials, and there is no standardized approach on its measurement between studies. The objective of this report is to systematically review the literature on the different ways of measuring TRO. This would be a first step to establish a consensus on a standard method for TRO. We performed a systematic literature review from 1968 to October 2019 for clinical trials with a sample size of more than 50 and published in English that describe measures of TRO. We searched both PubMed and Web of Science databases. The literature search generated 12 clinical trials, which met the inclusion criteria. Studies measured TRO using 3-, 5-, or 10-item questionnaires. Most studies measured full orientation as answering 4/5 questions. Other studies smaller than 50 required 14/14 items to be answered correctly. There was wide variation between studies on how often and when orientation questions were initiated. These factors have obvious implications to the results and interpretation of the ECT literature.There was no clear consensus on measuring TRO in a uniform fashion. Research and clinical care would benefit from a standardized approach to measuring TRO. Measuring TRO using a 4/5 method seems the most common and quickest way, whereas using a 14/14 method seems to be the most rigorous and sensitive to change in trials.

The postictal state - What do we know?

This narrative review provides a broad and comprehensive overview of the most important discoveries on the postictal state over the past decades as well as recent developments. After a description and definition of the postictal state, we discuss postictal sypmtoms, their clinical manifestations, and related findings. Moreover, pathophysiological advances are reviewed, followed by current treatment options.

Association between Use of Angiotensin-converting Enzyme Inhibitors or Angiotensin Receptor Blockers and Postoperative Delirium.

BACKGROUND: Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers improve cognitive function. The authors therefore tested the primary hypothesis that preoperative use of angiotensin inhibitors is associated with less delirium in critical care patients. Post hoc, the association between postoperative use of angiotensin system inhibitors and delirium was assessed. METHODS: The authors conducted a single-site cohort study of adults admitted to Cleveland Clinic critical care units after noncardiac procedures between 2013 and 2018 who had at least one Confusion Assessment Method delirium assessment. Patients with preexisting dementia, Alzheimer's disease or other cognitive decline, and patients who had neurosurgical procedures were excluded. For the primary analysis, the confounder-adjusted association between preoperative angiotensin inhibitor use and the incidence of postoperative delirium was assessed. Post hoc, the confounder-adjusted association between postoperative angiotensin system inhibitor use and the incidence of delirium was assessed. RESULTS: The incidence of delirium was 39% (551 of 1,396) among patients who were treated preoperatively with angiotensin system inhibitors and 39% (1,344 of 3,468) in patients who were not. The adjusted odds ratio of experiencing delirium during critical care was 0.98 (95% CI, 0.86 to 1.10; P = 0.700) for preoperative use of angiotensin system inhibitors versus control. Delirium was observed in 23% (100 of 440) of patients who used angiotensin system inhibitors postoperatively before intensive care discharge, and in 41% (1,795 of 4,424) of patients who did not (unadjusted P < 0.001). The confounder-adjusted odds ratio for experiencing delirium in patients who used angiotensin system inhibitors postoperatively was 0.55 (95% CI, 0.43 to 0.72; P < 0.001). CONCLUSIONS: Preoperative use of angiotensin system inhibitors is not associated with reduced postoperative delirium. In contrast, treatment during intensive care was associated with lower odds of delirium. Randomized trials of postoperative angiotensin-converting enzymes inhibitors and angiotensin receptor blockers seem justified.

Visual Vertigo, Motion Sickness, and Disorientation in Vehicles.

Environmental circumstances that result in ambiguity or conflict with the patterns of sensory stimulation may adversely affect the vestibular system. The effect of this conflict in sensory information may be dizziness, a sense of imbalance, nausea, and motion sickness sometimes even to seemingly minor daily head movement activities. In some, it is not only exposure to motion but also the observation of objects in motion around them such as in supermarket aisles or other places with visual commotion; this can lead to dizziness, nausea, or a feeling of motion sickness that is referred to as visual vertigo. All people with normal vestibular function can be made to experience motion sickness, although individual susceptibility varies widely and is at least partially heritable. Motorists learn to interpret sensory stimuli in the context of the car stabilized by its suspension and guided by steering. A type of motorist's disorientation occurs in some individuals who develop a heightened awareness of perceptions of motion in the automobile that makes them feel as though they may be rolling over on corners and as though they are veering on open highways or in streaming traffic. This article discusses the putative mechanisms, consequences and approach to managing patients with visual vertigo, motion sickness, and motorist's disorientation syndrome in the context of chronic dizziness and motion sensitivity.