Acute necrotizing encephalopathy.

Influenza virus infection is a rare cause of neurological complications, with acute necrotizing encephalopathy (ANE) being among the deadliest. Due to the low incidence of ANE, literature about its association with influenza B infection is limited. We present the case of a 29-year-old previously healthy man with an imaging and clinical diagnosis of influenza B virus infection and sudden decline in mental status. Magnetic resonance imaging showed multifocal areas of abnormal T2 FLAIR signal and restricted diffusion without significant enhancement, with negative mircobiological studies of cerebrospinal fluid. The patient died despite multiple treatments including an antiviral, steroids, and intravenous immunoglobulin. Due to ANE's more common presentation during childhood, this case report represents one of the few available publications in the adult population.

Increased creativity associated with dopamine agonist therapy: A case report and short review of the literature.

Impulse control disorder (ICD) has been linked to dopamine agonist use in patients with Parkinson's disease. Increased creativity is another cognitive side effect of dopaminergic therapy. While ICD is well recognized in the literature, enhanced creativity as a positive phenomenon is underreported because it does not negatively affect the patients' quality of life. Herein, we report a case of a 49-year-old man with Parkinson's disease who developed enhanced creativity expressed by the acquisition of multiple, new artistic skills with ropinirole treatment. He spent a significant amount of time on painting, carving and axe restoration, selling these artistic products became a source of income. He also reports that these hobbies help him cope with physical limitations caused by Parkinson's disease.

Validity of the Clinical Dementia Rating Scale Sum of Boxes in Staging and Detection of Cognitive Impairment in Mexican Americans.

OBJECTIVE: We evaluated the utility of the Clinical Dementia Rating Sum of Boxes score (CDR-SB) in staging and detecting amnestic-mild cognitive impairment (a-MCI) and Alzheimer's disease (AD) among Mexican Americans. METHODS: Receiver operator curves were generated to evaluate the validity of the CDR-SB in staging and detecting a-MCI and AD in 1,073 Mexican Americans (758 controls, 163 a-MCI, and 152 AD). RESULTS: Optimal ranges of the CDR-SB were 0, 0.5-4, 4.5-8.0, 8.5-13 and 13.5-18 for staging the global CDR score of 0, 0.5, 1, 2, and 3, respectively. The CDR-SB ≥ 0.5 differentiated the a-MCI patients from the controls (sensitivity 100% and specificity 99.5%) and ≥ 2.0 distinguished the AD from a-MCI patients (sensitivity 83.6% and specificity 87.1%). These cutoffs were also appropriate for patients with ≤6 years of education. CONCLUSION: The CDR-SB is useful to detect and stage a-MCI and AD in Mexican Americans with diverse education levels.

Psychosis.

PURPOSE OF REVIEW: Psychosis can manifest in primary psychotic disorders, neurologic diseases, and medical conditions. This article reviews the definition of psychotic symptoms and the evaluation and management of psychosis in primary psychiatric and neurologic disorders frequently seen in neurologic practice. RECENT FINDINGS: Emerging evidence supports significant connections between psychosis and structural and functional brain changes in both primary psychotic and neurologic disorders. In addition to antidopaminergic activity, the mechanism of new-generation antipsychotics shifts to act on serotonin receptors, which potentially contributes to their benefits in the treatment of negative symptoms of psychosis and a lesser frequency of extrapyramidal side effects compared with typical antipsychotics. This is also helpful in the treatment of psychosis in patients who have neurodegenerative diseases and are vulnerable to developing extrapyramidal side effects from typical antipsychotics. SUMMARY: Even with significant overlap, management of psychosis in primary psychotic disorders differs from the approach of psychosis in neurologic diseases. This article helps clinicians learn how to practically evaluate psychosis from both psychiatric and neurologic perspectives.

Foix-Chavany-Marie syndrome due to unilateral anterior opercular infarction with leukoaraiosis.

Foix-Chavany-Marie syndrome (FCMS) is a cortical-subcortical pseudobulbar palsy characterized by automatic voluntary dissociation of facio-masticatory-pharyngo-glosso-laryngeal movements. FCMS is typically caused by vascular insults on the bilateral anterior opercular or adjacent subcortical areas. Acute onset of FCMS secondary to a unilateral lesion is extremely rare. Herein we present a case of FCMS caused by acute unilateral anterior opercular infarction with preexisting bilateral leukoaraiosis. Our case shows that an acute unilateral anterior opercular lesion can decompensate preexisting corticobulbar-subcortical lesions and cause the typical features of FCMS.

Degenerative dementia with proximal radial and lower vertical spatial neglect.

A 75 year-old man had a two-year history of progressive memory loss, trouble with finances and getting lost. On examination, he scored 16/30 in MoCA test, noticeably impaired on the attentional tasks. His screening bloodtests werenormal. Brain imaging revealed hippocampal atrophy and bilaterallarge areas of leukoaraiosis below posterior parietal lobes. On vertical line bisection he revealed a large upward bias and on radial bisection, a distal bias. Degeneration of his posterior parietal cortex may have caused both the leukoaraiosis and vertical-radial neglect. Unawareness of portions of space can be a source of disability and cause injury. Therefore, patients with degenerative dementia, especially those with similar patterns of leukoaraiosis or parietal degeneration should be tested for vertical and radial forms of spatial neglect.

Rapidly progressive dementia-associated N-type voltage-gated calcium channel antibody encephalopathy.

Autoimmune encephalopathy is one of the treatable causes of rapidly progressive dementia; however, it is often underdiagnosed. Autoantibodies against voltage-gated calcium channel (VGCC) have been linked to several neurological disorders, including Lambert-Eaton syndrome, but VGCC antibody-associated encephalopathy is uncommon. Herein, we present a case of a 74-year-old woman with prominent neuropsychiatric symptoms followed by rapid cognitive decline. Extensive initial studies were nondiagnostic. Subsequently, serum N-type VGCC antibody was positive. After treatment with intravenous immunoglobulin, the patient's cognition and neuropsychiatric symptoms significantly improved.

Proactive interference apraxic agraphia: a writing and drawing disorder associated with corticobasal syndrome.

Proactive interference is when a previously performed task impairs performance on a current task. It is often associated with memory tasks and has not been reported to interfere with writing or drawing. We evaluated a left-handed man diagnosed with corticobasal syndrome who had a two-year history of progressive agraphia. On the sentence writing and clock drawing tasks, he initially wrote letters and numbers correctly but revealed an increase of movement errors as the tasks progressed. We propose the term "proactive interference apraxic agraphia" for this novel disorder. Prefrontal dysfunction may cause an impairment in disengaging from previously activated motor programs.

Early-Motor Phenotype Relates to Neuropsychiatric and Cognitive Disorders in Huntington's Disease.

OBJECTIVE: To determine the relationships between the motor phenotype and the presence of specific neuropsychiatric and neuropsychological disorders in patients with early motor-manifest Huntington's disease (HD). METHODS: From the Enroll-HD study, 3,505 individuals with HD who had ≤5 years of motor symptoms were classified based on the predefined parkinsonism/chorea index into chorea-dominant (n = 1125), parkinsonism-dominant (n = 867), and mixed-motor phenotype (n = 1513) groups. An analysis was performed on the results of the short Problem Behaviors Assessment. This test assesses patients for neuropsychiatric disorders such as depression, irritability/aggression, apathy, obsessive-compulsive behaviors, and psychosis as well as cognitive disorders by using neuropsychological tests such as the Stroop Interference test, Trail Making Test Part A and B, letter fluency, Symbol Digit Modality test, and animal fluency test. RESULTS: In these early motor-manifest HD individuals, motor phenotype was associated with neuropsychiatric and cognitive changes. Independent of the age of motor onset, motor duration and severity, CAG repeat expansion, and medication use, the parkinsonism-dominant group had more severe neuropsychiatric disorders (depression, apathy, obsessive-compulsive behaviors, and psychosis) and poorer performance on all cognitive tests than those patients in the mixed-motor phenotype and chorea-dominant groups. The patients in the chorea-dominant group had less apathy and depression than those in the mixed-motor phenotype group. CONCLUSIONS: In the early stage of motor-manifest HD, parkinsonism-dominant patients appear to have more severe neuropsychiatric disturbances and more cognitive impairments than those HD patients with chorea-dominant and mixed-motor phenotypes. Future studies should explore the brain mechanisms of these disorders and the possible treatments. © 2020 International Parkinson and Movement Disorder Society.

Mild cognitive impairment and dementia in motor manifest Huntington's disease: Classification and prevalence.

OBJECTIVES: To identify the characteristics and prevalence of mild cognitive impairment in patients with motor-manifest Huntington's disease (HD) and to propose a new mild cognitive impairment (HD-MCI) classification for HD. METHODS: We included 307 motor-manifest HD participants from the ENROLL-HD study who completed the evaluation in four neurocognitive domains including executive functions, processing speed, language, and memory. Cognitive impairment in each domain was determined by age- and education-adjusted cutoffs (> 1.5 standard deviations below the mean). HD-MCI was defined as an impairment in at least one cognitive domain without a loss of functional independence (Function Independence Scale, FIS ≥85). Dementia (HD-Dem) was defined as at least two domains of cognitive impairment with functional impairment (FIS ≤80). RESULTS: At the onset of motor symptoms, MCI was present in 84% and dementia in 5% of patients. After 5 years of motor symptoms, 24% of participants met the criteria for MCI and 69% for dementia. Executive dysfunction was the most common impairment, being present in 70% of participants, followed by slowed processing speed in 67%. Language impairment was reported in 55% and memory deficits in 53%. MCI subtypes were classified as "Executive-predominant" (executive impairment and slowed processing speed), "Representational-predominant" (impaired language and memory) and "Mixed Executive-Representational". Executive-predominant MCI comprised 30%, Representational-predominant 15% and Mixed 55% of this cohort. CONCLUSION: MCI is highly prevalent in the early stage of motor-manifest HD. Three MCI subgroups are defined suggesting at the earlier stage of this disease the frontal-striatal-executive and/or the temporoparietal-representational functional network can be impaired.

Vertical and Radial Attentional Neglect in Corticobasal Syndrome.

Corticobasal degeneration (CBD), a tau-related neurodegenerative disease, is characterized by limb rigidity, dystonia, myoclonus, apraxia, and cognitive deficits. We report a patient with probable corticobasal syndrome, a major phenotype of CBD, who revealed both lower vertical and proximal radial attentional neglect on line bisection tests. Brain imaging revealed bilateral parietal atrophy with hypometabolism in the bilateral parietal, dorsolateral prefrontal, and premotor cortices. Bilateral impairment in the dorsal attentional network reduces the allocation of spatial attention to lower and proximal space, causing lower vertical and proximal radial attentional neglect. Screening for various types of spatial neglect may be important in tailoring management and rehabilitation strategies for patients with CBD.

Sudden unexpected death in epilepsy: ongoing challenges in finding mechanisms and prevention.

Purpose/aim of the study: To summarize recent studies on the pathophysiology and preventive strategies for SUDEP. Materials and methods: Databases and literature review. Results: Patients with epilepsy have a significantly higher risk of death than the general population. Sudden unexpected death in epilepsy (SUDEP) is the leading cause of sudden death among patients with epilepsy. Despite on-going research, there are still deficits in our knowledge about the mechanisms, genetic factors, and prevention of SUDEP. Current evidence suggests that cardiac arrhythmias, respiratory dysfunction, and brainstem arousal system dysfunction are the major mechanisms of SUDEP, and animal models support the role of neurotransmitters, especially serotonin and adenosine, in pathophysiology of SUDEP. Several mutations in the neurocardiogenic channelopathy genes have been identified as a possible cause of epilepsy and increased SUDEP risk. The lack of awareness that SUDEP can be a potential cause of premature death has been found in several surveys. In addition, medical legal cases demonstrate the need for more education about this condition. Several preventive strategies to reduce SUDEP have been proposed, including effective seizure control, nocturnal supervision, seizure monitoring, devices to protect the airway, and selective serotonin reuptake inhibitors. Further research is needed to determine the efficacy of these interventions. Conclusions: The major mechanisms of SUDEP include cardiac arrhythmias, respiratory dysfunction, and brainstem arousal system dysfunction. Effective control of seizures is the only effective strategy to prevent SUDEP. Other preventive interventions require more research.

The illiterate brain and the neuropsychological assessment: From the past knowledge to the future new instruments.

The lifetime learning of illiterate and low-educated individuals shapes their cognitive skills, which are challenging to grade by the current available neuropsychological tools. Eight hundred million of the global population are illiterate. It is very challenging to interpret the cognitive performance of this population by the available formal neuropsychological tests, mainly developed for higher educated people. From extensive literature investigation, we reviewed the cognitive process and performance of illiterate and low-educated population on various cognitive domains including language, executive function, memory, visual-related function, and motor skills. We also suggested the concept in the development of the appropriate tools for the cognitive assessment among this population. Finally, we provide the available cognitive screening tools validated in the illiterate and low-educated subjects within the last three decades.

Lower Activation in Frontal Cortex and Posterior Cingulate Cortex Observed during Sex Determination Test in Early-Stage Dementia of the Alzheimer Type.

Face-labeling refers to the ability to classify faces into social categories. This plays a critical role in human interaction as it serves to define concepts of socially acceptable interpersonal behavior. The purpose of the current study was to characterize, what, if any, impairments in face-labeling are detectable in participants with early-stage clinically diagnosed dementia of the Alzheimer type (CDDAT) through the use of the sex determination test (SDT). In the current study, four (1 female, 3 males) CDDAT and nine (4 females, 5 males) age-matched neurotypicals (NT) completed the SDT using chimeric faces while undergoing BOLD fMRI. It was expected that CDDAT participants would have poor verbal fluency, which would correspond to poor performance on the SDT. This could be explained by decreased activation and connectivity patterns within the fusiform face area (FFA) and anterior cingulate cortex (ACC). DTI was also performed to test the association of pathological deterioration of connectivity in the uncinate fasciculus (UF) and verbally-mediated performance. CDDAT showed lower verbal fluency test (VFT) performance, but VFT was not significantly correlated to SDT and no significant difference was seen between CDDAT and NT for SDT performance as half of the CDDAT performed substantially worse than NT while the other half performed similarly. BOLD fMRI of SDT displayed differences in the left superior frontal gyrus and posterior cingulate cortex (PCC), but not the FFA or ACC. Furthermore, although DTI showed deterioration of the right inferior and superior longitudinal fasciculi, as well as the PCC, it did not demonstrate significant deterioration of UF tracts. Taken together, early-stage CDDAT may represent a common emerging point for the loss of face labeling ability.

White Matter Deterioration May Foreshadow Impairment of Emotional Valence Determination in Early-Stage Dementia of the Alzheimer Type.

In Alzheimer Disease (AD), non-verbal skills often remain intact for far longer than verbally mediated processes. Four (1 female, 3 males) participants with early-stage Clinically Diagnosed Dementia of the Alzheimer Type (CDDAT) and eight neurotypicals (NTs; 4 females, 4 males) completed the emotional valence determination test (EVDT) while undergoing BOLD functional magnetic resonance imaging (fMRI). We expected CDDAT participants to perform just as well as NTs on the EVDT, and to display increased activity within the bilateral amygdala and right anterior cingulate cortex (r-ACC). We hypothesized that such activity would reflect an increased reliance on these structures to compensate for on-going neuronal loss in frontoparietal regions due to the disease. We used diffusion tensor imaging (DTI) to determine if white matter (WM) damage had occurred in frontoparietal regions as well. CDDAT participants had similar behavioral performance and no differences were observed in brain activity or connectivity patterns within the amygdalae or r-ACC. Decreased fractional anisotropy (FA) values were noted, however, for the bilateral superior longitudinal fasciculi and posterior cingulate cortex (PCC). We interpret these findings to suggest that emotional valence determination and non-verbal skill sets are largely intact at this stage of the disease, but signs foreshadowing future decline were revealed by possible WM deterioration. Understanding how non-verbal skill sets are altered, while remaining largely intact, offers new insights into how non-verbal communication may be more successfully implemented in the care of AD patients and highlights the potential role of DTI as a presymptomatic biomarker.

Isolated left posterior insular infarction and convergent roles in verbal fluency, language, memory, and executive function.

The posterior insular cortex-a complex structure interconnecting various brain regions for different functions-is a rare location for ischemic stroke. We report a patient with isolated left posterior insular infarction who presented with multiple cognitive impairment, including impairment in semantic and phonemic verbal fluency.

Idiopathic intracranial hypertension: ongoing clinical challenges and future prospects.

Idiopathic intracranial hypertension (IIH) is an uncommon disorder characterized by increased intracranial pressure without radiological or laboratory evidence of intracranial pathology except empty sella turcica, optic nerve sheath with filled out cerebrospinal fluid spaces, and smooth-walled nonflow-related venous sinus stenosis or collapse. This condition typically affects obese women. The incidence of IIH is increasing with the rising prevalence of obesity. Persistent headache is the most common symptom. Visual impairment is a serious complication that may not be recognized by the patients. This paper reviews clinical manifestations, diagnostic challenges, and current treatments of IIH in adults. Various imaging modalities have been studied on their validity for detection of IIH and papilledema. This review also includes new studies on medical, surgical, and interventional management of this condition. Acetazolamide and topiramate are the only two medications that have been studied in randomized controlled trials about their efficacy in treatment of IIH. In patients who have severe visual impairment or progressive visual deterioration despite medical management, surgical or interventional treatment may be considered. The efficacy and complications of cerebrospinal fluid diversion, optic nerve sheath fenestration, and endovascular venous stenting reported in the last 3 decades have been summarized in this review. Finally, the prospective aspects of biomarkers and treatments are proposed for future research.