Cerebral ventricular empyema associated with severe adult pyogenic meningitis: computed tomography findings

Cerebral ventricular empyema (CVE), also known as pyocephalus, is a rare form of pyogenic ventriculitis. We present cranial computed tomography (CT) in an adult who developed a bilateral CVE associated with acute pyogenic meningitis. CT showed an obstructive ventriculomegaly and fluid–fluid levels layering in the lateral ventricles and the third ventricle. Frank neutrophilic pus was taken from the subarachnoid space. After antibiotic treatment, the pyocephalus resolved. CVE may be visualized on CT with pus layering in the ventricular CSF, creating a fluid level of intermediate hypodensity.     

A Preliminary Epidemiologic Study of Social (Pragmatic) Communication Disorder Relative to Autism Spectrum Disorder and Developmental Disability Without Social Communication Deficits

The goal of this preliminary investigation was to compare demographic and clinical characteristics in a sample of children with likely Social (Pragmatic) Communication Disorder (SCD) (N?=?117) to those in children with possible (N?=?118) and some (N?=?126) SCD traits, other developmental delay (DD) (N?=?91) and autism spectrum disorder (ASD) (N?=?642). We used data from the Study to Explore Early Development (SEED), a multi-site case–control study. Items reflecting SCD DSM-5 criteria were selected from an autism diagnostic measure, with SCD categories identified by creating quartiles. Our results suggest that SCD may fall along a continuum involving elevated deficits (in comparison to DD with no SCD) in social communication and restricted and repetitive behavior that do not reach the clinical threshold for ASD.

     

Neurobehavioral impact of sickle cell disease in early childhood.

The physical effects of sickle cell disease (SCD) begin in infancy or early childhood, yet most behavioral studies have focused on school-age children. We evaluated the impact of higher versus lower neurologic risk on language, motor abilities, executive functions, and temperament in toddlers and early preschoolers with SCD. Thirty-nine children with higher risk SCD were compared to 22 children with lower risk SCD. Language and motor abilities were lower in older compared with younger children but were unrelated to sickle cell subgroups. Executive functions, particularly working memory, were poorer in children with higher risk SCD regardless of age. Parent-reported activity level was also lower in children with higher risk. Specific behavioral influences of SCD are evident early in childhood and include working memory decrements. Executive function deficits in SCD can emerge early in life and may be an important context for other areas of cognitive and behavioral development.     

Olfactory processing in schizophrenia,non-ill first-degree family members,and young people at-risk for psychosis

Objectives. While deficits in odor identification and discrimination have been reported in schizophrenia, few studies have examined the relative specificity of these deficits in patients and at-risk youth. Method. Sniffin’ Sticks odor identification and discrimination were assessed in schizophrenia outpatients and non-ill first-degree relatives (Study One), as well as youth at clinical (CR) or genetic (GR) risk for schizophrenia (Study Two). Scores were z-transformed, using the performance of a demographically-matched adult or adolescent comparison group. Results. Patients and relatives were impaired on odor identification, but odor discrimination impairment was limited to the patient group. A similar pattern of impairment emerged in at-risk youth. GR youth were impaired on odor identification but not discrimination, while CR youth were impaired on both tasks. In patients, olfactory impairment was correlated with negative symptomatology. Conclusions. To our knowledge, this is the first study to show that CR youth are impaired on both olfactory tasks, as observed in adult schizophrenia patients. GR youth were impaired only on odor identification like their adult counterparts. These data suggest that odor identification impairment, in isolation, may represent a genetic marker of vulnerability for schizophrenia, while odor discrimination deficits may be a biomarker associated with the development of psychosis.     

Need for cognitive rehabilitation for children with sickle cell disease and strokes

Stroke is a significant cause of morbidity among children with sickle cell disease (SCD). Approximately 30% of children with sickle cell anemia will have either an overt stroke or silent infarct (an injury to the brain that does not have any focal neurological findings or a history of focal neurological deficits). Despite the strong association between stroke and cognitive deficits and poor educational attainment, few interventions have been developed to address this vulnerable population of children. A cognitive rehabilitation program was piloted to improve the memory and educational achievement of children with sickle cell disease and stroke. While the intervention was feasible, additional research is needed to establish efficacy. Ongoing studies are designed to improve the cognitive impairment for children with SCD and stroke. The health and educational systems will need to work as partners to improve the cognitive and educational outcomes of these children after they suffer from stroke.     

Hyperintense signal on spinal cord diffusion-weighted imaging in a patient with subacute combined degeneration

Symmetrical hyperintense signals in lateral columns and posterior columns in T2-weighted images is a characteristic finding in subacute combined degeneration (SCD) secondary to vitamin B12 deficiency. Manifestation of SCD on diffusion-weighted imaging (DWI) has not been well characterised till date. We reported a case of SCD with spinal cord hyperintense signals in DWI sequence. We suggest that spinal cord DWI may be a useful magnetic resonance sequence for the diagnosis of SCD.     

Depression and sickle cell disease.

Depressive symptoms are very common in patients with a chronic medical illness such as sickle cell disease (SCD). Clinicians may fail to recognize depression in such patients when, as in SCD, the two conditions have many overlapping symptoms. Based on a Medline database search, we review papers addressing the relationship between depression and SCD. Data regarding this relationship are conflicting. We provide recommendations for improving the treatment of patients with SCD and depression.     

Platelet P-selectin and platelet mass, volume and component in sickle cell disease: relationship to genotype

BACKGROUND AND PURPOSE: Excess platelet activation (e.g. increased soluble P selectin [sPsel] and beta thromboglobulin [beta-TG]) is well established in sickle cell disease (SCD) and may contribute to the prothrombotic/hypercoagulable state and vascular occlusion characteristic of the disease. We hypothesised altered whole platelet P-selectin (pPsel), and morphological platelet indices mass, volume and component in SCD and two of its major genotypes. METHODS: We recruited 35 SCD patients [mean age 31 years, 54% men]. Of these, 16 had homozygous sickle cell (HbSS) disease and 19 had sickle-haemoglobin-C (HbSC) disease. Patients were compared with 29 subjects with normal haemoglobin (HbAA) matched for age and ethnicity. Platelet mass, volume and component were measured by flow cytometry, pPsel in platelet lysate, sP-sel and beta-TG by ELISA. RESULTS: SCD patients had lower pP-sel and mean platelet volume (MPV) but elevated platelet component (MPC), and, as expected, elevated platelet count, and sP-sel (all p<0.05) compared to HbAA subjects. In both groups, pPsel correlated with MPV, and MPV correlated positively with mean platelet mass (MPM) and negatively with MPC. sPsel correlated with platelet count only in SCD, not in the controls. Platelet count alone was different (higher) in HbSS compared to HbSC, and sPsel correlated with platelet count only in HbSC disease, not in HbSS disease. CONCLUSION: Patients with SCD have various abnormalities in their platelets regardless of genotype: there are more numerous platelets, which are smaller, contain less P selectin per cell, but have a higher concentration of granules than those of HbAA subjects. These differences may mark and/or promote the prothrombotic state in SCD.     

Cognitive flexibility and its electrophysiological correlates in Gilles de la Tourette syndrome

Motor symptoms in Gilles de la Tourette syndrome (GTS) have been related to changes in frontostriatal brain networks. These changes may also give rise to alterations in cognitive flexibility. However, conclusive evidence for altered cognitive flexibility in patients with GTS is still lacking. Here, we meta-analyzed data from 20 neuropsychological studies that investigated cognitive flexibility in GTS using the Wisconsin Card Sorting Test (WCST). Results revealed medium-sized GTS-related performance deficits, which were significantly modulated by age: Whilst being substantial in children and adolescents with GTS, WCST deficits seem to dissolve in adult patients with GTS. This age-related normalization of WCST performance might result from the compensatory recruitment of cognitive control in adult patients with GTS. We addressed this possibility by examining neural correlates of proactive and reactive cognitive control in an event-related potential (ERP) study. We analyzed cue- and target-locked ERPs from 23 adult patients with GTS and 26 matched controls who completed a computerized version of the WCST. Compared to controls, patients with GTS showed a marked increase in parietal cue-locked P3 activity, indicating enhanced proactive cognitive control. We conclude that the additional recruitment of proactive cognitive control might ensure flexible cognitive functioning in adult patients with GTS.     

Cobalamin (vitamin B(12)) in subacute combined degeneration and beyond: traditional interpretations and novel theories

Subacute combined degeneration (SCD) is a neuropathy due to cobalamin (Cbl) (vitamin B(12)) deficiency acquired in adult age. Hitherto, the theories advanced to explain the pathogenesis of SCD have postulated a causal relationship between SCD lesions and the impairment of either or both of two Cbl-dependent reactions. We have identified a new experimental model, the totally gastrectomized rat, to reproduce the key morphological features of the disease [spongy vacuolation, intramyelinic and interstitial edema of the white matter of the central nervous system (CNS), and astrogliosis], and found new mechanisms responsible for the pathogenesis of SCD: the neuropathological lesions in TGX rats are not only due to mere vitamin withdrawal but also to the overproduction of the myelinolytic tumor necrosis factor (TNF)-alpha and the reduced synthesis of the two neurotrophic agents, epidermal growth factor (EGF) and interleukin-6. This deregulation of the balance between TNF-alpha and EGF synthesis induced by Cbl deficiency has been verified in the sera of patients with pernicious anemia (but not in those with iron-deficient anemia), and in the cerebrospinal fluid (CSF) of SCD patients. These new functions are not linked to the coenzyme functions of the vitamin, but it is still unknown whether they involve genetic or epigenetic mechanisms. Low Cbl levels have also been repeatedly observed in the sera and/or CSF of patients with Alzheimer's disease or multiple sclerosis, but whether Cbl deficit plays a role in the pathogenesis of these diseases is still unclear.     

Poor school and cognitive functioning with silent cerebral infarcts and sickle cell disease

The authors evaluated education attainment and neuropsychological deficits in children with sickle cell disease (SCD) and silent cerebral infarcts. Children with silent infarcts had twice the rate of school difficulties as children without infarcts. Eighty percent of silent infarct cases had clinically significant cognitive deficits, whereas 35% had deficits in academic skills. Children with silent cerebral infarcts show high rates of poor educational attainment, cognitive deficits, and frontal lobe injury. Poor school performance in SCD is one indicator of silent infarcts.     

Cross-sensory gating in schizophrenia and autism spectrum disorder: EEG evidence for impaired brain connectivity?

Autism spectrum disorders (ASD) and schizophrenia are both neurodevelopmental disorders that have extensively been associated with impairments in functional brain connectivity. Using a cross-sensory P50 suppression paradigm, this study investigated low-level audiovisual interactions on cortical EEG activation, which provides crucial information about functional integrity of connections between brain areas involved in cross-sensory processing in both disorders. Thirteen high functioning adult males with ASD, 13 high functioning adult males with schizophrenia, and 16 healthy adult males participated in the study. No differences in neither auditory nor cross-sensory P50 suppression were found between healthy controls and individuals with ASD. In schizophrenia, attenuated P50 responses to the first auditory stimulus indicated early auditory processing deficits. These results are in accordance with the notion that filtering deficits may be secondary to earlier sensory dysfunction. Also, atypical cross-sensory suppression was found, which implies that the cognitive impairments seen in schizophrenia may be due to deficits in the integrity of connections between brain areas involved in low-level cross-sensory processing.     

Peripheric smell regions in patients with semicircular canal dehiscence: An MRI evaluation

ObjectivesWe investigated the peripheric smell regions in patients with semicircular canal dehiscence (SCD) by cranial MRI.MethodsIn this retrospective study, cranial MRI images of 186 adult patients were included. Group 1 consisted of 83 adult patients with SCD. The control group (Group 2) consisted of 83 healthy subjects without central vertigo. Olfactory bulb (OB) volume and olfactory sulcus (OS) depth were measured in all groups.ResultsIn group 1, SCD was detected on the right (33.7%), left (26.5%) sides and bilateral (39.8%). Localization of dehiscence was at superior SC (75.9%), posterior SC (21.7%), lateral SC (1.2%), and posterior + superior SCs (1.2%). OB volumes of the SCD group were significantly lower than the control group bilaterally (p < 0.05). There were no significant differences between OS depths of groups 1 and 2 (p > 0.05). In SCD group, there were positive correlations between OB volumes; OS depths; and OB volumes and OS depths (p < 0.05). In older patients, bilateral OS depth values got lower (p < 0.05). In females, left OB volume values were lower than males (p < 0.05). In right SCD (+) patients, left OS depth values got lower (p < 0.05).ConclusionWe concluded that possible changes in CSF dynamics may cause the development of SCD at thin bone segments; and a decrease in the OB volume. CSF leaks into the perineural sheet of the olfactory bulb (OB) maybe responsible for the decrease in the OB volume. In addition, minor trauma, infection, and inflammation may also be responsible for both coexistences of SCD development and OB volume decrease.     

Logical processing, affect, and delusional thought in schizophrenia

Deficits of logical reasoning have long been considered a hallmark of schizophrenia and delusional disorders. We provide a more precise characterization of "logic" and, by extension, of "deficits in logical reasoning." A model is offered to categorize different forms of logical deficits. This model acknowledges not only problems with making inferences, which is how logic deficits are usually conceived, but also problems in the acquisition and evaluation of premises (i.e., filtering of "input"). Early (1940-1969) and modern (1970-present) literature on logical reasoning and schizophrenia is evaluated within the context of the presented model. We argue that, despite a substantial history of interest in the topic, research to date has been inconclusive on the fundamental question of whether patients with delusional ideation show abnormalities in logical reasoning. This may be due to heterogeneous definitions of "logic," variability in the composition of patient samples, and floor effects among the healthy controls. In spite of these difficulties, the available evidence suggests that deficits in logical reasoning are more likely to occur due to faulty assessment of premises than to a defect in the structure of inferences. Such deficits seem to be provoked (in healthy individuals) or exacerbated (in patients with schizophrenia) by emotional content. The hypothesis is offered that delusional ideation is primarily affect-driven, and that a mechanism present in healthy individuals when they are emotionally challenged may be inappropriately activated in patients who are delusional.     

Psychosocial consequences of caregiver transitions for maltreated youth entering foster care: the moderating impact of community violence exposure

Youth who experience a greater number of caregiver transitions during childhood are at risk for developing a host of psychosocial problems. Although researchers have examined individual-level factors that may moderate this association, no known studies have examined the impact of community-level factors. The current study investigated whether community violence exposure (CVE) moderated the association between number of prior caregiver transitions and increases in levels of externalizing and internalizing problems for a sample of youth entering foster care. Participants included 156 youth (aged 9-11 at first assessment) removed from their homes because of maltreatment. Youth provided reports of caregiver transitions and CVE at baseline, and caregivers, teachers, and youth reported on externalizing and internalizing problems 18-22 months later. Results from hierarchical multiple regression analyses indicated that youth with a greater number of caregiver transitions and higher levels of CVE evidenced significant increases in levels of psychosocial problems. The results of the study are discussed in terms of their implications for child welfare services.     

Characteristics of cloned cerebrovascular endothelial cells following infection with Theiler's virus II. Persistent infection

Cloned cerebrovascular endothelial cells (CVE) persistently infected with Theiler's virus (PI-CVE) have been established and characterized. The CVE were derived from strains of mice that are susceptible (SJL/J and CBA) and resistant (BALB/c) to Theiler's virus-induced demyelination (TVID). The cells were persistently infected with either the BeAn or GDVII strains of Theiler's virus in vitro and studied at various passage levels for infectious virus, viral antigen and the expression of major histocompatibility complex (MHC) Class I and II antigens. The virus replicated to lower titers than in acutely infected CVE and appeared to be more cell-associated. Flow cytometric analysis revealed that 18–39% of the PI-CVE contained viral antigen. Persistently infected CVE derived from SJL/J and CBA mice expressed high levels of MHC Class I, whereas BALB/c PI-CVE did not. MHC Class II was upregulated by IFN-γ in SJL/J PI-CVE albeit at a slightly lower level than in uninfected CVE. In addition, the PI-CVE demonstrated increased levels of mRNA for IL-1β when compared to uninfected CVE.     

Sheehan’s syndrome and sickle cell disease: the story of Natasha*

ABSTRACT

Sheehan’s syndrome (SS) is one of the pituitary disorders caused by severe blood loss during childbirth leading to necrosis of the pituitary gland. Diagnosis is made following severe haemorrhage, failure to produce milk and failure to menstruate. Rare in countries with good obstetric care, SS is still frequent in those countries with poor healthcare services. The majority of papers published on SS do not mention psychological or neuropsychological sequelae. Of those that do, mood disorders are sometimes reported and occasionally cognitive problems are noted, typically attention, memory or executive deficits. We report on Natasha, a British woman of Caribbean ancestry with sickle cell disease (SCD), diagnosed at age 33 years with SS following the birth of her second child. We know of no reports of a person with both SCD and SS. Natasha has been seen regularly for neuropsychological assessment and treatment for over two years. There has been a slight increase in her cognitive functioning but she remains with Balint’s syndrome, so is unable to read. Treatment for this has been partially successful. Natasha presented with psychotic type symptoms when first seen but these have improved to a large extent. We believe that these symptoms are consistent with SS but the severe cognitive problems are more likely to be due to the strokes she suffered at the time of the haemorrhage and, possibly, to hypoxic damage caused by very low blood pressure.     

Detecting white matter injury in sickle cell disease using voxel-based morphometry

OBJECTIVE: Sickle cell disease (SCD) is associated with cerebrovascular disease, cerebral infarction, and cognitive dysfunction. This study aimed to detect the presence and extent of white matter abnormalities in individuals with SCD using voxel-based morphometry (VBM). METHODS: Thirty-six children and adolescents with SCD (age range, 9-24 years) and 31 controls (8-25 years) underwent magnetic resonance investigations using T1- and T2-weighted protocols. White and gray matter density maps were obtained from three-dimensional magnetic resonance imaging (MRI) data sets. Using VBM, we compared the maps between controls and SCD individuals with silent white matter infarct lesions (SCD+L; n = 16), and those without visible abnormality (SCD-L; n = 20). RESULTS: In comparison with controls, intelligence quotients (IQs) were lower in both SCD groups irrespective of presence of visible lesions. VBM showed widespread bilateral white matter abnormalities in the SCD+L group, extending beyond the regions of focal infarction in the deep anterior and posterior white matter borderzones. Bilateral white matter abnormalities were also observed in the SCD-L group, in locations similar to those in the SCD+L group. INTERPRETATION: VBM is sensitive to detection of widespread white matter injury in SCD patients in borderzones between arterial territories even in the absence of evidence of infarction. Those changes may contribute to cognitive deficits in this population.     

Red blood cells and thrombin generation in sickle cell disease

The prothrombotic nature of sickle cell disease (SCD) is evidenced by the chronically elevated levels of almost all coagulation activation biomarkers, and an increased incidence of certain thrombotic events, including venous thromboembolism. Numerous studies have attempted to define the extent and elucidate the mechanism of the observed increase in thrombin generation in SCD patients in vivo. In general, these studies were performed using thrombin generation assays in platelet poor or platelet rich plasma and showed little difference in endogenous thrombin potential between the SCD cohort and healthy matched controls. In SCD, erythrocytes and monocytes have been demonstrated to exhibit procoagulant characteristics. Thus, the absence of these cellular components in standard thrombin generation assays may fail to reflect global hypercoagulability in the whole blood of patients with SCD. We were therefore surprised to see no difference in net thrombin generation in tissue factor-initiated initiated clotting of whole blood from patients with SCD. However, we are continuing to reconcile these seemingly disparate observations by slight modifications of the whole blood model that include alternative coagulation triggers and a re-examination of the net thrombin generation when the protein/protein S system is simultaneously interrogated.     

"Time, place and person" and neurology]

The Department of Neurology, Hokkaido University, has been established since 1987, however the bases for neurology had been cultivated through psychiatry and neurosurgery in Hokkaido, especially under the pioneering work by Professor Mitsuo Tsuru. Hokkaido, the northernmost island of Japan, is located between 42 and 46 degrees, and the people have ancestors from various parts of Japan, since this island was reclaimed about 130 years ago. Three major neurological disorders, such as multiple sclerosis(MS), spinocerebellar degeneration(SCD) and Parkinson disease (PD), were discussed on the bases of Hokkaido island. We proved the existence of definite north-south gradient of MS, prevalence rate of 8.6 in Tokachi, Hokkaido, compared with 1-2 in the southern island. SCD, especially hereditary SCD, such as SCA1, SCA2 and SCA14 were reported from Hokkaido. The epidemiological studies of PD showed the prevalence rate of 104.6 at Iwamizawa, Hokkaido in 2000, and we proposed the prevalence rate of PD was close to those of Western countries under the collaborative works with Yonago, Kyoto, and Kagoshima by 2001. Neurology, and its specialization and training programs were also discussed to propose the importance of Neurology in Japan.