Chronic uncal herniation secondary to posterior fossa shunting: case report and literature review

Introduction  

Chronic herniation syndromes other than tonsillar herniation are not well-recognized. Transtentorial uncal herniation in its chronic form has been reported in only few case reports (Horowitz et al., J Neuroimaging 12:78–79, 2002; Naidich et al., Radiology 158:431–434, 1986; Ng and Valiante, J Clin Neurosci 16:944, 2009; Ng and Valiante, J Clin Neurosci 16:984, 2009). We hereby illustrate a case with this rare finding, including MR imaging, and analyze this phenomenon.     

Rare brain biopsy findings in a first ADEM-like event of pediatric MS: histopathologic,neuroradiologic and clinical features

Pediatric MS tends to present more often with an acute onset and a polysymptomatic form of the disease, possibly with encephalopathy and large tumefactive lesions similar to those observed in some cases of acute disseminated encephalomyelitis (ADEM), which makes it more difficult to differentiate between an explosive and severe onset of MS vs. ADEM. An ADEM-like first demyelinating event can be the first attack of pediatric MS, but international consensus definitions require two or more non-ADEM demyelinating events for diagnosis of MS. In our patient KIDMUS MRI criteria for MS (Mikaeloff et al. J Pediatr 144:246–252, 2004a; Mikaeloff et al. Brain 127:1942–1947, 2004b) were negative at first attack, but Barkhof criteria for lesion dissemination in space in adults (Barkhof et al. 120:2059–2069, 1997), Callen modified MS-criteria and Callen MS-ADEM criteria for children (Callen et al. Neurology 72:961–967, 2009a; Callen et al. Neurology 72:968–973, 2009b) were positive suggesting pediatric MS. As the clinical course was devastating with non-responsiveness upon high-dose immune modulatory therapy and due to the absence of an alternative diagnosis other than demyelinating disease brain biopsy was performed. Brain biopsy studies or autopsy case reports of fulminant pediatric MS patients are extremely rare. Histopathology revealed an inflammatory demyelinating CNS process with confluent demyelination, indicating the likelihood of a relapsing disease course compatible with an acute to subacute demyelinating inflammatory disease. This pattern was corresponding to the early active multiple sclerosis subtype I of Lucchinetti et al. (Ann Neurol 47(6):707–717, 2000).     

Microvessel length density, total length, and length per neuron in five subcortical regions in schizophrenia

Recent studies (Prabakaran et al. in Mol Psychiat 9:684–697, 2004; Hanson and Gottesman in BMC Med Genet 6:7, 2005; Harris et al. in PLoS ONE 3:e3964, 2008) have suggested that microvascular abnormalities occur in the brains of patients with schizophrenia. To assess the integrity of the microvasculature in subcortical brain regions in schizophrenia, we investigated the microvessel length density, total microvessel length, and microvessel length per neuron using design-based stereologic methods in the caudate nucleus, putamen, nucleus accumbens, mediodorsal nucleus of the thalamus, and lateral nucleus of the amygdala in both hemispheres of 13 postmortem brains from male patients with schizophrenia and 13 age-matched male controls. A general linear model multivariate analysis of variance with diagnosis and hemisphere as fixed factors and illness duration (patients with schizophrenia) or age (controls), postmortem interval and fixation time as covariates showed no statistically significant differences in the brains from the patients with schizophrenia compared to the controls. These data extend our earlier findings in prefrontal cortex area 9 and anterior cingulate cortex area 24 from the same brains (Kreczmanski et al. in Acta Neuropathol 109:510–518, 2005), that alterations in microvessel length density, total length, and particularly length per neuron cannot be considered characteristic features of schizophrenia. As such, compromised brain metabolism and occurrence of oxidative stress in the brains of patients with schizophrenia are likely caused by other mechanisms such as functional disruption in the coupling of cerebral blood flow to neuronal metabolic needs.     

Evidence that preimmunization with a heat-killed preparation of Mycobacterium vaccae reduces corticotropin-releasing hormone mRNA expression in the extended amygdala in a fear-potentiated startle paradigm

Posttraumatic stress disorder (PTSD) is a trauma and stressor-related disorder that is characterized by dysregulation of glucocorticoid signaling, chronic low-grade inflammation, and impairment in the ability to extinguish learned fear. Corticotropin-releasing hormone (Crh) is a stress- and immune-responsive neuropeptide secreted from the paraventricular nucleus of the hypothalamus (PVN) to stimulate the hypothalamic-pituitary-adrenal (HPA) axis; however, extra-hypothalamic sources of Crh from the central nucleus of the amygdala (CeA) and bed nucleus of the stria terminalis (BNST) govern specific fear- and anxiety-related defensive behavioral responses. We previously reported that preimmunization with a heat-killed preparation of the immunoregulatory environmental bacterium Mycobacterium vaccae NCTC 11659 enhances fear extinction in a fear-potentiated startle (FPS) paradigm. In this follow-up study, we utilized an in situ hybridization histochemistry technique to investigate Crh, Crhr1, and Crhr2 mRNA expression in the CeA, BNST, and PVN of the same rats from the original study [Fox et al., 2017, Brain, Behavior, and Immunity, 66: 70–84]. Here, we demonstrate that preimmunization with M. vaccae NCTC 11659 decreases Crh mRNA expression in the CeA and BNST of rats exposed to the FPS paradigm, and, further, that Crh mRNA expression in these regions is correlated with fear behavior during extinction training. These data are consistent with the hypothesis that M. vaccae promotes stress-resilience by attenuating Crh production in fear- and anxiety-related circuits. These data suggest that immunization with M. vaccae may be an effective strategy for prevention of fear- and anxiety-related disorders.     

Ataxia with vitamin E deficiency: update of molecular diagnosis

Ataxia with vitamin E deficiency (AVED) is a rare autosomal recessive neurodegenerative disease, due to mutations in TTPA gene (Arita et al. in Biochem J 306(Pt. 2):437–443, 1995; Hentati et al. in Ann Neurol 39:295–300, 1996), which encodes for α-TTP, a cytosolic liver protein that is presumed to function in the intracellular transport of α-tocopherol. This disease is characterized clinically by symptoms with often striking resemblance to those of Friedreich ataxia. The neurological symptoms include ataxia, dysarthria, hyporeflexia, and decreased vibration sense, sometimes associated with cardiomyopathy and retinitis pigmentosa (Mariotti et al. in Neurol Sci 25:130–137, 2004). Vitamin E supplementation improves symptoms and prevents disease progress (Doria-Lamba et al. in Eur J Pediatr 165(7):494–495, 2006). Over 20 mutations have been identified in patients with AVED. In the present paper we summarize the recent findings on molecular genetic of this disease including the list of the known mutations.     

Growing skull fractures

Introduction Growing skull fractures are a rare complication of head injuries (Ersahin et al. in Neurosurg Rev 23:139–144, 2000; Hayashi et al. in Childs Nerv Syst 13:349–351, 1997; Ramamurthi and Kalyanaraman in Neurosurgery 32:427–430, 1970; Zegers et al. in Eur J Pediatr 162:556–557, 2003). Although early diagnosis and prompt treatment are important to prevent the underlying progressive brain damage, the clinical presentation and the morphological investigations are rarely specific or sensitive shortly after the trauma.Discussion The authors present three cases of growing skull fractures: the use of ultrasonography (US) via the fracture line contributed to early diagnosis and prompt treatment in two cases. US was not performed in the third case, and this delayed management. Treatment consisted of a watertight duraplasty with a free flap of pericranium without cranioplasty. US via the fracture line appears to be a sensitive and reliable method of detecting the dural tears in the early stages of growing skull fractures.Conclusion Duraplasty alone with a flap of pericranium remains the simplest and least expensive method of treatment. Cranioplasty is not necessary in young children.     

A Qualitative Study of the Sources and Impact of Stress Among Urban Teachers

Although urban teachers are at-risk of experiencing significant work-related stress, urban teacher stress has been neglected in the research literature to date. Through semi-structured interviews conducted with a sample of K-4 urban teachers (N = 14) from three high-poverty schools in a large, Midwestern city, we examined teachers’ perceptions regarding sources and impact of stress and the resources needed to address identified stressors. Results from consensual qualitative research (CQR; Hill et al. in Couns Psychol, 25:517–572, 1997; Hill et al. in Consensual qualitative research: an update, 2005) suggest that at least one-half of the cases identified lack of resources, excessive workload, school-level disorganization, managing behavior problems, and accountability policies as significant sources of stress. The majority of teachers reported that occupational stress significantly impacted their personal relationships and physical health, and teachers identified human and material resources as most important to reducing work-related stress. Implications for organizationally based interventions and school policies to address urban teacher stress are discussed.     

Methylation and the human brain: towards a new discipline of imaging epigenetics

The field of imaging genetics traditionally studies unidirectional associations between genes, brain functioning, and behavior. In a recent study by Ursini et al. (J Neurosci 31:6692–6698, 2011), imaging genetics methods are combined with epigenetic marks in living human beings. This approach may lead to a new field of imaging epigenetics, providing more mechanistic insight into causal pathways of how gene and environment interact and affect brain development.     

Differential Regulation of Vasoactive Intestinal Peptide (VIP) in the Dentate Gyrus and Hippocampus via the NO-cGMP Pathway Following Kainic Acid-Induced Seizure in the Rat

We have previously shown that kainic acid (KA) increases nitric oxide (NO) synthase (NOS) production in the rat dentate gyrus (DG) and hippocampus (CA3), and NOS inhibition [(by N^G-nitro-L-arginine methylester (L-NAME)] modulates the vasoactive intestinal peptide (VIP)-responsive gene, activity-dependent neuroprotective protein, and alters neuro- and astrogliogenesis (Cosgrave et al. in Neurobiol Dis 30(3):281–292 2008, J Mol Neurosci 39(1–2):9–21, 2009, 2010). In the present study, using the same model we demonstrate that VIP synthesis is differentially regulated by the NO-cyclic guanosine monophosphate (cGMP) pathway in the DG and CA3 at 3 h and 3 days post-KA. At 3 h post-KA: In L-NAME+KA/7-nitroindazole (7-NI)+KA, stratum granulosum (SG) and subgranular zone (SGZ) cells were intensely stained for VIP when compared with L-NAME/7-NI/KA alone. Soluble guanylyl cyclase inhibitor, 1H-[1,2,4]oxadiazolo[4,3-a]quinoxalin-1-one (ODQ, blocks cGMP production), suppressed astrocytic activation (glial fibrillary acidic protein) but other cell types were VIP⁺; however, ODQ+KA suppressed overall VIP synthesis in the DG. At 3 days post-KA: In L-NAME+KA/7-NI+KA, SGZ and SG cells continued to express VIP, while in the KA alone, only SGZ cells were VIP⁺. ODQ increased VIP⁺ cells in the SG, and in contrast to 3 h, VIP-containing nNOS⁺ cells increased in ODQ+KA when compared to vehicle+KA. In the hippocampus, 7-NI/ODQ had no effect on VIP at 3 h/3 days, while L-NAME+KA at 3 days increased VIP⁺ cells, but reduced VIP-like immunoreactivity in astrocytes. These results suggest that the NO-cGMP pathway differentially regulates VIP in the DG and hippocampus during seizure.     

The Use of Structural Analysis to Develop Antecedent-based Interventions for Students with Autism

Evidence continues to maintain that the use of antecedent variables (i.e., instructional practices, and environmental characteristics) increase prosocial and adaptive behaviors of students with disabilities (e.g., Kern et al. in J Appl Behav Anal 27(1):7–19, 1994; Stichter et al. in Behav Disord 30:401–418, 2005). This study extends the literature by systematically utilizing practitioner-implemented structural analyzes within school settings to determine antecedent variables affecting the prosocial behavior of students with autism. Optimal antecedents were combined into intervention packages and assessed utilizing a multiple baseline design across settings. All three students demonstrated improvement across all three settings. Rates of engagement and social interaction were obtained from classroom peers to serve as benchmark data. Findings indicate that practitioners can implement structural analyzes and design corresponding interventions for students with ASD within educational settings.     

The Orexin 1 Receptor (HCRTR1) Gene as a Susceptibility Gene Contributing to Polydipsia-Hyponatremia in Schizophrenia

The underlying pathophysiology of primary polydipsia in schizophrenia (SCZ) is poorly understood. Our previous study, however, suggested that this condition may have a genetic component [Shinkai et al 2003 Am J Med Genet 119B 7–12]. Orexins, also called hypocretins, play an important role in feeding and drinking behavior. Administration of orexin in rats has been shown to induce increased water intake with a longer-lasting effect than angiotensin II, which is also known as a potent dipsogen. Meerabux et al. [2005 Biol Psychiatry 58 401–407] reported an association between the 408Val allele of the orexin 1 receptor (HCRTR1) gene and polydipsia-hyponatremia in a sample of Japanese patients with SCZ. In the present study, we attempted to replicate the findings of Meerabux et al. in an independent Japanese case-control sample. Our sample included 312 patients with SCZ (DSM-IV) (65 with polydipsia and 247 without polydipsia). We also observed an association between the HCRTR1 Ile408Val polymorphism and polydipsia (genotype distribution: χ² = 9.85, df = 2, P = 0.007). Meerabux et al. (2005) previously demonstrated an association between the 408Val allele of the HCRTR1 gene and polydipsia. In contrast with Meerabux et al. study, we found that the 408Ile allele was associated with polydipsia in our sample (χ² = 8.00, df = 1, P = 0.0047; OR = 0.53; 95%CI = 0.34–0.83). How either allele contributes to the development of polydipsia in SCZ is unclear at this stage. It is possible that Ile408Val polymorphism is a non-functional marker that lies in linkage disequilibrium with an as-yet undetected functional variant. In any case, our results support the hypothesis that the HCRTR1 Ile408Val polymorphism may confer susceptibility to polydipsia in SCZ. Further studies examining the association between the orexin system and polydipsia in SCZ are warranted. Yuko Fukunaka and Takahiro Shinkai contributed equally to this work.     

The Walking Corsi Test (WalCT): standardization of the topographical memory test in an Italian population

Selective visuo-spatial memory deficits can seriously affect many aspects of daily life; for example, an individual may not remember where he put an object or which path he took to reach his destination. In general, visuo-spatial memory is assessed through pen-and-paper tests that mainly assess memory components in peripersonal space. Recent studies (Piccardi et al. in Exp Brain Res 206:171–177, 2010; Piccardi et al. in Neuropsychol Dev Cogn B Aging Neuropsychol Cogn 18:362–384, 2011) have shown that brain-damaged patients selectively fail on navigation memory tasks but not on other tests of visuo-spatial memory ability. These findings underline the need for a standardized test that measures memory in navigation separately from other types of visuo-spatial memory. Here, we report the validation of the Walking Corsi Test (WalCT: Piccardi et al. in Neurosci Lett 432:127–131, 2008) on 289 individuals aged 15–86 years. The WalCT is a new instrument that assesses topographical memory in real environments and reproduces on a large-scale version the Corsi Block-Tapping Test (CBT: Corsi in Unpublished doctoral dissertation, McGill University, Montreal, 1972). The WalCT has been used in clinical practice and has proven sensitive in detecting navigational memory deficits even in individuals who have no other memory impairments (Piccardi et al. in Exp Brain Res 206:171–177, 2010; Piccardi et al. in Neuropsychol Dev Cogn B Aging Neuropsychol Cogn 18:362–384, 2011; Bianchini et al. in Neuropsychologia 48:1563–1573, 2010 ).     

Cognitive Behavioral Therapy and Schizophrenia: A Survey of Clinical Practices and Views on Efficacy in the United States and United Kingdom

Research has shown that cognitive-behavioral therapy (CBT) is effective in the treatment of schizophrenia (Wykes et al. in Schizophr Bull 34(3):523–537, 2008). The majority of this research has been conducted in the United Kingdom (Beck and Rector in Am J Psychother 54:291–300, 2000) where the National Health Service recommends that CBT be delivered to all people with schizophrenia (NICE in Schizophrenia: core interventions in the treatment and management of schizophrenia in primary and secondary care (update). , 2009). In contrast, the corresponding American Psychiatric Association guidelines describe CBT as an adjunctive technique that “may benefit” patients (Lehman et al. in Am J Psychiatry 161:1–56, 2004, p. 35). Anecdotal evidence also suggests a difference between UK and US clinicians’ use of and views on CBT with schizophrenia (Tarrier in Clinical handbook of psychological disorders: a step-by-step treatment manual. Guilford, New York, 2008). In the present study 214 clinicians in the UK and US completed an internet survey examining this apparent discrepancy. UK and US participants were equally aware that empirical research supports the efficacy of CBT with schizophrenia. However, UK participants were more likely to practice CBT, rated CBT effectiveness more highly, and were more optimistic about the chances of recovery. These findings suggest fundamental differences in the attitudes and practices of UK and US clinicians.     

Investigating Multitasking in High-Functioning Adolescents with Autism Spectrum Disorders Using the Virtual Errands Task

Using a modified version of the Virtual Errands Task (VET; McGeorge et al. in Presence-Teleop Virtual Environ 10(4):375–383, 2001), we investigated the executive ability of multitasking in 18 high-functioning adolescents with ASD and 18 typically developing adolescents. The VET requires multitasking (Law et al. in Acta Psychol 122(1):27–44, 2006) because there is a limited amount of time in which to complete the errands. ANCOVA revealed that the ASD group completed fewer tasks, broke more rules and rigidly followed the task list in the order of presentation. Our findings suggest that executive problems of planning inflexibility, inhibition, as well as difficulties with prospective memory (remembering to carry out intentions) may lie behind multitasking difficulties in ASD.     

Inferior Olive Response to Passive Tactile and Visual Stimulation with Variable Interstimulus Intervals

The unique anatomical and electrophysiological features of the inferior olive and its importance to cerebellar function have been recognized for decades. However, understanding the exact function of the inferior olive has been limited by the general lack of correlation between its neural activity and specific behavioral states. Electrophysiological studies in animals showed that the inferior olive response to sensory stimuli is generally invariant to stimulus properties but is enhanced by unexpected stimuli. Using functional magnetic resonance imaging in humans, we have shown that the inferior olive is activated when subjects performed a task requiring perception of visual stimuli with unpredictable timing (Xu et al. J Neurosci 26(22):5990–5995, 2006, Liu et al. J Neurophysiol 100(3):1557–1561, 2008). In the current study, subjects were scanned while passively perceiving visual and tactile stimuli that were rendered unpredictable by continuously varying interstimulus intervals (ISIs). Sequences of visual stimuli and tactile stimuli to the right hand were presented separately within the same scanning session. In addition to the activation of multiple areas in the cerebellar cortex consistent with previous imaging studies, the results show that both tactile and visual stimulation with variable ISIs were effective in activating the inferior olive. Together with our previous findings, the current results are consistent with the electrophysiological studies in animals and further support the view that the inferior olive and the climbing fiber system primarily convey the temporal information of sensory input regardless of the modality.     

Differentiation of High-Functioning Autism and Asperger’s Disorder Based on Neuromotor Behaviour

Autism and Asperger’s disorder (AD) are characterised by impairments in social interaction, stereotypic behaviours or restricted interests. Although currently listed as distinct clinical disorders, the validity of their distinction remains controversial. This study examined gait in children with autism and AD. Eleven children with high-functioning autism and eleven children with AD completed a series of walking tasks. Results indicated distinct movement disturbance; these findings are discussed in light of seminal papers in this field by Vilensky et al. (Arch Neurol 38:646–649, 1981) and Hallett et al. (Arch Neurol 50:1304–1308, 1993) who interpret the gait of individuals with autism using parkinsonian and cerebellar-ataxia patient models, respectively. Distinctions in gait patterns implicating perhaps unique motor circuit disturbances support the hypothesis that autism and AD may have unique neurodevelopmental trajectories.     

Triple jeopardy: impact of partner violence perpetration,mental health and substance use on perceived unmet need for mental health care among men

Objectives  

To examine the relationship between intimate partner violence (IPV) perpetration, serious mental illness, and substance use and perceived unmet need for mental health treatment in the past year among men in the general population using the behavioral model for health-care use (Aday and Anderson in Health Serv Res 9:208–220, 1974; Andersen in A behavioral model of families’ use of health services, 1968; Andersen in Med Care 46:647–653, 2008).     

Brief Report: Predicting Inner Speech Use Amongst Children with Autism Spectrum Disorder (ASD): The Roles of Verbal Ability and Cognitive Profile

Studies of inner speech use in ASD have produced conflicting results. Lidstone et al., J Autism Dev Disord (2009) hypothesised that Cognitive Profile (i.e., discrepancy between non-verbal and verbal abilities) is a predictor of inner speech use amongst children with ASD. They suggested other, contradictory results might be explained in terms of the different composition of ASD samples (in terms of Cognitive Profile) in each study. To test this, we conducted a new analysis of Williams et al.’s, J Child Psychol Psychiatry 48(1): 51–58 (2008) data on inner speech use in ASD. This revealed verbal ability predicted inner speech use on a short-term memory task over and above Cognitive Profile, but not vice versa. This suggests multiple factors determine whether children with ASD employ verbal mediation.     

The changing incidence of myelomeningocele and its impact on pediatric neurosurgery: a review from the Children’s Memorial Hospital

Incidence  Worldwide, the incidence of neural tube defects (NTDs) varies from 0.17 to 6.39 per 1,000 live births. The declining prevalence of myelomeningocele, the most common NTD, is secondary to several factors including folic acid fortification, prenatal diagnosis with termination of affected fetuses, and unknown factors. Impact of changes  Of those born with myelomeningocele, survival during infancy and preschool years has improved over the last 25 years (Bowman et al., Pediatr Neurosurg 34:114–120, 4). Fewer newborns today require shunt placement, which will hopefully improve the long-term mortality associated with this disease (Chakraborty et al., J Neurosurg Pediatr 1(5):361–365, 13, unpublished data). Of a cohort born in 1975–1979 and treated at a single US institution, 74% have survived into young adulthood. Clinical implications  One of the greatest challenges facing these young adults is the transitioning of their medical care into an adult medical community.