The whole spectrum of cystic periventricular leukomalacia of the preterm infant: results from a large consecutive case series

Purpose

The purpose of this study is to describe features of cystic periventricular leukomalacia (PVL) in a large consecutive cohort study including long-term neurodevelopmental follow-up.

Methods

We performed a retrospective single-centre cohort study including all preterm infants ≤35 weeks of gestational age with PVL diagnosed by ultrasound scans (US) from a tertiary care university hospital between 1988 and 2012.

Results

The majority of 160 consecutively diagnosed cases had a gestational age between 28 and 32 weeks (60.6 %), and male sex was predominant (60.6 %). The most common associated clinical findings included respiratory distress syndrome, preterm premature rupture of the membranes, and chorioamnionitis (57.5, 49.4, and 39.4 %, respectively). Infants presented with apnoeas in 66.3 and neonatal seizures in 23.1 %. Any kind of respiratory support was present in 75.0 %. Associated low-grade intraventricular haemorrhage was evident in 33.1, high-grade haemorrhage in 9.4 %. Cysts were located on both hemispheres in 75 % and PVL grades 3 and 4 were predominant (75.6 %). Neurodevelopmental follow-up of 146 cases at a median age of 72 months revealed normal development in 11.0, mental retardation in 50.0, and cerebral palsy in 83.6 %. Visual impairment was diagnosed in 21.9 % and hearing impairment in one case. A quarter of cases (27.4 %) developed seizure disorders. Outcome data were significantly better in unilateral compared to bilateral PVL.

Conclusions

Long-term neurodevelopmental outcome of bilateral PVL always was adverse and different from unilateral PVL. The latter might be negatively influenced by associated intra- and periventricular haemorrhages.

     

Autonomic dysfunction,immune regulation,and multiple sclerosis

Objective

To review existing evidence regarding interactions between the autonomic nervous system and the immune system functions in multiple sclerosis.

Methods

We reviewed the literature regarding new insights linking autonomic dysfunction to immune deregulation in multiple sclerosis, with particular focus on the specific influence of sympathetic and parasympathetic dysfunction on inflammatory and neurodegenerative processes.

Results

Autonomic dysfunction is common in multiple sclerosis, representing a significant cause of disability. Several connections between pathologic immune pathways and the autonomic nervous system function were found.

Conclusions

Autonomic dysfunction may enhance inflammatory and neurodegenerative pathways that are of major importance in multiple sclerosis. Autonomic dysfunction can present with highly variable manifestations. Sympathetic and parasympathetic dysfunction displays different patterns in multiple sclerosis, with specific impact on inflammation and neurodegeneration.

     

Autonomic function and brain volume

Objective

The aim of this study is to review the evidence on the role of the autonomic nervous system as a determinant of brain volume. Brain volume measures have gained increasing attention given its biological importance, particularly as a measurement of neurodegeneration.

Methods

Using an integrative approach, we reviewed publications addressing the anatomical and physiological characteristics of brain autonomic innervation focusing on evidence from diverse clinical populations with respect to brain volume.

Results

Multiple mechanisms contribute to changes in brain volume. Autonomic influence on cerebral blood volume is of significant interest.

Conclusion

We suggest a role for the autonomic innervation of brain vessels in fluctuations of cerebral blood volume. Further investigation in several clinical populations including multiple sclerosis is warranted to understand the specific role of parenchyma versus blood vessels changes on final brain volume.

     

Clinical Relevance of Steroid Use in Neuro-Oncology

Background

Corticosteroids are commonly used in the management of primary central nervous system (CNS) tumors and CNS metastases to treat cancer- and treatment-related cerebral edema and improve neurologic function. However, they are also associated with significant morbidity and mortality, given their wide range of adverse effects.

Purpose of Review

To review the mechanism of action, pharmacology, and toxicity profile of corticosteroids and to critically appraise the evidence that supports their use in neuro-oncologic practice based on the latest scientific and clinical data.

Recent Findings

Recent data suggest that corticosteroids may negatively impact survival in glioma patients. In addition, corticosteroids should be incorporated as a standard criterion to assess a patient’s clinical and radiographic response to treatment.

Summary

Corticosteroids should be used judiciously in neuro-oncologic patients, given the potential deleterious effects on clinical outcome and patient survival. Anti-angiogenic agents, which lack these adverse effects, may be a reasonable alternative to corticosteroids.

     

Does fatigue in Parkinson’s disease correlate with autonomic nervous system dysfunction?

Background

Despite its negative impact on quality of life, fatigue in Parkinson’s disease (PD) remains an under-recognized issue and the underlying pathology is undetermined.

Objective

To contribute at understanding the pathogenesis of fatigue in a naturalistic cohort of cognitively intact PD patients.

Methods

In a Caucasian population of PD patients (n?=?27), we evaluated to what extent fatigue (quantified as PFS-16 score) is associated with PD duration and with autonomic dysfunction, studied by both MIBG scintigraphy and autonomic nervous system testing. The latter included the head-up tilt test, Valsalva maneuver, deep breathing, and handgrip tests.

Results

PFS-16 score correlated with disease duration (R?=?0.57, p?=?0.002). Fatigue showed a clear correlation with deep breathing test (R?=???0.53, p?=?0.004) but not with the MIBG H/M ratios.

Conclusions

Our data are consistent with a multifactorial pathogenesis of fatigue and with effects of dopamine depletion in PD-related fatigue; on the other hand, our findings do not support a role for sympathetic denervation in PD-related fatigue.

     

Influence of sex,menstrual cycle,and oral contraceptives on the cerebrovascular response to paced deep breathing

Purpose

Deep breathing assesses autonomic function; however, many researchers/clinicians do not account for hyperventilation, brain blood flow or blood pressure.

Methods

Men and women (with/without oral contraceptives) participated. women participated during low and high hormone phases of the menstrual cycle. Blood pressure, end-tidal carbon dioxide, middle cerebral artery velocity and cerebrovascular resistance were assessed.

Results

Deep breathing decreased end-tidal carbon dioxide and middle cerebral artery velocity while increasing cerebrovascular resistance in all participants; blood pressure decreased in men. There were no influences of menstrual cycle or oral contraceptives.

Conclusions

Men have different autonomic responses to deep breathing compared to women.

     

Prevalence and characteristics of self-reported physical and mental disorders among adults with hearing loss in Denmark: a national survey

Purpose

Existing research shows that people with hearing loss have a high risk of additional physical and mental disorders. However, only a few population-based studies have been conducted. This study assesses the prevalence and characteristics of additional disorders among adults with hearing loss in Denmark and thereby contributes a population-based study to this area of research.

Method

Data on self-reported physical and mental disorders from a national survey of 772 adults with hearing loss were compared to corresponding data from a national survey of 18,017 adults from the general population.

Results

People with hearing loss reported more physical and mental disorders than the general population. Specifically, they reported higher incidences of visual impairment, cerebral palsy, intellectual impairment, and “other mental disorders”.

Conclusion

Adults with hearing loss have a greater risk of additional physical and mental disorders. It is important for clinicians to have some understanding of the communication needs and characteristics of deaf and hard-of-hearing patients, so that they can recognize and treat symptoms and provide appropriate support.

     

Transsphenoidal meningoencephalocele associated with cleft lip and palate: challenges for diagnosis and surgical treatment

Purpose

The aim of this study was to highlight the challenges for early diagnosis and the difficulties observed in surgical treatment of patients with transsphenoidal meningoencephalocele associated with cleft lip and/or palate.

Method

We evaluated six male patients treated over the course of 4 years. Five patients presented encephalic herniation with nonfunctional brain tissue; one of these presented herniation of the pituitary gland and cerebral ventricles.

Results

All the patients received surgical treatment for the cleft lip and/or palate. Only one patient underwent repair of the meningoencephalocele, via nasal endoscopy. There were no postprocedural clinical or surgical complications.

Conclusion

The tendency is to avoid neurosurgery, opting for periodic follow-up with magnetic resonance imaging. In the presence of cleft palate, palatoplasty is essential to protect the meningoencephalocele.

     

Anti-MAG associated cerebellar ataxia and response to rituximab

Background

Myelin-associated glycoprotein (MAG) is a glycoprotein specific to Schwann cells. Schwann cells produce myelin for nerve cells in the peripheral nervous system. MAG also plays a role in the central nervous system (CNS) by maintaining myelin integrity and inhibiting axonal regeneration from cerebellar neurons. There is a well-established link between distal demyelinating neuropathy and anti-MAG antibodies in patients with monoclonal gammopathy of unknown significance. We describe a series of five patients with anti-MAG antibodies with evidence of cerebellar rather than just sensory ataxia and our experience of treatment with rituximab.

Methods

Cerebellar ataxia was clinically suspected and confirmed using magnetic resonance spectroscopy (MRS) of the cerebellum. All patients underwent detailed nerve conduction studies.

Results

Four patients were males. The ages ranged from 64 to 82 years. All patients were anti-MAG positive and also had IgM monoclonal gammopathy. Four patients had neuropathy, whilst one had no evidence of neuropathy. All patients were treated with rituximab and showed improvement in the MRS parameters of the cerebellum.

Conclusion

Anti-MAG antibodies might be involved in the pathogenesis of idiopathic sporadic ataxias, even in the absence of peripheral neuropathy. Rituximab seems to be a promising therapeutic intervention for those cases.

     

Changes in the sympathetic innervation of the gut in rotenone treated mice as possible early biomarker for Parkinson’s disease

Introduction

Involvement of the peripheral nervous system (PNS) is relatively common in Parkinson’s disease (PD) patients. PNS alterations appear early in the course of the disease and are responsible for some of the non-motor symptoms observed in PD patients. In previous studies, we have shown that environmental toxins can trigger the disease by acting on the enteric nervous system.

Material and methods

Here, we analyzed the effect of mitochondrial Complex I inhibition on sympathetic neuritis in vivo and sympathetic neurons in vitro. Combining in vivo imaging and protein expression profiling.

Results

we found that rotenone, a widely used mitochondrial Complex I inhibitor decreases the density of sympathetic neurites innervating the gut in vivo, while in vitro, it induces the redistribution of intracellular alpha-synuclein and neurite degeneration. Interestingly, sympathetic neurons are much more resistant to rotenone exposure than mesencephalic dopaminergic neurons.

Conclusion

Altogether, these results suggest that enteric sympathetic denervation could be an initial pre-motor alteration in PD progression that could be used as an early biomarker of the disease.

     

Heart-rate response to alpha<Subscript>2</Subscript>-adrenergic receptor antagonism by antipsychotics

Purpose

To explore the relationship between antipsychotic-associated antagonism of alpha₂-adrenergic receptors and resting heart rate in individuals with schizophrenia.

Methods

Thirty-one inpatients treated with antipsychotics were included in this exploratory analysis. Antipsychotic doses were converted to haloperidol equivalents for alpha₂-adrenergic receptor antagonism. Resting heart rate was measured with the patient in the seated upright posture.

Results

After controlling for confounding variables, the relationship between alpha₂-adrenergic receptor antagonism and resting heart rate demonstrated a positive linear effect (P = 0.002) as well as a nonlinear effect that accounted for an additional 14% of the variability in resting heart rate (P = 0.005).

Conclusion

The observed inverted-U relationship between alpha₂-adrenergic receptor antagonism and resting heart rate can possibly be attributed to an altered response of beta₁-adrenergic receptors to increased norepinephrine release. Further investigations are required to confirm this exploratory finding, taking into account additional variables that include other receptors which either directly or indirectly influence heart rate.

ClinicalTrials.gov Identifier

NCT01392885.

     

Dual-Energy CT to Diagnose Pseudoedema in Contrast-Induced Encephalopathy Following Cerebral Angiography

Background

Contrast-induced encephalopathy (CIE) is a syndrome that may be clinically unrecognized and misdiagnosed as cerebral edema.

Methods

Case report and review.

Results

A 72-year-old woman was admitted for elective endovascular embolization of a 10-mm left anterior communicating artery aneurysm. One hour post-procedure, she acutely developed global aphasia. Emergent head computed tomography (CT) and computed tomography-angiography (CTA) showed high attenuation of the left hemispheric subarachnoid spaces interpreted as hemispheric edema; emergent magnetic resonance imaging revealed left hemispheric punctate infarcts. At 12 h, she developed right hemiparesis and encephalopathy. Repeat CTA and CT perfusion revealed decreased left hemisphere cerebral blood flow and diminutive caliber of distal left middle cerebral artery territory vasculature. Repeated angiography with intra-arterial verapamil and systemic blood pressure augmentation were performed for presumed vasospasm. At 20 h, head CT was concerning for worsening left hemispheric edema, but dual-energy, iodine-subtracting sequences revealed significant contrast extravasation contributing to the appearance of sulcal effacement but without actual edema. Out of concern for blood–brain barrier breakdown from CIE, pressor augmentation was discontinued and the patient gradually improved to full neurological recovery within 72 h of symptom onset.

Conclusions

Our case is the first known to report the use of dual-energy, iodine-subtracting CT as a diagnostic tool in differentiating between cerebral edema and pseudoedema in CIE.

     

Cerebral hemispheric low-grade glial tumors in children: preoperative anatomic assessment with MRI and DTI

Purpose

The aims of this study are to analyze how the nature and the behavior of low-grade glial tumors (LGGT) in children may correlate with the anatomy of the cerebral hemispheres and to evaluate the consequent impact of diffusion tensor imaging (DTI) techniques in the presurgical assessment.

Methods

This is a combined review of a series of 155 cases of LGGT and of the recent literature on the subject.

Results

The cases retrieved from our data bank were divided in central hemispheric tumors (basal ganglia and thalami) (36 cases), glioneuronal cortical-based tumors (49 cases), and glial tumors of the cerebral mantle (70 cases). A close correlation was found in the thalamus between the primary location of the tumor (juxta-ventricular, inferior, lateral, bilateral) and its extension (ventricular lumen, midbrain and mesial temporal, globus pallidus, respectively) which may relate to the connectivity. Among the glioneuronal tumors, most gangliogliomas were located in the temporal lobe and especially in the mesial temporal structures. In addition, the morphologic feature of the ganglioglioma was different there from the neocortical areas. As a complementary approach, DTI data may assist in evaluating the structure and the extension of the LGGT, in addition to planning the surgical strategy.

Conclusions

In the cerebral hemispheres like in the rest of the central nervous system, there is some degree of correlation between the anatomy and the nature, appearance, and behavior of the LGGT in children.

     

The Mediating Role of Meaning in the Association between Stress and Health

Background

Stress is a common feature of life and has routinely been linked with negative health outcomes. However, meaning has been identified as a possible buffer against stress.

Purpose

The purpose of the current study was to examine whether the relationship between stress and health was mediated by meaning in life.

Methods

Drawing from Wave 1 of the Landmark Spirituality and Health Study, a nationally representative sample of adults, participants (N = 1871) reported their level of stress in the past 12 months, current meaning in life, health (measured as minor symptoms, major conditions, and overall health), and provided a blood sample for biomarker of immune system functioning (i.e., presence of Epstein–Barr virus antibodies).

Results

Results revealed an indirect effects model in which stress was inversely associated with meaning. Higher meaning was related to better self-reported health (across minor, major, and overall health measures), which, in turn, was associated with better immune system functioning.

Conclusions

These findings suggest that part of the negative effect of stress on health is accounted for by reduced meaning.

     

Is cerebral vasomotor reactivity impaired in Parkinson disease?

Purpose

The ability of a blood vessel to change diameter in response to a change in carbon dioxide concentration is often referred to as vasomotor reactivity. This study aimed to determine whether vasomotor reactivity is impaired in patients with idiopathic Parkinson's Disease in comparison to healthy controls.

Methods

Transcranial Doppler was used to measure cerebral blood flow velocity in the middle cerebral arteries at baseline and under hypocapnic conditions in 40 patients with idiopathic Parkinson's disease and 50 healthy controls.

Results/Conclusions

Vasomotor reactivity, assessed under hypocapnic conditions, is not impaired in patients with idiopathic Parkinson's Disease in comparison to healthy controls.

     

Nonkonvulsiver Status epilepticus

Background

Nonconvulsive status epilepticus (NCSE) is associated with a poor outcome and is furthermore a diagnostic challenge in routine clinical work.

Objective

Several sets of diagnostic criteria exist. What is the difference between them and which ones have been evaluated? Which concept can be recommended in the light of current knowledge?

Material and methods

A survey on the current literature is provided and a structured approach for diagnosing NCSE is given.

Results

Only one study on evaluation of the diagnostic criteria for NCSE exists, i.?e. the “Salzburg criteria for NCSE”, which were investigated retrospectively in a multicenter study. The diagnosis of NCSE is based on clinical and EEG data. The additional implementation of paraclinical information, such as cerebral imaging, emergency and toxicology laboratories is mandatory.

Conclusion

Currently available criteria enable efficient and effective management of the majority of patients suspected of having NCSE; however, in individual cases it has to be taken into consideration that the criteria can be falsely positive or falsely negative.

     

Selective dorsal rhizotomy as an alternative to intrathecal baclofen pump replacement in GMFCS grades 4 and 5 children

Background

Conventionally, selective dorsal rhizotomy (SDR) has been reserved for ambulant children and implantation of intrathecal baclofen (ITB) pump for non-ambulant children with cerebral palsy. Rather than replacing the ITB pump in selected Gross Motor Function Classification System (GMFCS) grades 4 and 5 children, we elected to undertake SDR instead. We discuss the rationale and outcomes.

Objectives

To assess if children with severe spasticity treated with long-term ITB pump would benefit from SDR as alternative procedure to replacement of ITB pump.

Method

This study is a prospective review of ten children with severe spasticity. Indications for ITB pump replacement in 3/10 children were previous ITB pump infection and the remaining seven were nearing depletion of drug delivery system. Pre- and post-SDR mean modified Ashworth scores, assessment of urological function and survey of parent/carer satisfaction were undertaken.

Result

Mean Ashworth score reductions post-SDR in the lower limbs and upper limbs were 2.4 and 1.70, respectively. An improvement in urological function was also noticed in 27 % of patients. Overall, 90 % of parents/carers felt that functional outcome with SDR was improved compared with that of ITB.

Conclusion

SDR in comparison to ITB in this subgroup is cheaper, less intrusive by avoiding refills/replacement and found to be more effective than ITB in reducing spasticity and providing ease for nursing care. We therefore suggest that consideration should be given to SDR as an alternative in patients previously implanted with ITB systems complicated by infection or nearing end of battery life.

     

PHACE syndrome is associated with intracranial cavernous malformations

Introduction

PHACE syndrome is a neurocutaneous disorder involving large facial hemangiomas in association with posterior fossa abnormalities, cerebral arterial anomalies, cardiac defects, and eye abnormalities. A recent consensus statement has delineated criteria necessary for the diagnosis of PHACE syndrome. Extracutaneous manifestations of PHACE syndrome predominately affect the cerebrovascular system. To date, there are no reports of cerebral cavernous malformations (CCMs) in children with PHACE syndrome.

Methods

We reviewed the charts of children admitted to the Children’'s Hospital of Pittsburgh who met criteria for PHACE syndrome, and evaluated neuroimaging for cerebrovascular abnormalities, including the finding of CCMs.

Results

Six children met criteria for PHACE syndrome at our institution over a 10-year period. All children were female. All children had cerebrovascular abnormalities sufficient to meet major criteria for diagnosis. Four children (66.7 %) were found incidentally to have CCMs; all lesions measured less than 5 mm at the time of diagnosis and were asymptomatic.

Conclusion

At present, CCMs are not listed among the diagnostic criteria for PHACE syndrome, and they have not previously been reported in association with PHACE syndrome. Hypoxic injury in utero may be the common denominator in the pathogenesis of many of the abnormalities already accepted in the criteria for PHACE syndrome and the formation of CCMs. In the setting of PHACE syndrome, we encourage clinicians to evaluate children for CCMs, which are readily apparent on the already-recommended screening MRIs.

     

Glioma after cerebral hydatid disease

Background

The authors present the case of a 31-year-old man with a malignant glioma. He had been treated for cerebral hydatid as a child, and 22 years later he developed a glioma at the site of his previous disease.

Discussion

Could chronic inflammatory change following intracranial hydatid disease have induced neoplastic transformation of glial cells?

     

Perceptions of Neighborhood Park Quality: Associations with Physical Activity and Body Mass Index

Background

Parks are important resources for physical activity (PA), yet few studies have examined how perceptions of park characteristics relate to PA and health.

Purpose

This study investigated associations between perceptions of neighborhood park quality and overall moderate-to-vigorous PA (MVPA), park-based PA, and body mass index (BMI).

Methods

Data were collected via questionnaire from 893 households in Kansas City, Missouri.

Results

The newly developed neighborhood park quality scale demonstrated good test–retest and internal reliability. Residents’ perceptions of neighborhood park quality were related to PA and health outcomes. Perceiving parks as a benefit was positively related to overall MVPA and park-based PA and negatively related to BMI. Perceptions of well-used parks were positively related to BMI, while perceived cleanliness was negatively related to park-based PA.

Conclusions

Better measuring and understanding how perceptions of local parks are associated with PA and health can improve appreciation of how parks facilitate active living.