Impact of an education program to facilitate nurses' discussions of existential issues in neurological care.

OBJECTIVES: Discussing existential issues is integral to caring for people with acute, progressive, or life-limiting neurological illness, but there is a lack of research examining how nurses approach existential issues with this patient group and their family members. The purpose was to examine the experiential impact of an educational program for nurses designed to facilitate discussions of existential issues with patients and family members in neurological wards. METHOD: Nurses in inpatient and outpatient care at a neurological clinic in Sweden were invited to participate in an education program about discussing existential issues with patients and their family members as related to neurological conditions. The evaluation of the program and of the nurses' view of discussing existential issues was conducted through focus groups before and after participation. The data were analyzed by qualitative content analysis. RESULTS: The program gave nurses a deeper understanding of existential issues and how to manage these conversations with patients and their family members. Both internal and external barriers remained after education, with nurses experiencing insecurity and fear, and a sense of being inhibited by the environment. However, they were more aware of the barriers after the education, and it was easier to find strategies to manage the conversations. They demonstrated support for each other in the team both before and after participating in the program. SIGNIFICANCE OF RESULTS: The educational program gave nurses strategies for discussing existential issues with patients and family members. The knowledge that internal and external barriers impede communication should compel organizations to work on making conditions more conducive, for example, by supporting nurses to learn strategies to more easily manage conversations about existential issues and by reviewing the physical environment and the context in which they are conducted.

Wearables in epilepsy and Parkinson's disease-A focus group study.

OBJECTIVES: Wearable sensors that measure movement and physiological variables are attractive for clinical evaluation of neurological diseases such as epilepsy and Parkinson's disease (PD). The aim of this study was to explore perceptions regarding the use of wearable technology in disease monitoring and management as reported by individuals with epilepsy and Parkinson's disease as well as health professionals working with these patient groups. MATERIALS AND METHODS: Six patient groups (n=25) and two groups with health professionals (n=15) participated in this qualitative, descriptive study with focus group interviews. A manifest qualitative content analysis was used. RESULTS: Four categories and nine subcategories emerged from the analysis. Participants saw possible benefits for improved treatment effect and valued this benefit more than possible inconvenience of wearing the sensors. Discrete design and simplicity were considered as facilitators for improved usability. They emphasized the importance of interactive information between patients and health professionals. However, they were concerned about unclear information and inconclusive recordings and some fears about personal integrity were at odds with the expectations on interactivity. CONCLUSIONS: Patients need to feel well informed and find an added value in using wearables. Wearables need to be user-friendly, have an attractive design, and show clinical efficacy in improving disease management. Variations in perceptions regarding integrity, benefits, and effectiveness of monitoring indicate possible conflicts of expectations among participants. The engagement of end users, patients, and health professionals, in the design and implementation process, is crucial for the development of wearable devices that enhance and facilitate neurological rehabilitation practice.

A prospective study of quality of life in amyotrophic lateral sclerosis patients.

OBJECTS: The aim of this prospective and longitudinal study was to describe individual quality of life in patients with amyotrophic lateral sclerosis (ALS) and its correlations with physical function and emotional well-being from diagnosis and over time. MATERIALS AND METHODS: Thirty-six patients were included in the study. Individual quality of life was measured with the Schedule of Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW), illness severity was assessed using the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALS FRS-R), and emotional distress was measured using the Hospital Anxiety and Depression Scale (HADS). Data were collected from diagnosis and thereafter, every six months for a period of two years. Twelve patients completed the 24-month follow-up. RESULTS: Family, friends and own physical health were important for overall quality of life, from diagnosis and during the disease progression. Most patients had good quality of life, which remained stable, despite changed physical functions. Several patients scored above the cut-off score for doubtful and clinical anxiety and depression early on after diagnosis, and there was a significant decrease in anxiety over time. Soon after diagnosis, there was a correlation between depression and quality of life. CONCLUSION: The family, social relations and own physical health are important for overall quality of life in patients with ALS. Thus, supporting the family and facilitating so that patients can continue to stay in contact with friends are important aspects during the disease. Conducting an early screening for depression can be important for preventing decreased quality of life.

Persistent Opacification of the Woven EndoBridge Device: A Conebeam CT Analysis of the Bicêtre Occlusion Scale Score 1 Phenomenon.

BACKGROUND AND PURPOSE: Some Woven EndoBridge devices present a persistent intradevice opacification at imaging follow-up, described as the Bicêtre Occlusion Scale Score 1 (BOSS 1) phenomenon. The clinical implications remain unknown. We aimed here to analyze the factors influencing this occurrence and to precisely describe the evolution of BOSS 1 with time using conebeam CT. MATERIALS AND METHODS: We retrospectively analyzed a prospectively maintained Woven EndoBridge database at our tertiary center and included all patients with isolated BOSS 1 and BOSS 1 associated with small neck remnant (BOSS 1 + 2). RESULTS: Two hundred sixty-seven aneurysms were treated with a Woven EndoBridge device between July 2012 and December 2021. Follow-up with DSA was available for 220 aneurysms (median, 5 months), among which BOSS 1 and 1 + 2 were found in 9.1% (20/220) (95% CI, 5.5%-12.7%). A second DSA follow-up (median, 17 months) was performed in 15 of these 20 aneurysms, which revealed that 40% had evolved to complete Woven EndoBridge occlusion, 33% showed a decreased persistent opacification, and 27% remained stable. BOSS 1 was significantly associated with postoperative antiplatelet medication, a lower aneurysm aspect ratio, and the use of the Woven EndoBridge 17 (P < .05). The average Woven EndoBridge shape modification was less pronounced in the BOSS 1 population (P < .02). None of the BOSS 1 or 1 + 2 aneurysms required retreatment or were associated with hemorrhage occurrence. CONCLUSIONS: Isolated persistent flow inside the Woven EndoBridge device at follow-up is rare and notably associated with antiplatelet prescription. It seems to present a benign course in most cases.

International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia.

BACKGROUND: HHT is an autosomal dominant disease with an estimated prevalence of at least 1/5000 which can frequently be complicated by the presence of clinically significant arteriovenous malformations in the brain, lung, gastrointestinal tract and liver. HHT is under-diagnosed and families may be unaware of the available screening and treatment, leading to unnecessary stroke and life-threatening hemorrhage in children and adults. OBJECTIVE: The goal of this international HHT guidelines process was to develop evidence-informed consensus guidelines regarding the diagnosis of HHT and the prevention of HHT-related complications and treatment of symptomatic disease. METHODS: The overall guidelines process was developed using the AGREE framework, using a systematic search strategy and literature retrieval with incorporation of expert evidence in a structured consensus process where published literature was lacking. The Guidelines Working Group included experts (clinical and genetic) from eleven countries, in all aspects of HHT, guidelines methodologists, health care workers, health care administrators, HHT clinic staff, medical trainees, patient advocacy representatives and patients with HHT. The Working Group determined clinically relevant questions during the pre-conference process. The literature search was conducted using the OVID MEDLINE database, from 1966 to October 2006. The Working Group subsequently convened at the Guidelines Conference to partake in a structured consensus process using the evidence tables generated from the systematic searches. RESULTS: The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.

Impact of A1 Asymmetry on the Woven EndoBridge Device in Anterior Communicating Artery Aneurysms.

BACKGROUND AND PURPOSE: Woven EndoBridge (WEB) devices are increasingly used to treat intracranial aneurysms. A1 asymmetry contributes to anterior communicating artery aneurysm formation and to treatment instability after coiling. We sought to evaluate whether A1 asymmetry had an impact on angiographic outcome in anterior communicating artery aneurysms treated with the WEB. MATERIALS AND METHODS: Anterior communicating artery aneurysms treated between July 2012 and July 2020 with the WEB from an institutional review board-approved database were reviewed. A1 asymmetry was categorized as the following: absence of the A1 segment on 1 side (unilateral A1) versus bilateral A1. Univariate and multivariable analyses assessed independent predictors of adequate (WEB Occlusion Scale A, B, and C) and complete occlusion (WEB Occlusion Scale A and B). RESULTS: Forty-eight individual aneurysms (47 patients) were included in the final analysis, of which 16 (33%) were acutely ruptured. The mean size was 6.5 (SD, 2.2) mm. Adequate and complete occlusion was achieved in 33 (69%) and 30 (63%) cases, respectively. Unilateral A1 was associated with significantly higher rates of adequate (92% versus 60% for bilateral A1; P = .03) and complete occlusion (92% versus 50% for bilateral A1; P < .01). Multivariable logistic regression confirmed unilateral A1 as an independent predictor of both adequate (OR = 10.6; 95% CI, 1.6-220.7; P = .04) and complete occlusion (OR = 9.5, 95% CI, 1.5-190.2; P = .04. A sensitivity analysis comparing unilateral "functional" A1 with bilateral "functional" A1 showed similar results. WEB shape modification was not influenced by the unilateral A1 configuration (P = .70). CONCLUSIONS: Anterior communicating artery aneurysms with a unilateral A1 configuration treated with WEB devices are associated with better angiographic outcome than those with bilateral A1. This finding supports the hypothesis that WEB devices are resistant to unilateral flow in the aneurysm as opposed to coils.

Endovascular Treatment of Small and Very Small Intracranial Aneurysms with the Woven EndoBridge Device.

BACKGROUND AND PURPOSE: The Woven EndoBridge has proved to be a safe and effective treatment, especially for wide-neck intracranial aneurysms. The recent fifth-generation Woven EndoBridge came with smaller devices. The purpose of this study was to assess the safety and efficiency of Woven EndoBridge treatment of small and very small aneurysms. MATERIALS AND METHODS: Between September 2017 and March 2020, all consecutive patients treated with a 3- or 3.5 mm-width Woven EndoBridge device were included in this retrospective intention-to-treat study. Clinical and radiologic findings were evaluated at immediate and last-available follow-up. Angiographic outcome was assessed by an external expert reader. RESULTS: One hundred twenty-eight aneurysms were treated with a fifth-generation Woven EndoBridge device including 29 with a width of ≤3.5 mm. Ten aneurysms were ruptured (34%). In 3 cases (10%), Woven EndoBridge treatment could not be performed because the aneurysm was still too small for the smallest available Woven EndoBridge device and another endovascular strategy was chosen. The median follow-up time was 11.2 months. Complete and adequate occlusion was obtained in 71% and 90% of the treated aneurysms, respectively. Retreatment was needed in 2 cases (10%). Symptomatic ischemic complications leading to transient neurologic deficits occurred in 2 cases (7%) (1 procedure-related and 1 device-related) but with full spontaneous recovery at discharge. CONCLUSIONS: The fifth-generation Woven EndoBridge device seems to be a safe and technically feasible treatment for both ruptured and unruptured small and very small intracranial aneurysms, with satisfactory occlusion rates on midterm follow-up. However, further study is needed to evaluate longer-term efficiency.

MR diffusion tensor imaging and fiber tracking in spinal cord arteriovenous malformations: a preliminary study.

BACKGROUND AND PURPOSE: Diffusion tensor imaging (DTI) of the spinal cord in patients harboring spinal arteriovenous malformations (AVMs) was carried out to evaluate the feasibility of this new technique to determine the displacement of the spinal cord tracts and to correlate morphologic and functional DTI data (fractional anisotropy [FA] and apparent diffusion coefficient [ADC]) with the clinical symptoms. MATERIALS AND METHODS: Nine patients with spinal cord AVMs were investigated at 1.5T using a sagittal spin-echo single-shot echo-planar generalized autocalibrating partially parallel acquisition diffusion-weighted imaging sequence. ADC and FA maps were computed in different regions of interest (both above and below the nidus), and tractography was used to visualize the course of the tracts. The data were correlated with the clinical symptoms and compared with 12 healthy control subjects. RESULTS: At the level of the nidus, tracts were normal, shifted, separated, or interrupted but not intermingled with the nidus. Interruption of the tracts was coherent with the clinical symptoms. In patients with severe neurologic deficits, FA values caudal to the nidus showed a reduced anisotropy consistent with loss of white matter tracts. CONCLUSIONS: We demonstrate that AVMs may interrupt, displace, or separate the fiber tracts and that clinical symptoms may be reflected by the quantitative FA results and the morphologic loss of fibers distant to the lesion. DTI with fiber tracking offers a novel approach to image spinal cord AVMs and may open a window to understand the complex pathophysiology of these lesions.

Diagnosis and endovascular treatment of pediatric spinal arteriovenous shunts.

Spinal arteriovenous shunts (SAVSs) are rarely diagnosed in infants and children, but they are important clinically because morbidity can be significant. Although these lesions do not form a distinct pathologic group separate from the SAVSs seen in older patients, experience with these malformations in the pediatric population has led to the identification of several important features that are characteristic of this group of SAVSs. Association with genetic abnormalities, heritable (hereditary hemorrhagic telangiectasia) and nonheritable somatic (spinal arteriovenous metameric syndrome or Cobb syndrome), is relatively common and likely underrecognized. Male predominance is more pronounced than in the adult population. Hemorrhagic presentation is more frequent than in adults, except in extremely young children. The natural history seems to be better than previously thought, with early rehemorrhage uncommon. Despite early presentation and severe symptoms, these lesions are frequently amenable to endovascular treatment, often with anatomic cure achieved and with improvement or stabilization of symptoms after partial targeted treatment.

Management of spontaneous haemorrhagic intracranial vertebrobasilar dissection: review of 21 consecutive cases.

INTRODUCTION: Haemorrhagic intracranial vertebrobasilar dissection is an uncommon cause of nontraumatic subarachnoid haemorrhage (SAH) and accounts for only 1-10% of non-traumatic SAH. Treatment in the acute phase is considered to be essential because of the high risk of rebleeding and the consequent unfavourable outcome. However, the location, the potential for involvement of eloquent vessels and the histopathological characteristics of the vessel wall make treatment demanding from both a technical and anatomical point of view. We report our experience in the management of this disease. PATIENTS AND TREATMENTS: From 1989 to June 2006, we managed 21 patients with spontaneous haemorrhagic dissection located in the intracranial vertebrobasilar system, 13 patients were treated using an endovascular approach, 1 by surgical clipping, and 7 were managed conservatively. RESULTS: Among the 13 patients treated endovascularly, 7 underwent proximal occlusion, 4 underwent parent artery embolization at the site of dissection, and 2 underwent endovascular trapping. Severe, treatment-related complications due to dislodgement of the thrombus during the procedure occurred in 1 patient, who then died from brainstem ischaemia. One patient died from severe pneumonia and one patient was left disabled from vasospastic ischaemia resulting from severe initial SAH. The remaining 10 patients had satisfactory outcomes: none rebled after treatment and when discharged they had Karnovsky scores of 80-100. Of the 7 conservatively treated patients, three died of rebleeding and four were discharged with Karnovsky scores of 50-100. One patient, who was treated surgically, was discharged with a Karnovsky of 90. CONCLUSION: The high rate of rebleeding and consequent mortality among the patients treated conservatively argues for treatment in the acute phase. Treatment should be guided by each patient's angiomorphology, clinical condition and the experience of the neurosurgical/neuroradiological team. Options include endovascular or surgical trapping of the dissection and proximal occlusion and embolisation of the parent artery at the site of the dissection.

Segmental unfused basilar artery with kissing aneurysms: report of three cases and literature review.

Basilar artery "fenestration" is the result of a failed fusion of the bilateral longitudinal neural arteries and can be associated with a saccular aneurysm, which typically arises at the proximal juncture of the unfused segment. "Kissing" aneurysms at this site, i.e. two aneurysms arising from the proximal junction of the unfused segment of the basilar artery pointing anteriorly and posteriorly are reported to be exceedingly rare. We present three patients with this rare condition, all of them being treated by endovascular techniques.

[Pediatric neurovascular malformations: vein of Galen arteriovenous malformations (VGAM), pial arteriovenous malformations (pial AVM), dural sinus malformations (DSM)]. / Pathologie neurovasculaire malformative de l'enfant: malformations anévrismales de la veine de Galien (MAVG), malformations artérioveineuses piales (MAVP), malformations des sinus duraux (MSD).

The purpose of this article is to give an overview of the cerebral and spinal cord pediatric malformations; we particularly describe three of them: Vein of Galen arteriovenous Malformation, Pial Arteriovenous Malformation, and Dural Sinus Malformation. We report the experience of Bicêtre since 1981 to 2003, with 317 VGAM, 302 Pial AVM and 30 DSM. We describe natural history, clinical and imaging features, endovascular management and the clinical and morphologic results. The clinical consequences of the VGAM and Pial AVM are systemic or cerebral. When they are cerebral they are mainly from hydrovenous type, as melting brain, or chronic hydrocephalus with calcifications and seizures. Depending of their angioarchitecture and amount of arteriovenous shunt, their gravity and the time of revealing are variable. Pial AVM, they carry the risk of hemorrhage, unlike VGAM. The presence of a fistula type should lead to suspect hereditary hemorrhagic telangiectasia. The main risk of DSM is hemorrhage due to thrombosis of the malformation, more than hydrovenous disorders. In VGAM, we obtain the complete cure of the shunt in 55% of case, and reduction of more than half of the shunt in 93.8%. Seventy-four percent of treated cases had a normal neurologic examination. DSM have a worse prognostic, and mortality is 38% despite treatment.

Role of preoperative embolization of intramedullary hemangioblastoma.

OBJECT: Hemangiobastomas (HB) are rare lesions accounting for 1 to 5% of all spinal cord tumors. Due to their hypervascular nature, an angiography may be proposed preoperatively in order to identify tumoral vascular anatomy. Preoperative embolization may be indicated to reduce intraoperative bleeding, thus facilitating tumor resection and minimizing surgical risk. The aim of this paper is to report our experience of preoperative embolization in intramedullary hemangioblastomas. METHODS: We performed a retrospective analysis of all patients operated on for intramedullary hemangioblastomas between 1995 and 2014 who had undergone embolization before surgery. RESULTS: Seven patients were analyzed: there were 6 females and 1 male, mean age 43years, 6 patients had Von Hippel-Lindau disease. Four tumors were located in the cervical spine and three in the dorsal spine. The average maximum sagittal diameter was 19mm (range 8-32mm), while the average maximum axial diameter was 11.5mm (range 6-21mm). The embolic agent used was Histoacryl (NBCA). Endovascular embolization was routinely performed the day before surgery. One patient experienced a major preoperative complication with a vertebrobasilar infarctus with consequent unilateral cerebellar syndrome and gait instability. Minor extravasation of embolic agent was observed in two cases. In one of these two cases, there was also the penetration of the embolic agent in the tumor; the resection was impossible due to the hard consistency of the tumor. In the other 6 patients, the resection was total. Six patients had identical preoperative and postoperative McCormick score and one patient shifted to a better score at follow-up. CONCLUSION: Preoperative endovascular embolization is an effective adjunct treatment. It is useful in reducing the surgical bleeding and thus the operative risks. The procedure is not always safe and complications could occur. We recommend preoperative embolization in selected cases.

Communication abilities and Rett syndrome.

Investigated 6 girls with Rett syndrome (RS), ages between 2 and 13 years, to provide comprehensive descriptions of their communicative behaviors. Previous studies have not focused on communicative competence nor on the intentionality of the language used by children with RS. This study concluded that all members of the group investigated were at a preintentional level of communication. Intentional communication has previously been reported to develop in normally developing and intellectually disabled children attaining Piagetian Sensorimotor Stage V in Means-End behavior. The present study also investigated the cognitive performance of the group. We concluded that the preintentional level of communication noted was consistent with the subjects' profound intellectual disability. No Means-End (i.e., purposeful) behavior beyond Piagetian Sensorimotor Stage III could be elicited from the 6 girls. The relevance of this study for therapeutic intervention is discussed.

Speech and language skills in children with early treated phenylketonuria.

The speech and language skills of 29 children with early treated phenylketonuria (PKU) were reported. The performances of two experimental groups that differed in language development level on standardized speech/language tests were compared with the performances of appropriate control groups matched for age, sex, and socioeconomic level. No significant differences were found on speech and/or language measures between either of the two experimental groups and their respective controls. Examination of individual scores, however, did reveal linguistic impairment in a small number of persons with PKU. No pattern of linguistic deficit was found nor were there any consistent relations between individual speech/language scores, cognitive abilities, or dietary control ratings.

Acute lymphoblastic leukaemia: language deficits in children post-treatment.

The language abilities of a group of 22 children (aged between 5 years and 17 years, 9 months) treated for acute lymphoblastic leukaemia (ALL) were investigated and compared with those of a group of non-neurologically impaired, age- and sex-matched controls. The language test battery included an age-appropriate measure from the Test of Language Development (TOLD) series, consisting of either the Test of Adolescent Language (TOAL--2), or the Test of Language Development--Intermediate or --Primary (TOLD--I or --P); the timed subtests of the Clinical Evaluation of Language Function (CELF); the Boston Naming Test; and one of either the Token Test or the Token Test for Children, depending on the age of the subject. As a group, the leukaemia subjects performed significantly worse than the controls on the TOAL--2, TOLD--I, TOLD--P, Token Test, and Boston Naming Test. The need for post-treatment monitoring of the language abilities of children treated for ALL to enable early remediation of areas of deficit is highlighted.

[MRI and in utero ventriculomegaly]. / IRM et ventriculomégalie in utero.

Ventriculomegaly constitutes the major indication of fetal brain MRI. MRI is therefore of utmost importance to look for a cause through the depiction of criteria of malformations and through the definition of criteria of destructive lesions. Malformations and destructive lesions are the most common causes of ventricular dilatation. Some challenging points are worth mentioning in term of mechanism with the challenge of hydrocephalus (in term of increased in intracranial pressure) and of isolated ventriculomegaly. The image itself is also challenging since a similar image may be of different origin. In term of natural history of fetal brain injury an irregular, nodular aspect of the ventricular wall and/or the germinal matrix is often the only pathologic MRI finding that is known to be of clastic origin. In term of prognosis the challenge is represented by the isolated mild ventriculomegaly, the literature being quite confusing. The purpose of this review paper is to highlight the underlying mechanisms and pathophysiology of ventricular dilatation based on results from the literature as well as from personal experience.

Risk factors of hematomyelia recurrence and clinical outcome in children with intradural spinal cord arteriovenous malformations.

BACKGROUND AND PURPOSE: Few published data are available concerning the risk of re-bleeding of spinal cord AVM after an hematomyelia and concerning the long-term clinical outcome. Our aim was to assess the risk of recurrence and long-term clinical outcome after hematomyelia in children with spinal cord AVMs. MATERIALS AND METHODS: This single-center retrospective study reviewed the clinical and radiologic data of 28 children younger than 18 years of age with arteriovenous malformation who had experienced at least 1 episode of hematomyelia between 1988 and 2012. Long-term clinical outcome was assessed by the American Spinal Injury Association Impairment Scale, and radiologic review included MR imaging and angioarchitecture on angiography (blinded to clinical information) before treatment and at recurrence. RESULTS: Sixteen children (57%) experienced 1 episode of hematomyelia, while 12 children (43%) experienced recurrence. Girls and boys were equally affected (sex ratio, 1:1), and mean clinical follow-up was 5.7 ± 4.4 years. The risk of recurrence was higher for AVMs of the cervical and upper thoracic spine, 12 (100%) versus 11 (69%) (P = .01). A high American Spinal Injury Association scale score at last follow-up was reported for 11 children (39%), and the risk of recurrence tended to be associated with poorer functional prognosis (7 [64%] versus 5 [29%], P = .07). At the time of recurrence, perimedullary venous drainage was the main factor associated with recurrence (P = .002). Occlusion rate ≥50% was associated with a decreased risk of recurrence (P = .047). CONCLUSIONS: In the present series, cervical and upper thoracic spinal cord AVMs and microarchitecture were predictive of the risk of hematomyelia recurrence. Perimedullary venous drainage was one of the main parameters associated with recurrence. Functional prognosis was better in patients with a single episode of hematomyelia.