Moderate Neurological Impairment Research Articles

Accidental transdermal fentanyl overdose in a child

Fentanyl is a synthetic opioid that provides analgesia for procedures with moderate to severe pain and also can be used to manage acute or chronic pain. Fentanyl is available in several dosing forms and is more widely used as a transdermal patch for the management of chronic pain at home. Accidental exposure to even one dose of fentanyl, especially by children can result in a fatal overdose leading to disturbances of consciousness and/or respiratory depression. A nine-year-old boy was referred to the pediatric emergency department because of disturbed consciousness and respiratory depression. A patch of fentanyl 100 mcg/h was found on the bruised knee during the examination. For treatment, he got naloxone 0.1 mg, infusion therapy and artificial lung ventilation due to respiratory depression. Signs characteristic of acute toxic leukoencephalopathy and hypoxic-ischemic encephalopathy were found on brain magnetic resonance imaging (MRI). After 29 days in the inpatient unit and 120 days of rehabilitation, the patient was discharged home with moderate neurological impairment. Opioid misuse can cause serious life-threatening consequences especially in children resulting in death or severe neurological impairment. When dealing with cases of fentanyl overdose, doctors should keep in mind not only respiratory depression and hypotension, but the presence of toxic leukoencephalopathy as well. These cases may be preventable through better education of prescribers and patients.

The Quality of Life of the Patients with Thoracic Ossification of the Ligamentum Flavum after Surgery Using EQ-5D-5L.

There is a lack of studies on the quality of life (QoL) after posterior laminectomy in patients with thoracic ossification of the ligamentum flavum (TOLF), and risk factors associated with poor prognosis remain controversial. Therefore, the present study was conducted to illustrate the QoL for TOLF patients after surgery and determine its relationship with their demographic, surgery-related, clinical characteristics and imaging data. One hundred and eighteen patients diagnosed with thoracic myelopathy because of TOLF were enrolled in this retrospective study. They all underwent posterior decompressive laminectomy from August 2010 to January 2022. The QoL was evaluated based on the EQ-5D-5L. Collecting gender, age, number of operations, compressed segments, Frankel grade, complications, body mass index (BMI), symptoms and duration, modified Japanese Orthopaedic Association (mJOA) score (preoperative), follow-up time and type of ossification, increased signal on Sagittal T2-weighted Images (ISST2I), occupancy rate and analyzing by Mann-Whitney U-test, Kruskal-Wallis test, the χ2 -test, and logistic regression tests. Average follow-up 70.8 months (18-149), the mean age was 59.74 ± 9.81 years and the mean score for the QoL based on the EQ-5D-5 L and visual analogue scale (VAS) score were 0.71 ± 0.28 and 78.88 ± 10.21 at the final follow-up. Moderate and severe problems were found in the pain/discomfort in 22.0% of the patients. These mobility and usual activities numbers were slightly higher (24.6% and 30.4%, respectively). The mean scores for QoL and VAS were significantly higher in patients with mild and moderate neurological impairment, normal BMI, <60 years of age, no dural tears, symptom relief at hospital discharge, unilateral and bilateral ossification on axial CT scan, ≤ 50% spinal canal occupancy on CT and MRI, and none or fuzzy on ISST2I. QoL after posterior laminectomy in TOLF patients is generally satisfactory compared to preoperative levels. Preoperative mJOA score, Age, comorbid diabetes, the major symptom is activity limitation, BMI, ISST2I, Intraoperative dural tears and spinal canal occupancy rate correlate significantly with the corresponding dimensions and are predictive. Age, spinal canal occupancy rate, ISST2I, preoperative mJOA score, BMI are significantly associated with and have predictive value for overall postoperative QoL.

Developmental outcomes for survivors of placental laser photocoagulation for the management of twin-to-twin transfusion syndrome

BackgroundFetoscopic laser coagulation of placental anastomoses reverses the pathological process in twin-to-twin transfusion syndrome, thereby increasing survival, but there are a paucity of studies addressing long-term neurodevelopmental outcome of survivors. This study aimed to ascertain the presence of neurodevelopmental disabilities in child survivors of monochorionic pregnancies managed by placental laser photocoagulation in the Australian state of Victoria.MethodsAll pregnancies undergoing placental laser photocoagulation with the Victorian Fetal Therapy Service between 2006–2017 were included. Information on each surviving child, including demographics, perinatal course, and developmental progress was collected from parents, and consent was sought to complete the Child Behaviour Checklist. Interviewers evaluated whether this information was consistent with a diagnosis of any of 14 neurodevelopmental conditions. A three-tiered outcome measure was allocated for each child: (1) unimpaired or developmentally normal, (2) mild or moderate neurological impairment, or (3) severe neurological impairment. Clinical predictors for adverse outcome were identified.ResultsOf 116 pregnancies (113 twin, 3 triplet), 96 (83%) resulted in 1 + surviving fetuses. 57/113 (50%) twin pregnancies resulted in 2 survivors, 36 (32%) in 1 survivor, and 20 (18%) in no survivors. Of the 235 fetuses, 154 (65.5%) survived to follow-up. Survival increased from 59% in 2006–2008 to 73% in 2015–2017. 90/154 (58%) survivors were followed up at a mean age of 7.5 [SD 3.0] years. Based on parental interview and Child Behaviour Checklist data, 28/90 (31%) participants were assessed as having neurodevelopmental impairment, 27 of mild-moderate severity and 1 severe. Speech/language disorders, attention deficit (hyperactivity) disorders, and fine motor impairment were most common. Neonatal length of stay conferred the highest risk of impairment.ConclusionSubstantial variation exists between fetal therapy services in the type and length of neonatal follow-up following fetoscopic laser coagulation, contributing to a lack of data on long-term outcomes. The findings from this study support increasingly urgent calls to undertake systematic and sustained follow-up of fetoscopic laser coagulation survivors until school age. Information from this study may assist parents in their decision-making when offered fetal surgery. Importantly, it highlights a group for targeted surveillance and early intervention.

Clinical and molecular heterogeneity of syndromic hypothalamic hamartoma.

Two children are presented who have a distinct syndrome of multiple buccolingual frenula, a stiff and short fifth finger with small nails, a hypothalamic hamartoma, mild to moderate neurological impairment, and mild endocrinological symptoms. No variant assessed to be pathogenic or likely pathogenic was detected in the GLI3 gene in either child. This syndrome appears to be distinct from the inherited Pallister-Hall syndrome associated with GLI3 variants, which is characterized by hypothalamic hamartoma, mesoaxial polydactyly, and other anomalies. In the individuals described here, manifestations outside of the central nervous system were milder and the mesoaxial polydactyly, which is common in individuals with Pallister-Hall syndrome, was absent. Instead, these children had multiple buccolingual frenula together with the unusual appearance of the fifth digit. It remains unclear whether these two individuals represent a separate nosologic entity or if they represent a milder manifestation of one of the more severe syndromes associated with a hypothalamic hamartoma.

The relationship between neurological function trajectory, assessed by repeated NIHSS measurement, and long-term cardiovascular events, recurrent stroke, and mortality after ischemic stroke.

Clinically significant changes in neurological deficits frequently occur after stroke onset, reflecting further neurological injury or neurological improvement. However, the National Institutes of Health Stroke Scale (NIHSS) score is only evaluated once in most studies, usually at stroke onset. Utilizing repeated measures of NIHSS scores to identify different trajectories of neurological function may be more informative and provide more useful predictive information. We determined the association of neurological function trajectories with long-term clinical outcomes after ischemic stroke. A total of 4025 participants with ischemic stroke from the China Antihypertensive Trial in Acute Ischemic Stroke were included. Patients were recruited from 26 hospitals across China between August 2009 and May 2013. A group-based trajectory model was used to identify distinct neurological function trajectories, as measured by NIHSS at admission, 14 days or hospital discharge, and 3 months. Study outcomes were cardiovascular events, recurrent stroke, and all-cause mortality during 3-24 months after ischemic stroke onset. Cox proportional hazards models were used to examine the associations of neurological function trajectories with outcomes. We identified three distinct subgroups of NIHSS trajectories: persistent severe (persistent high NIHSS scores during the 3-month follow-up), moderate (NIHSS scores started at around 5 and gradually reduced), and mild (NIHSS scores always below 2). The three trajectory groups had different clinical profiles and different risk of stroke outcomes at 24-month follow-up. Compared to the mild trajectory group, patients in the persistent severe trajectory group had a higher risk of cardiovascular events (multivariable-adjusted hazard ratios (95% confidence intervals) = 1.77 (1.10-2.86)), recurrent stroke (1.82 (1.10-3.00)), and all-cause mortality (5.64 (3.37-9.43)). Those with moderate trajectory had an intermediate risk: 1.45 (1.03-2.04) for cardiovascular events and 1.52 (1.06-2.19) for recurrent stroke. Longitudinal neurological function trajectories derived from repeated NIHSS measurements during the first 3 months after stroke provide additional predictive information and are associated with long-term clinical outcomes. The trajectories characterized by persistent severe and moderate neurological impairment were associated with increased risk of subsequent cardiovascular events.

Flow augmentation STA-MCA bypass evaluation for patients with acute stroke and unilateral large vessel occlusion: a proposal for an urgent bypass flowchart.

Endovascular recanalization trials have shown a positive impact on the preservation of ischemic penumbra in patients with acute large vessel occlusion (LVO). The concept of penumbra salvation can be extended to surgical revascularization with bypass in highly selected patients. For selecting these patients, the authors propose a flowchart based on multimodal MRI. All patients with acute stroke and persisting internal carotid artery (ICA) or M1 occlusion after intravenous lysis or mechanical thrombectomy undergo advanced neuroimaging in a time window of 72 hours after stroke onset including perfusion MRI, blood oxygenation level-dependent functional MRI to evaluate cerebrovascular reactivity (BOLD-CVR), and noninvasive optimal vessel analysis (NOVA) quantitative MRA to assess collateral circulation. Symptomatic patients exhibiting persistent hemodynamic impairment and insufficient collateral circulation could benefit from bypass surgery. According to the flowchart, a bypass is considered for patients 1) with low or moderate neurological impairment (National Institutes of Health Stroke Scale score 1-15, modified Rankin Scale score ≤ 3), 2) without large or malignant stroke, 3) without intracranial hemorrhage, 4) with MR perfusion/diffusion mismatch > 120%, 5) with paradoxical BOLD-CVR in the occluded vascular territory, and 6) with insufficient collateral circulation. The proposed flowchart is based on the patient's clinical condition and multimodal MR neuroimaging and aims to select patients with acute stroke due to LVO and persistent inadequate collateral flow, who could benefit from urgent bypass.

Effectiveness of robotic assisted laparoscopic nissen fundoplication in neurologically impaired children with severe gastroesophageal reflux disease

Laparoscopic Nissen Fundoplication is still the most widely used surgical technique in the treatment of gastroesophageal reflux disease (GERD) in children. However, there are some technical and anatomical difficulties in the treatment of reflux in NIC with GERD. All these difficulties lead to the high rate of surgical failure observed in neurological impaired children (NIC). Robotic surgery provides several advantages in overcoming these difficulties. This study is the first study to evaluate the effectiveness of robotic surgery in the treatment of GERD in children with severe or moderate neurological impairments reported in Turkey. This study took place between January 2018 and February 2020. We retrospectively evaluated the records of eleven children with severe or moderate neurological problems who were treated using the robotically assisted laparoscopic Nissen fundoplication (RALNF) technique for demographic data, anesthesia time, pre-console time, console time, and postoperative complications. Nine of the patients (81.8%) had serious and two had moderate neurological problems. All of the patients applied to the emergency department at different times due to recurrent lung infections. While the first RALNF console time was 240 minutes, this study revealed that this time decreased to 45 minutes. None of the patients had complications related to the surgical procedure, but four patients required postoperative intensive care unit up to two months due to several problems related to NIC. RALNF can be safely applied to pediatric patients with GERD with severe and moderate NIC.

A Case Report of Acute Necrotizing Encephalitis

Acute necrotizing encephalitis (ANEC) is a rare entity seen primarily in East Asian infants and previously healthy children. A 5-year-old boy complained of fever and seizures, which developed into status epilepticus. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) brains showed acute necrotizing encephalitis features. Empirical treatment for meningoencephalitis with supportive therapy was administered. MRI was then repeated 25 days post-therapy, which showed the previously seen abnormal signal intensities resolution. The patient was subsequently discharged home with moderate neurological impairment. Although ANEC is a rare disease, a typical clinical scenario and MRI findings should prompt recognition of the disease, essential for treatment.

Atherothrombotic stroke in women: blood thrombogenicity and the vascular wall

Introduction. Haemostasis and functional changes of the vascular wall are the strategic and actively developing aspects in the study of ischaemic stroke pathogenesis. The aim of current work was to examine the relationship between hormonal status, thrombogenic blood factors, and the structural and functional changes in the vascular wall in women after atherothrombotic stroke in the internal carotid territory. Materials and methods . Twenty-nine women (mean age 55 years) in the first 3 months after atherothrombotic stroke, diagnosed using the international TOAST criteria, were included in the study. The severity of neurological impairment was evaluated using the NIH Stroke Scale. The follicular stimulating hormone (FSH) and homocysteine levels, along with haemostasis parameters and rheological properties of red blood cells were examined. Ultrasound of the major arteries of the head, assessment of the elastic properties and mural shear stress in the common carotid artery, as well as evaluation of the vasomotor function of the brachial artery endothelium were conducted. Results. Clinical differences in the atherosclerotic stroke were found in women depending on their FSH levels: moderate and severe neurological impairments were noted when the FSG level was ³30 mIU/mL, while mild impairments were present when the FSG level was <30 mIU/mL. It was found that a high FSH level in women is associated with significant arterial hypertension, obesity and type 2 diabetes mellitus. The group of women with high FSH levels had elevated fibrinogen, homocysteine, von Willebrand factor, erythrocyte aggregation amplitude, along with the accelerated formation of 3D aggregates and increased erythrocyte aggregate strength. In women with insufficient estrogen production in the ovaries (FSH ³30 mIU/mL), the stiffness index of the common carotid artery wall was significantly higher, while the mural shear stress and endothelial vasomotor function were lower than in the group of women with preserved ovarian function. Conclusions. A link between menopause, increased blood thrombogenicity, and structural and functional changes in the vascular wall (increased stiffness, reduced mural shear stress and endothelial vasomotor function) was found in women after atherothrombotic stroke.

How does the ball influence the performance of change of direction and sprint tests in para-footballers with brain impairments? Implications for evidence-based classification in CP-Football

The aims of this study were: i) to analyze the reliability and validity of three tests that require sprinting (10 m, 25 m, 40 m), accelerations/decelerations (Stop and Go Test) and change of direction (Illinois Agility Test), with and without ball, in para-footballers with neurological impairments, and ii) to compare the performance in the tests when ball dribbling is required and to explore the practical implications for evidence-based classification in cerebral palsy (CP)-Football. Eighty-two international para-footballers (25.2 ± 6.8 years; 68.7 ± 8.3 kg; 175.3 ± 7.4 cm; 22.5 ± 2.7 kg·m-2), classified according to the International Federation of Cerebral Palsy Football (IFCPF) Classification Rules (classes FT5-FT8), participated in the study. A group of 31 players without CP was also included in the study as a control group. The para-footballers showed good reliability scores in all tests, with and without ball (ICC = 0.53–0.95, SEM = 2.5–9.8%). Nevertheless, the inclusion of the ball influenced testing reproducibility. The low or moderate relationships shown among sprint, acceleration/deceleration and change of direction tests with and without ball also evidenced that they measure different capabilities. Significant differences and large effect sizes (0.53 < ηp2 < 0.97; p < 0.05) were found when para-footballers performed the tests with and without dribbling the ball. Players with moderate neurological impairments (i.e. FT5, FT6, and FT7) had higher coefficients of variation in the trial requiring ball dribbling. For all the tests, we also obtained between-group (FT5-FT8) statistical and large practical differences (ηp2 = 0.35–0.62, large; p < 0.01). The proposed sprint, acceleration/deceleration and change of direction tests with and without ball may be applicable for classification purposes, that is, evaluation of activity limitation from neurological impairments, or decision-making between current CP-Football classes.

The Subclinical Cardiomyopathy of Friedreich's Ataxia in a Pediatric Population

BackgroundIdentification of a subclinical cardiomyopathy in pediatric patients with Friedreich's ataxia (FA) has not been well-described. MethodsWe performed echocardiography (Echo), cardiac magnetic resonance imaging (cMRI), and neurologic assessment in a cross-sectional analysis of 48 genetically confirmed FA subjects aged 9–17 years with moderate neurologic impairment but without a cardiovascular history. Echo- and cMRI-determined left ventricular mass were indexed (LVMI) to height in grams/m2.7. LV remodeling was categorized as concentric remodeling (CR), concentric hypertrophy (CH), or eccentric hypertrophy based upon Echo- determined relative LV wall thickness. ResultsEcho LVMI exceeded age-based normal values in 85% of subjects, and cMRI-determined LVMI correlated with depression of both diastolic and systolic tissue Doppler velocity (E′: r = –0.65, P < .001, S′: r = –0.46, P < .001) as well as increased early diastolic Doppler flow velocity/tissue velocity ratio (r= 0.55, P < .001), a marker of elevated LV filling pressure. Similar associations were found with echo-determined LV mass. Evidence of depressed LV relaxation and increased LV stiffness were observed in 88% and 71%, of subjects, respectively, despite a normal LV ejection fraction in almost all cases (mean = 60% + 7%). CR and CH were present in 40% and 44% of the study group, respectively, although significant depressions of E′ and S′ were observed only in subjects with CH (P < .005). ConclusionsA subclinical hypertrophic cardiomyopathy is common in pediatric FA patients and CH is associated with both diastolic and systolic dysfunction.

Spinal epidural hematomas: personal experience and literature review of more than 1000 cases.

OBJECTIVE The goal of this study was to identify factors that contribute to the formation of acute spinal epidural hematoma (SEH) by correlating etiology, age, site, clinical status, and treatment with immediate results and long-term outcomes. METHODS The authors reviewed their series of 15 patients who had been treated for SEH between 1996 and 2012. In addition, the authors reviewed the relevant international literature from 1869 (when SEH was first described) to 2012, collecting a total of 1010 cases. Statistical analysis was performed in 959 (95%) cases that were considered valid for assessing the incidence of age, sex, site, and clinical status at admission, correlating each of these parameters with the treatment results. Statistical analysis was also performed in 720 (71.3%) cases to study the incidence of etiological factors that favor SEH formation: coagulopathy, trauma, spinal puncture, pregnancy, and multifactorial disorders. The clinical status at admission and long-term outcome were studied for each group. Clinical status was assessed using the Neuro-Grade (NG) scale. RESULTS The mean patient age was 47.97 years (range 0-91 years), and a significant proportion of patients were male (60%, p < 0.001). A bimodal distribution has been reported for age at onset with peaks in the 2nd and 6th decades of life. The cause of the SEH was not reported in 42% of cases. The etiology concerned mainly iatrogenic factors (18%), such as coagulopathy or spinal puncture, rather than noniatrogenic factors (29%), such as genetic or metabolic coagulopathy, trauma, and pregnancy. The etiology was multifactorial in 11.1% of cases. The most common sites for SEH were C-6 (n = 293, 31%) and T-12 (n = 208, 22%), with maximum extension of 6 vertebral bodies in 720 cases (75%). At admission, 806 (84%) cases had moderate neurological impairment (NG 2 or 3), and only lumbar hematoma was associated with a good initial clinical neurological status (NG 0 or 1). Surgery was performed in 767 (80%) cases. Mortality was greater in patients older than 40 years of age (9%; p < 0.01). Sex did not influence any of these data (p > 0.05). CONCLUSIONS Factors that contribute to the formation of acute SEH are iatrogenic, not iatrogenic, or multifactorial. The treatment of choice is surgery, and the results of treatment are influenced by the patient's clinical and neurological status at admission, age, and the craniocaudal site.

Clinical recovery and health-related quality of life in ischaemic stroke survivors receiving thrombolytic treatment: a 1-year follow-up study.

The aims of this study were to examine prospectively the course of neurological and functional recovery and health related quality of life (HRQoL) in ischaemic stroke patients after intravenous thrombolysis, to assess the 1-year outcome and to determine the early predictors. A group of 53 consecutive patients were assessed at admission to the acute stroke unit (T0), before their discharge (T1), and at 3 (T2) and 12 (T3) months after hospital discharge. The National Institutes of Health Stroke Scale (NIHSS), the Barthel Index (BI) and the Stroke Specific Quality of Life Scale (SSQoL) were used to evaluate stroke severity, functional disability and health related quality of life (HRQoL). A good outcome was defined as simultaneous improvement in all three scales. At T0, 83 % of the patients had severe or moderate neurological impairment. Additionally, 77 % demonstrated very severe or severe disability when assessed by the BI. 74 and 79 % of the patients reported an acceptable HRQoL at 3 and 12months following discharge, respectively. Neurological and functional status improved significantly over time with most changes occurring during hospitalization and the subsequent 3months. At T3, 75.5 % of the patients had the good outcome which had been independently predicted by the level of stroke related disability at T0. A majority of the stroke survivors treated with thrombolysis achieved a good outcome. The measurement of stroke specific HRQoL, as a tool for gathering information from the patient, should be incorporated in the assessment of post-stroke recovery and outcome.

Utility of peritoneal dialysis in neonates affected by inborn errors of metabolism

Some inborn errors of metabolism induce metabolic encephalopathy through accumulation of neurotoxic metabolites. Rapid elimination of these metabolites by peritoneal or extracorporeal dialysis is crucial to prevent neuronal damage or death. In this retrospective study, we evaluated the outcomes of nine neonates with metabolic crisis treated with peritoneal dialysis. Six neonates with hyperammonemic coma (four with organic acidemias, two with urea cycle disorders) and three with leucine accumulation due to maple syrup urine disease (MSUD) were managed with peritoneal dialysis in conjunction with dietary and pharmacological therapy. Three patients with organic acidemia survived. One of the patients was normal; others had moderate and severe neurological impairments. One neonate with organic acidemia and both neonates with urea cycle disorders died. Two of the three patients with MSUD survived without neurological impairment; the other had severe neurological damage and died at 9 months of age due to sepsis. Theoretically, extracorporeal dialysis should be the first dialysis treatment of choice; however, this report demonstrates that peritoneal dialysis has a chance to prevent neurological damage in some patients. Therefore, in developing countries without extracorporeal dialysis opportunities, it can be still a life-saving procedure, if it is applied with skilled staff and standard procedures.

Long-term outcomes after liver transplantation for deoxyguanosine kinase deficiency: A single-center experience and a review of the literature

Deoxyguanosine kinase (DGUOK) deficiency is a well-known cause of hepatocerebral mitochondrial DNA depletion syndromes, which include a broad spectrum of clinical presentations. Affected patients often develop life-threatening liver failure, but the benefits of liver transplantation (LT) are controversial because of the frequently severe neurological involvement due to the underlying mitochondrial disease. We describe the long-term clinical course of 2 patients from our institution and provide an update on their outcomes after LT with this condition. Another 12 pediatric patients were identified through a systematic search of the literature. All 14 reported patients underwent transplantation in infancy despite mild to moderate neurological impairment in some cases. The 2 DGUOK-deficient patients from our center displayed liver failure and mild to moderate neurological involvement. At the time of this writing, they had been followed for 5 and 8 years after LT, both patients were alive, and they had only mild neurological symptoms. Three of the 12 patients identified through the literature review survived for a long time (17, 12, and 23 years); 8 died during early follow-up; and for 1 patient, no follow-up information was available. The 1-year survival rate was 64%; 36% survived for more than 5 years. The long-term survivors had good quality of life. In conclusion, although survival after LT for DGUOK deficiency is lower than survival after LT for other indications, a significant proportion of patients benefit from LT with long-term survival and a stable neurological situation despite initial neurological abnormalities. Nevertheless, a decision to carry out LT for patients with DGUOK deficiency remains difficult because neurological symptoms may occur and worsen after LT despite their absence before transplantation.

Acute tension pneumocephalus secondary to whole spine pneumorrhachis as an unusual presentation of a colon cancer complicated by a transsacral cerebrospinal fluid leak

A 52-year-old woman who was having chemotherapy for treatment of an adenocarcinoma of the colon, was admitted to the emergency department because of a moderate neurological impairment. Head CT scan showed bifrontal pneumocephalus without fractures or discontinuities of the skull base. A few hours later, following the patient’s neurologic deterioration, a new CT scan showed tension pneumocephalus with air diffusion throughout the posterior cranial fossa and cervical spine. Because of air bubbles into the cervical spine, an MRI of the entire spinal canal was done. This exam revealed a whole spine pneumorrhachis along with a transforaminal air passage through the first right sacral foramen and a pyogenic collection anterior to sacral bone. An abdomen CT scan showed a massive relapse of the colon cancer and confirmed a hypodense collection contiguous to the anterior sacral surface, causing erosion of the sacral bone and dural layer with air penetration into the spinal canal. Neurosurgical treatment by a lumbosacral laminectomy and duraplasty was followed by tumour removal and omental covering of the pelvis. Her neurological symptoms were resolved completely. One month later, the patient began adjuvant chemotherapy.

Interdisciplinary team approach for complicated type B aortic dissection with concomitant hematothorax by endovascular stent grafting and left side mini thoracotomy: a case report

Due to high mortality rates in surgical treatment, total endovascular stent grafting has become a promising therapeutic option in patients with acute aortic dissection type B. In our case, a 76- year- old patient with acute ruptured aortic dissection type B and hematothorax achieved concomitant total endovascular stent grafting and left side mini thoracotomy. With moderate neurologic impairment he was discharged from hospital after 20 days.This case shows that early mortality of live threatening acute aortic dissection type B with hemorrhagic pleural effusion may be reduced by total endovascular stent grafting and concomitant mini thoracotomy.

Surgical treatment of symptomatic cerebral cavernous malformations in eloquent brain regions

Despite the increased risk of hemorrhage and deteriorating neurological function of once-bled cerebral cavernous malformations (CM), the management of eloquently located CMs remains controversial. All eloquently located CMs (n = 45) surgically treated between 03/2006 and 04/2011 in our department were consecutively evaluated. Eloquence was characterized according to Spetzler and Martin's definition. The following locations were approached: brainstem, n = 16; sensorimotor, n = 8; visual pathway, n = 7; cerebellum (deep nuclei and peduncles), n = 7; basal ganglia, n = 4, and language, n = 3. Follow-up data was available for 41 patients (91%) with a median interval of 14months. Outcomes were evaluated according to the Glasgow outcome and the modified Rankin scale. Immediately after surgery, 47% (n = 21) had a new deficit. At follow-up, 80% (n = 36) recovered to at least preoperative status or were better than before surgery, 9% (n = 4) exhibited a slight, and 7% (n = 3) had a moderate neurological impairment. Only two cases (4%) with a new permanent severe deficit were observed, both related to dorsal brainstem surgery. The outcome after the surgery of otherwise located brainstem CMs was as beneficial as that for non-brainstem CMs. Patients with initially poor neurological performance fared worse than oligosymptomatic patients. Despite the high postoperative transient morbidity, the majority improved profoundly during follow-ups. Compared with natural history, surgical treatment should be considered for all eloquent symptomatic CMs. Dorsal brainstem location and poor preoperative neurological status are associated with an increased postoperative morbidity.

Aspartoacylase-LacZ Knockin Mice: An Engineered Model of Canavan Disease

Canavan Disease (CD) is a recessive leukodystrophy caused by loss of function mutations in the gene encoding aspartoacylase (ASPA), an oligodendrocyte-enriched enzyme that hydrolyses N-acetylaspartate (NAA) to acetate and aspartate. The neurological phenotypes of different rodent models of CD vary considerably. Here we report on a novel targeted aspa mouse mutant expressing the bacterial β-Galactosidase (lacZ) gene under the control of the aspa regulatory elements. X-Gal staining in known ASPA expression domains confirms the integrity of the modified locus in heterozygous aspa lacZ-knockin (aspalacZ/+) mice. In addition, abundant ASPA expression was detected in Schwann cells. Homozygous (aspalacZ/lacZ) mutants are ASPA-deficient, show CD-like histopathology and moderate neurological impairment with behavioural deficits that are more pronounced in aspalacZ/lacZ males than females. Non-invasive ultrahigh field proton magnetic resonance spectroscopy revealed increased levels of NAA, myo-inositol and taurine in the aspalacZ/lacZ brain. Spongy degeneration was prominent in hippocampus, thalamus, brain stem, and cerebellum, whereas white matter of optic nerve and corpus callosum was spared. Intracellular vacuolisation in astrocytes coincides with axonal swellings in cerebellum and brain stem of aspalacZ/lacZ mutants indicating that astroglia may act as an osmolyte buffer in the aspa-deficient CNS. In summary, the aspalacZ mouse is an accurate model of CD and an important tool to identify novel aspects of its complex pathology.

Mechanical ventilation for respiratory failure in children with severe neurological impairment: is it futile medical treatment?

To assess outcome for children with severe neurological impairment receiving invasive mechanical ventilation for respiratory failure. Medical charts for all such children treated in our intensive care unit (ICU) between January 2003 and July 2008 were reviewed. Outcomes were compared with those for children with moderate neurological impairment. Twenty-two children with severe neurological impairment were included (nine females, 13 males; median age 7y 10mo; range 4mo-17y). The median duration of mechanical ventilation was 16 days. Six children had an uneventful 1-year survival, the others required reintubation or readmission to the ICU, or died. Eleven children were still alive 1 year after discharge from the ICU. Nine patients died of respiratory failure. None of the children in the severe group died of a heart defect. Eleven children with moderate neurological impairment were included (eight females, three males; median age 1y 1mo, range 4mo-13y). Four children had an uneventful 1-year survival. Eight children were still alive 1 year after discharge from the ICU. Two of the three non-survivors died of their heart defects. Mechanical ventilation for respiratory failure in children with severe neurological impairment is complex and associated with limited survival. However, it cannot be regarded as futile medical treatment. Further studies are urgently needed for the rational guidance of clinical decision-making.