Severity Of Neurological Dysfunction Research Articles

Risk factors for postoperative late deterioration in patients with spinal dural arteriovenous fistulas.

Approximately 86% of patients with spinal dural arteriovenous fistulas (SDVAFs) exhibit clinical improvement after surgery. However, 12%-55.8% of these patients experience late deterioration (LD) after an initial period of improvement. The risk factors for LD remain unclear. The aim of this study was to explore the risk factors for LD in SDVAF patients. The clinical data of patients who were admitted to two tertiary hospitals between June 2014 and May 2022 were reviewed. Patients were divided into two groups: the LD group and the no LD group. The severity of neurological dysfunction (NDF) was evaluated using the Modified Aminoff and Logue Scale. Univariable and multivariable Cox regression analyses were performed. A total of 105 eligible patients were enrolled, with a mean age of 57.55 ± 9.42 years. The LD group comprised 37 individuals, while the no LD group consisted of 68 individuals. According to the univariable analysis, preoperative NDF severity and treatment strategy were associated with the risk of LD. According to the multivariable analysis, patients who underwent microsurgery (MS) had a lower risk of LD than did those who underwent endovascular treatment (EVT; HR 0.197, 95% CI 0.085-0.457), and patients with severe NDF had a higher risk of LD than did those with mild NDF (HR 3.604, 95% CI 1.226-10.588), whereas the risk of LD in patients with moderate NDF was similar to that of patients with mild NDF (HR 1.352, 95% CI 0.519-3.524). EVT and severe preoperative NDF are independent risk factors for LD.

Neuroprotective Effects of Inhaled Xenon for Sedation Compared With Propofol Intravenous Sedation in Severe Ischemic Stroke

ABSTRACTS Ischemic stroke occupies a leading position among the causes of mortality and disability. Long-lasting motor and cognitive impairments, a decrease in the level of consciousness over time aggravate the course of the disease, leading to immobilization syndrome and comorbidity load, which contributes to the development of life-threatening conditions in this category of patients. In this regard, the search for new neuroprotective strategies used at an early stage and capable of minimizing the severe consequences of stroke for the patient in particular and society as a whole seems extremely relevant. The paper presents the effect of inhaled xenon for sedation in patients with severe ischemic stroke on the level of consciousness and severity of neurological disorders, and also shows its effect on S100B protein, a marker for blood brain barrier damage.AIM OF STUDY To evaluate the effect of inhaled xenon for sedation in comparison with propofol intravenous sedation on the dynamics of the level of consciousness, the severity of neurological dysfunction and changes in the concentration of astroglial-derived S100B protein in severe ischemic stroke.MATERIAL AND METHODS The research was conducted on two groups of patients comparable in age, gender, comorbid background, as well as the severity of the underlying disease. In both groups, the condition of patients required the start of mechanical ventilation immediately after admission to the intensive care unit. Group I (control, n=12). After intubation and the start of ventilation, patients were sedated with propofol at a dose of 1-2 mg/ kg / hour for 24–72 hours. Group II (xenon, n=12). After intubation and the start of ventilation, patients underwent xenon inhalation at a concentration of 40 vol. % for the first 6 hours. If it was necessary to continue sedation after the end of xenon inhalation, propofol was used in doses similar to Group I. Neurological status was assessed on days 1, 3 and 8 using the Glasgow Coma Scale (GCS), the Full Outline of UnResponsivness (FOUR) score and the National Institutes of Health Stroke Scale (NIHSS). The critical analysis of the value of S100B as a marker of brain damage was carried out before the start of sedation, on the 3rd and 8th days.RESULTS Xenon inhalation (40 vol. %) in comparison with propofol intravenous sedation significantly increases the level of consciousness in patients with severe ischemic stroke (p=0,026), reduces neurological disorders assessed using NIHSS (p=0,007) on day 7, and also reduces serum S100B levels on day 3 (p<0,05) after ischemic stroke.CONCLUSION Our open randomized clinical trial of xenon inhalation versus propofol intravenous sedation revealed the neuroprotective properties of xenon anesthesia in patients with severe ischemic stroke. Based on the obtained clinical and laboratory data, it can be concluded about the effective implementation of the neuroprotective effects of xenon in the administration scheme used in the research.

Surgical Considerations to Improve Recovery in Acute Spinal Cord Injury.

Acute traumatic spinal cord injury (SCI) can be a devastating and costly event for individuals, their families, and the health system as a whole. Prognosis is heavily dependent on the physical extent of the injury and the severity of neurological dysfunction. If not treated urgently, individuals can suffer exacerbated secondary injury cascades that may increase tissue injury and limit recovery. Initial recognition and rapid treatment of acute SCI are vital to limiting secondary injury, reducing morbidity, and providing the best chance of functional recovery. This article aims to review the pathophysiology of SCI and the most up-to-date management of the acute traumatic SCI, specifically examining the modern approaches to surgical treatments along with the ethical limitations of research in this field.

Enlarged perivascular spaces, neuroinflammation and neurological dysfunction in NMOSD patients.

Background and objectivesCerebrospinal fluid (CSF) and interstitial fluid exchange along a brain-wide network of perivascular spaces (PVS) termed the ‘glymphatic system’. The aquaporin-4 (AQP4) water channels abundantly expressed on astrocytic endfeet play a key role in the CSF circulation in the glymphatic system. Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory demyelinating autoimmune disease of the central nervous system (CNS) featured with a specific autoantibody directed against AQP4 in most of patients. Anti-AQP4 antibodies are likely resulting in the impairment of the brain glymphatic system and the enlargement of PVS in NMOSD patients. In the current study, we aimed to demonstrate the features of EPVS detected by MRI and its association with the CSF anti-AQP4 antibody titer, CNS inflammatory markers, and disease severity in NMOSD patients.MethodsWe conducted a retrospective review of a consecutive cohort of 110 patients with NMOSD who had brain MRI. We assessed the correlation of EPVS with markers of neuroinflammation, blood-brain barrier (BBB) function and severity of neurological dysfunction in patients. We used multivariate logistic regression analysis to determine the independent variables associated with disease severity.ResultsThe median number of total-EPVS was 15.5 (IQR, 11-24.2) in NMOSD patients. The number of total-EPVS was significantly related to EDSS score after correcting for the effects of age and hypertension (r=0.353, p<0.001). The number of total-EPVS was also significantly associated with the titer of CSF anti-AQP4 antibody, the albumin rate (CSF/serum ratios of albumin), the CSF albumin, IgG and IgA levels. Logistic regression analysis showed that total-EPVS and serum albumin level were two independent factors to predict disease severity in NMOSD patients (OR=1.053, p=0.028; OR=0.858, p=0.009 respectively). Furthermore, ROC analysis achieved AUC of 0.736 (0.640-0.831, p<0.001) for total-EPVS to determine severe NMOSD (EDSS 4.5-9.5).DiscussionIn our cohort, we found a relationship between EPVS and neuroinflammation and BBB function in NMOSD. Moreover, EPVS might independently predict neurological dysfunction in patients with NMOSD.

Spinal canal invasion as a predictor of neurological deficit in traumatic vertebral burst fractures

This study correlated the extent of spinal canal compression from retropulsed traumatic burst cervical, thoracic, and lumbar spine fractures with the severity of neurological dysfunction. One hundred and sixty-nine patients with cervical, thoracic, or lumbar sub-axial traumatic burst fractures were seen in an emergency department from 2019 to 2021; 79.3% were men, averaging 37 years of age. The lumbar spine was most frequently involved (42%), followed by the thoracic (36.1%) and cervical (21.9%) levels. The extent of spinal canal compression was quantitated utilizing Hashimoto's method, and correlated with patients' extent of neurological injury based on their American Spinal Injury Association scores. There was a positive correlation between the extent of cervical and thoracic spinal cord compression due to retro pulsed burst fragments and the severity of the patients' neurological deficits, but this was not true for the lumbar spine. The extent of spinal cord compression from retropulsed cervical and thoracic traumatic burst fractures was readily correlated with the severity of patients' neurological dysfunction. However, there was no such correlation between the extent of cauda equina compression from retropulsed lumbar burst fractures and the severity of their cauda equina syndromes.

Management of Acute Traumatic Spinal Cord Injury: A Review of the Literature

Traumatic spinal cord injury (TSCI) is a debilitating disease that poses significant functional and economic burden on both the individual and societal levels. Prognosis is dependent on the extent of the spinal injury and the severity of neurological dysfunction. If not treated rapidly, patients with TSCI can suffer further secondary damage and experience escalating disability and complications. It is important to quickly assess the patient to identify the location and severity of injury to make a decision to pursue a surgical and/or conservative management. However, there are many conditions that factor into the management of TSCI patients, ranging from the initial presentation of the patient to long-term care for optimal recovery. Here, we provide a comprehensive review of the etiologies of spinal cord injury and the complications that may arise, and present an algorithm to aid in the management of TSCI.

The Severity of Neurological Dysfunction in Preschool Children, Secondary to Damage Generated During the Perinatal Period, is Associated With a Pro-Inflammatory Pattern of Serum Molecules.

Disorders in the child’s neurological development caused by perinatal risks can lead to long-term altered neurological signs that begin at an early age and involve persistent functional disorders. Recent data suggest that tissue dysfunction, not just acute damage, may initiate or perpetuate an inflammatory response. The aim of this study was to find out if any neurological dysfunction in preschool children secondary to damage generated during the perinatal period is associated with the magnitude of perinatal risks and long-term modifications in the serum concentrations of inflammatory molecules. The participants, aged 1–4 years, were on neurodevelopmental follow-up and rehabilitation therapy from the first three months of life and had no acute disease data. We classified the children into three groups according to the importance of their perinatal risks: low, medium, and high. The results show that 1) the magnitude of perinatal risks correlated with the severity of neurological dysfunction; 2) the greatest changes in the concentrations of the molecules of the inflammatory process were associated with the most altered neurological signs. This suggests that persistent nervous system dysfunction keeps inflammatory responses active even in the absence of an acute process of infection or damage.

Prevention of thromboembolism in spinal cord injury -S1 guideline

IntroductionTraumatic and non-traumatic spinal cord injury bears a high risk for thromboembolism in the first few months after injury. So far, there is no consented guideline regarding diagnostic and prophylactic measures to prevent thromboembolic events in spinal cord injury. Based on a Pubmed research of related original papers and review articles, international guidelines and a survey conducted in German-speaking spinal cord injury centers about best practice prophylactic procedures at each site, a consensus process was initiated, which included spinal cord medicine experts and representatives from medical societies involved in the comprehensive care of spinal cord injury patients. The recommendations comply with the German S3 practice guidelines on prevention of venous thromboembolism.RecommendationsSpecific clinical or instrument-based screening methods are not recommended in asymptomatic SCI patients. Based on the severity of neurological dysfunction (motor completeness, ambulatory function) low dose low molecular weight heparins are recommended to be administered up to 24 weeks after injury. Besides, mechanical methods (compression stockings, intermittent pneumatic compression) can be applied. In chronic SCI patients admitted to the hospital, thromboembolism prophylactic measures need to be based on the reason for admission and the necessity for immobilization.ConclusionsRecommendations for thromboembolism diagnostic and prophylactic measures follow best practice in most spinal cord injury centers. More research evidence needs to be generated to administer more individually tailored risk-adapted prophylactic strategies in the future, which may help to further prevent thromboembolic events without causing major side effects. The present article is a translation of the guideline recently published online (https://www.awmf.org/uploads/tx_szleitlinien/179-015l_S1_Thromboembolieprophylaxe-bei-Querschnittlaehmung_2020-09.pdf).

Combination of mild therapeutic hypothermia and adipose-derived stem cells for ischemic brain injury.

Mild therapeutic hypothermia has been shown to mitigate cerebral ischemia, reduce cerebral edema, and improve the prognosis of patients with cerebral ischemia. Adipose-derived stem cell-based therapy can decrease neuronal death and infiltration of inflammatory cells, exerting a neuroprotective effect. We hypothesized that the combination of mild therapeutic hypothermia and adipose-derived stem cells would be neuroprotective for treatment of stroke. A rat model of transient middle cerebral artery occlusion was established using the nylon monofilament method. Mild therapeutic hypothermia (33°C) was induced after 2 hours of ischemia. Adipose-derived stem cells were administered through the femoral vein during reperfusion. The severity of neurological dysfunction was measured by a modified Neurological Severity Score Scaling System. The area of the infarct lesion was determined by 2,3,5-triphenyltetrazolium chloride staining. Apoptotic neurons were detected by terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling (TUNEL) staining. The regeneration of microvessels and changes in the glial scar were detected by immunofluorescence staining. The inflammatory responses after ischemic brain injury were evaluated by in situ staining using markers of inflammatory cells. The expression of inflammatory cytokines was measured by reverse transcription-polymerase chain reaction. Compared with mild therapeutic hypothermia or adipose-derived stem cell treatment alone, their combination substantially improved neurological deficits and decreased infarct size. They synergistically reduced the number of TUNEL-positive cells and glial fibrillary acidic protein expression, increased vascular endothelial growth factor levels, effectively reduced inflammatory cell infiltration and down-regulated the mRNA expression of the proinflammatory cytokines interleukin-1β, tumor necrosis factor-α and interleukin-6. Our findings indicate that combined treatment is a better approach for treating stroke compared with mild therapeutic hypothermia or adipose-derived stem cells alone.

Cerebral ischemia/reperfusion injury induces airway mucus hypersecretion in rats and activates IL‑13‑associated inflammatory mechanisms.

The majority of patients that suffer a stroke have excessive sputum, which accelerates the development of pulmonary complications. However, it is unclear whether cerebral ischemia and reperfusion (I/R) injury induces mucus hypersecretion, and the potential role of inflammation remains unknown. In the present study, the reversible middle cerebral artery occlusion model was applied in rats to induce cerebral I/R injury. The rats were grouped according to the duration of reperfusion (6, 12, 24, 48 and 72h). Neurological dysfunction was evaluated by Longa scoring and lung dry‑to‑wet weight (dw/ww) ratios were determined to reflect the degree of mucus secretion. Inflammatory factor interleukin‑13 (IL‑13) and tumor necrosis factor‑α (TNF‑α) levels in serum and bronchoalveolar lavage fluid (BALF) were determined by enzyme‑linked immunosorbent assay. Pulmonary levels of mucin 5AC (MUC5AC) and key molecules involved in nuclear factor‑κB (NF‑κB) signaling were determined by western blotting and immunohistochemistry. Rats with cerebral I/R had impaired neurological function, which was associated with the length of reperfusion time. In addition, the dw/ww lung ratio decreased and the pulmonary expression of MUC5AC increased with the increase in severity of neurological dysfunction, indicating that cerebral I/R may induce mucus hypersecretion in a reperfusion time‑dependent manner. IL‑13 and TNF‑α levels in serum and BALF, as well as the nuclear translocation of NF‑κB p65 in pulmonary tissues, significantly increased following cerebral I/R, which suggests that the activation of IL‑13 and NF‑κB inflammatory pathways may be involved. The present study concluded that cerebral I/R injury may induce airway mucus hypersecretion by activating IL‑13 and NF‑κB inflammatory pathways.

Spinal Dural Arteriovenous Fistula: A Review.

Spinal dural arteriovenous fistula (SDAVF) is a rare disease, the etiology of which is not entirely clear. It is the most common vascular malformation of the spinal cord, comprising 60-80 % of the cases. The clinical presentation and imaging findings may be nonspecific and misleading, often mistaking it for other entities like demyelinating or degenerative diseases of the spine.This chapter describes the imaging findings, clinical signs, and symptoms of this disease and also the available treatment options according to the current literature.Angiography is still considered the gold standard for diagnosis; however, MRI/MRA is increasingly used as a screening tool. Modern endovascular techniques are becoming increasingly more effective in treating SDAVF offering a less invasive treatment option; however, they still lag behind surgical success rates which approach 100 %. The outcome of both treatment options is similar if complete obliteration of the fistula is obtained and depends mainly on the severity of neurological dysfunction before treatment.Heightened awareness by radiologists and clinicians to this rare entity is essential to make a timely diagnosis of this treatable disease. A multidisciplinary treatment approach is required in order to make appropriate treatment decisions.

Brain-Derived Neurotrophic Factor in children with Autism Spectrum Disorder

Autism Spectrum Disorder (ASD) is a complex neurobehavioral syndrome with no known biomarker so far for early detection. It has been challenging, both to classify typical autism and associate a suitable biomarker with clinical phenotype spectrum. Brain-derived neurotrophic factor (BDNF) has emerged as a key neurotrophin regulating synaptic plasticity, neuronal differentiation and survival. Recently, BDNF depletion is reported in neurodegenerative as well as in psychiatric disorders, associated with severity of neurological dysfunction. Role of BDNF as a biomarker in ASD is gaining significance. Pre-clinical results have linked BDNF depletion in autism and mental retardation, however, with conflicting findings. In view of this, a preliminary study was carried out to measure serum BDNF levels in 48 children with ASD and mental retardation, and 29 age-matched controls. Serum BDNF levels were found significantly higher (p<0.001) in atypical autistic subjects (clinically milder phenotype) as compared to controls, but not in typical ASD cases (clinically severe phenotype). BDNF levels were significantly lower in females with typical/Rett Syndrome (p<0.05), but not in males with typical autism (p>0.1), as compared to controls. Lower BDNF levels indicate impairment in neuroprotective mechanism, while higher levels may imply a manifested protective response. Our study highlights the differential BDNF response based on the severity of neurobehavioral deficit, indicating a possible neuroprotective role of this molecule and supporting its exploration in targeted therapy in ASD.

Brain-Derived Neurotrophic Factor (BDNF) in Children with Autism Spectrum Disorder

Background: Autism Spectrum Disorder (ASD) is a complex neurobehavioral syndrome with no known biomarker so far for early detection. It has been challenging, both to classify typical autism and associate a suitable biomarker with clinical phenotype spectrum. Brain-derived neurotrophic factor (BDNF) has emerged as a key neurotrophin regulating synaptic plasticity, neuronal differentiation and survival. Purpose: Recently, BDNF depletion is reported in neurodegenerative as well as in psychiatric disorders, associated with severity of neurological dysfunction. Role of BDNF as a biomarker in ASD is gaining significance. Pre-clinical results have linked BDNF depletion in autism and mental retardation, however, with conflicting findings. Methods: In view of this, a preliminary study was carried out to measure serum BDNF in 48 children with ASD and mental retardation, and 29 age-matched controls. Results: Serum BDNF levels were found significantly higher in atypical autistic subjects (clinically milder phenotype) as compared to controls, but not in typical ASD cases (clinically severe phenotype). BDNF levels were significantly lower in females with typical / Rett Syndrome, but not in males with typical autism, as compared to controls. Lower BDNF levels indicate impairment in neuroprotective mechanism, while higher levels may imply a manifested protective response. Conclusion: Our study highlights the differential BDNF response based on the severity of neurobehavioral deficit, indicating a possible neuroprotective role of this molecule and supporting its exploration in targeted therapy in ASD.

CD40 ligand as a potential biomarker for atherosclerotic instability

Objective: The signaling protein CD40 and its ligand, CD40L, are thought to contribute to atherosclerotic plaque formation and rupture. We sought to determine their utility as markers of cerebral atherosclerosis and neurological dysfunction.Methods: We recruited 82 patients with acute cerebral infarction (ACI) and classified each as having large-artery atherosclerosis (LAA, 30), small-artery occlusion (36), or cardioaortic embolism (16). We also recruited 17 patients who had carotid artery stenosis (CAS) without stroke and 20 healthy individuals as controls. CD40L expression on peripheral blood monocytes (PBMCs) was detected by direct immunofluorescence with flow cytometry, and serum soluble CD40L (sCD40L) was measured by ELISA.Results: CD40L expression on PBMCs was highest in the LAA group, followed by that in the CAS group (both P < 0·01 versus control). It was also higher in patients with atherosclerotic infarction than in those without atherosclerosis (P < 0·05). PBMC CD40L expression was sensitive and specific for detecting atherosclerosis and LAA cerebral infarction and was superior to serum C-reactive protein for predicting cerebral atherosclerosis (P < 0·01). Serum sCD40L was higher in patients with ACI than in healthy controls (P < 0·01) or patients with CAS (P < 0·01) and correlated with increased disability on three scales (all P < 0·01).Conclusions: We conclude that patients with ACI have an upregulated CD40–CD40L system, which could be used as a clinical biomarker for assessing atherosclerotic instability and severity of neurological dysfunction.

Continuous and Intermittent CSF Diversion after Subarachnoid Hemorrhage: a Pilot Study

We examine two accepted methods of managing cerebrospinal fluid (CSF) drainage in patients following subarachnoid hemorrhage (SAH). The first is intermittent CSF drainage when intracranial pressure (ICP) reaches a pre-defined threshold (monitor-first) and the second is continuous CSF drainage (drain-first) at set pressure thresholds. This pilot study is designed to determine if there is a cause for a randomized study of comparing the two methods. This prospective observational pilot study enrolled 37 patients with SAH and external ventricular drainage between October 2008 and August 2009. Patients were treated with one of two methods of ICP management (drain-first vs. monitor-first) according to the discretion of the admitting physician. There were no significant differences in baseline characteristics including age, gender, severity of neurological dysfunction, and radiographic findings between the two groups. The incidence of vasospasm was not different between the drain-first group (66.7%; 16 of 24 patients) and the monitor-first group (53.9%; 7 of 13 patients). This pilot study was neither powered, nor expected to detect a difference between groups. The results of this study provide support for the design and conduct of a randomized study to assess the impact of two methods of CSF diversion for patients with SAH.

Meningomyelitis in dogs: a retrospective review of 28 cases (1999 to 2007)

To characterise dogs with meningomyelitis and to compare signalment, body temperature and vaccination status to a representative control group. Dogs with meningomyelitis were identified retrospectively. Signalment, history, vaccination status, body temperature, severity of neurological dysfunction (modified Frankel score), diagnostic procedures, aetiology, survival to discharge and long-term survival were analysed. Affected dogs were younger (P<0.05) and more frequently hound or toy breeds (P<0.05) when compared with controls. Hound and toy breed dogs less than or equal to three years of age had a 13 times higher odds of meningomyelitis compared with other breeds (P<0.001). General proprioceptive ataxia, limb paresis and paraspinal hyperaesthesia were the most common clinical signs. Meningomyelitis of unknown aetiology and granulomatous meningomyelitis were the most common diagnoses. The median time to death or continued follow-up in dogs alive at discharge was 213 days. Meningomyelitis resulted in death or euthanasia in 14 of 28 dogs. Clinical signs improved or resolved in seven of 28 dogs. Meningomyelitis is a differential diagnosis for dogs with clinical signs of myelopathy. Young dogs and toy or hound breeds seem to be predisposed. Clinical signs of meningomyelitis improve or resolve in some dogs.

High incidence of hyperoxaluria in generalized peroxisomal disorders

The Zellweger spectrum disorders (ZSDs) are characterized by a generalized loss of peroxisomal functions caused by deficient peroxisomal assembly. Clinical presentation and survival are heterogeneous. Although most peroxisomal enzymes are unstable in the cytosol of peroxisome-deficient cells of ZSD patients, a few enzymes remain stable among which alanine:glyoxylate aminotransferase (AGT). Its deficiency causes primary hyperoxaluria type 1 (PH1, MIM 259900), an inborn error of glyoxylate metabolism characterized by hyperoxaluria, nephrocalcinosis, and renal insufficiency. Despite the normal level of AGT activity in ZSD patients, hyperoxaluria has been reported in several ZSD patients. We observed the unexpected occurrence of renal stones in a cohort of ZSD patients. This led us to perform a study in this cohort to determine the prevalence of hyperoxaluria in ZSDs and to find clinically relevant clues that correlate with the urinary oxalate load. We reviewed medical charts of 31 Dutch ZSD patients with prolonged survival (>1 year). Urinary oxalate excretion was assessed in 23 and glycolate in 22 patients. Hyperoxaluria was present in 19 (83%), and hyperglycolic aciduria in 14 (64%). Pyridoxine treatment in six patients did not reduce the oxalate excretion as in some PH1 patients. Renal involvement with urolithiasis and nephrocalcinosis was present in five of which one developed end-stage renal disease. The presence of hyperoxaluria, potentially leading to severe renal involvement, was statistically significant correlated with the severity of neurological dysfunction. ZSD patients should be screened by urinalysis for hyperoxaluria and renal ultrasound for nephrocalcinosis in order to take timely measures to prevent renal insufficiency.

Duration and Severity of Clinical Signs as Prognostic Indicators in 30 Dogs with Thoracolumbar Disk Disease After Surgical Decompression

The objective of this study was to evaluate the duration and severity of neurological dysfunction as prognostic indicators for the final outcome in 30 dogs with thoracolumbar intervertebral disk disease, treated surgically with hemilaminectomy without concurrent prophylactic fenestration. The lesion localization was assessed comparing plain radiographic, myelographic and surgical findings and success rates were evaluated during case follow-up. Evidence of a single disk extrusion existed in 17 dogs on survey spinal radiographs, while myelography revealed definite spinal cord compression in 28 cases and was inconclusive in the remaining two cases. Case follow-up continued for a period ranging from 3 months to 2 years post-operatively, depending on the degree of neurological dysfunction on presentation and owner compliance. The severity of clinical signs and the interval elapsing prior to decompressive surgery did not correlate with the case outcome, with 12 of 20 dogs (60%) that exhibited the most pronounced neurological dysfunction (paraplegia with or without analgesia) regaining voluntary motor function during the follow-up period. Recurrences proven to be disk-related did not occur in any dog throughout the post-surgical observation period. It was concluded that the time elapsing from loss of motor function and the severity of clinical signs is not an objective method to predict the case outcome.

The neurology of learning and behavioural problems in pre-adolescent children.

The aim of the study was to evaluate whether in pre-adolescent children specific types of minor neurological dysfunction (MND) are related to specific types of learning and behavioural problems, and whether it is the type or the severity of neurological dysfunction that matters most. A total of 1186, 5.5-11-year-old, children were examined for the presence of clusters of signs of MND. Parents and teachers completed brief behaviour rating scales. In school-aged children (n = 580) cognitive achievement was assessed using standardized Dutch tests. Least-squares regression was used to examine neurobehavioural and neurocognitive links. Children with more MND clusters performed worse scholastically and had more signs of attention deficit than others. Externalizing and internalizing behaviour were only linked to specific forms of MND. Evaluation of the neurological condition of the child with learning and behavioural problems offers insight into the aetiology and pathogenesis of these problems.

Effectiveness of action on prevention in the Southern Poland epidemiologic survey. Distribution of blood pressure in relation to age and gender

The Zellweger spectrum disorders (ZSDs) are characterized by a generalized loss of peroxisomal functions caused by deficient peroxisomal assembly. Clinical presentation and survival are heterogeneous. Although most peroxisomal enzymes are unstable in the cytosol of peroxisome-deficient cells of ZSD patients, a few enzymes remain stable among which alanine:glyoxylate aminotransferase (AGT). Its deficiency causes primary hyperoxaluria type 1 (PH1, MIM 259900), an inborn error of glyoxylate metabolism characterized by hyperoxaluria, nephrocalcinosis, and renal insufficiency. Despite the normal level of AGT activity in ZSD patients, hyperoxaluria has been reported in several ZSD patients. We observed the unexpected occurrence of renal stones in a cohort of ZSD patients. This led us to perform a study in this cohort to determine the prevalence of hyperoxaluria in ZSDs and to find clinically relevant clues that correlate with the urinary oxalate load. We reviewed medical charts of 31 Dutch ZSD patients with prolonged survival (>1 year). Urinary oxalate excretion was assessed in 23 and glycolate in 22 patients. Hyperoxaluria was present in 19 (83%), and hyperglycolic aciduria in 14 (64%). Pyridoxine treatment in six patients did not reduce the oxalate excretion as in some PH1 patients. Renal involvement with urolithiasis and nephrocalcinosis was present in five of which one developed end-stage renal disease. The presence of hyperoxaluria, potentially leading to severe renal involvement, was statistically significant correlated with the severity of neurological dysfunction. ZSD patients should be screened by urinalysis for hyperoxaluria and renal ultrasound for nephrocalcinosis in order to take timely measures to prevent renal insufficiency.