Neurological Examination Scores Research Articles

Early Neurodevelopmental Assessments for Predicting Long-Term Outcomes in Infants at High Risk of Cerebral Palsy

Studies suggest that early neurodevelopmental assessments are beneficial for identifying cerebral palsy, yet their effectiveness in practical scenarios and their ability to detect cognitive impairment are limited. To assess the effectiveness of early neurodevelopmental assessments in identifying cerebral palsy and cognitive and other neurodevelopmental impairments, including their severity, within a multidisciplinary clinic. This diagnostic study was conducted at Monash Children's Hospital, Melbourne, Australia. Participants were extremely preterm infants born at less than 28 weeks' gestation or extremely low birth weight infants less than 1000 g and term encephalopathic infants who received therapeutic hypothermia, attending the early neurodevelopmental clinic between January 2019 and July 2021. Data were analyzed from December 2023 to January 2024. Early cerebral palsy or high risk of cerebral palsy, the absence of fidgety movements, and Hammersmith Infant Neurological Examination (HINE) scores at corrected age (CA) 3 to 4 months. Early cerebral palsy or high risk of cerebral palsy diagnosis was based on absent fidgety movements, a low HINE score (<57), and medical neurological examination. The outcomes of interest were cerebral palsy, cognitive and neurodevelopmental impairments and their severity, diagnosed at 24 to 36 months' CA. A total of 116 infants (median [IQR] gestational age, 27 [25-29] weeks; 65 [56%] male) were included. Diagnosis of early cerebral palsy or high risk of cerebral palsy demonstrated a sensitivity of 92% (95% CI, 63%-99%) and specificity of 84% (95% CI, 76%-90%) for predicting cerebral palsy and 100% (95% CI, 59%-100%) sensitivity and 80% (95% CI, 72%-87%) specificity for predicting moderate to severe cerebral palsy. Additionally, the accuracy of diagnosis of early cerebral palsy or high risk of cerebral palsy was 85% (95% CI, 77%-91%) for predicting cerebral palsy and 81% (95% CI, 73%-88%) for predicting moderate to severe cerebral palsy. Similarly, the absence of fidgety movements had an 81% (95% CI, 73%-88%) accuracy in predicting cerebral palsy, and HINE scores exhibited good discriminatory power with an area under the curve of 0.88 (95% CI, 0.79-0.97) for cerebral palsy prediction. However, for cognitive impairment, the predictive accuracy was 44% (95% CI, 35%-54%) for an early cerebral palsy or high risk of cerebral palsy diagnosis and 45% (95% CI, 36%-55%) for the absence of fidgety movements. Similarly, HINE scores showed poor discriminatory power for predicting cognitive impairment, with an area under the curve of 0.62 (95% CI, 0.51-0.73). In this diagnostic study of infants at high risk for cerebral palsy or other cognitive or neurodevelopmental impairment, early neurodevelopmental assessments at 3 to 4 months' CA reliably predicted cerebral palsy and its severity at 24 to 36 months' CA, signifying its crucial role in facilitating early intervention. However, for cognitive impairment, longer-term assessments are necessary for accurate identification.

Residual volume of extruded disc material and residual spinal cord compression measured on postoperative MRI do not predict neurological outcomes in dogs following decompressive surgery for thoracolumbar intervertebral disc extrusion.

Published studies on the validity of using quantitative MRI measures of pre- and postoperative spinal cord (SC) compression as prognostic indicators for dogs undergoing surgery for intervertebral disc extrusion (IVDE) are currently limited. The aim of this retrospective analytical study was to describe the volume of postoperative residual extradural material (VREM) and the ratio of the cross-sectional area (CSA) of maximum SC compression to the CSA of SC in a compression-free intervertebral space as MRI measures of preoperative and postoperative compression (residual spinal cord compression, RSCC), and to compare these measures between the neurological outcome in a group of dogs. Inclusion criteria were dogs that underwent surgery for thoracolumbar IVDE, were imaged pre- and immediately postoperatively by MRI, and had a neurological follow-up examination 2 to 5 weeks postoperatively. Two blinded observers independently performed measurements in pre- and postoperative MRI studies. Dogs were classified into positive outcome (PO) and negative outcome (NO) groups based on follow-up neurologic examination scores. Seventeen dogs were included (12 PO, 5 NO). Interobserver agreement for MRI measurements was good to excellent (ICCs: 0.76-0.97). The prevalence of residual extradural material in postoperative MRI studies was 100%. No significant differences in mean preoperative SC compression, mean RSCC, mean SC decompression, or VREM were found between outcome groups (P=.25; P=.28; P=.91, P=.98). In conclusion, neither postoperative VREM nor RSCC could predict successful neurological outcomes.

KONJENİTAL SANTRAL HİPOVENTİLASYON SENDROMU VE SEREBRAL PALSİLİ OLGUDA FİZYOTERAPİ VE REHABİLİTASYON PROGRAMININ ETKİSİNİN İNCELENMESİ: BİR OLGU SUNUMU

Congenital Central Hypoventilation Syndrome is a rare genetic disorder that presents from birth and prevents automatic control of respiration. Our aim in this study was to examine the effectiveness of a 6-month physical therapy program applied to a patient with Congenital Central Hypoventilation Syndrome and cerebral palsy. A 6-month-old patient who was diagnosed with Congenital Central Hypoventilation Syndrome and subsequently developed Cerebral Palsy due to asphyxia was included in the study. The case was enrolled in a physical therapy program for 2 days a week for 6 months in the home environment where they lived. The case was evaluated before and after treatment. In our case, the Modified Ashworth Scale, the Alberta Infant Motor Scale, the Hammersmith Infant Neurological Examination, Test of Sensory Functions in Infants, and the Face, Legs, Activity, Cry, Consol ability scale were used. In our case, pre-treatment and post-treatment scores were as follows, respectively: Alberta Infant Motor Scale score 3-8, Hammersmith Infant Neurological Examination score 6-17, Test of Sensory Functions in Infants score 2-9, and Face, Legs, Activity, Cry, Consol ability scale score 8-3. In our case, there was a significant improvement in Modified Ashworth Scale scores before and after treatment. Additionally, at the conclusion of the study, it was noted that the heightened pain and sensitivity resulting from the patient's extended stay in the intensive care unit reduced with the treatment. We believe that the administered physiotherapy and rehabilitation program offered support to the patient in achieving neuro-motor and sensory integration, underscoring the essential role of physiotherapy in rare diseases.

Safety and effectiveness of nusinersen, a treatment for spinal muscular atrophy, in 524 patients: results from an interim analysis of post-marketing surveillance in Japan

ABSTRACT Purpose Nusinersen is an antisense oligonucleotide approved for the treatment of spinal muscular atrophy (SMA). A post-marketing surveillance (PMS) has been ongoing (August 2017–August 2025) in all patients in Japan who were administered nusinersen intrathecally in real-world clinical settings. We report the interim analysis results for safety and effectiveness. Methods This interim analysis was conducted using data collected from 524 patients whose case report forms were obtained at least once by May 30, 2022. Collected data included patient demographics and adverse events (AEs) for safety, and motor function assessments and Clinical Global Impressions of Improvement (CGI-I) for effectiveness. Results Of the 524 patients in the safety analysis set, 522 patients who were diagnosed with SMA were included in the effectiveness analysis (infantile-onset SMA [n = 153, 29.3%], later-onset SMA [n = 369, 70.7%]). The median duration of treatment was 785.0 (range 1–1549) days. AEs occurred in 35.9% of patients (49.0% in infantile-onset SMA and 30.6% in later-onset SMA). Nusinersen treatment significantly improved Hammersmith Infant Neurological Examination scores in patients with infantile-onset SMA and Hammersmith Functional Motor Scale-Expanded scores in patients with later-onset SMA for up to nearly 3 years. Based on CGI-I assessments, 98.5–100% of patients receiving nusinersen "improved" or remain "unchanged". Conclusions This interim analysis of the large-scale, all-case PMS in patients who were administered nusinersen in Japan supports the safety and effectiveness of nusinersen. The benefit–risk balance of nusinersen treatment remains favorable.

Early neurodevelopmental outcomes of extreme preterm infants exposed to paracetamol: a retrospective cohort study

BackgroundParacetamol is commonly used for analgesia and patent ductus arteriosus (PDA) treatment in preterm infants. We aimed to evaluate early neurodevelopmental outcomes of extreme preterm infants exposed to paracetamol during their neonatal admission.MethodsThis retrospective cohort study included surviving infants born at <29 weeks gestation, or with a birth weight of <1000 grams. Neurodevelopmental outcomes studied were early cerebral palsy (CP) or high risk of CP diagnosis, Hammersmith Infant Neurological Examination (HINE) score and Prechtl General Movement Assessment (GMA) at 3–4 months corrected age.ResultsTwo hundred and forty-two infants were included, of which 123 were exposed to paracetamol. After adjusting for birth weight, sex and chronic lung disease, there were no significant associations between paracetamol exposure and early CP or high risk of CP diagnosis (aOR 1.46, 95% CI 0.61, 3.5), abnormal or absent GMA (aOR 0.82, 95% CI 0.37, 1.79) or HINE score (adjusted β −0.19, 95% CI −2.39, 2.01). Subgroup analysis stratifying paracetamol exposure into <180 mg/kg or ≥180 mg/kg cumulative dose found that neither had significant effects on outcomes.ConclusionsIn this cohort of extreme preterm infants, no significant association was found between exposure to paracetamol during the neonatal admission and adverse early neurodevelopment.ImpactParacetamol is commonly used in the neonatal period for analgesia and patent ductus arteriosus treatment in preterm infants, although prenatal paracetamol use has been associated with adverse neurodevelopmental outcomes.Exposure to paracetamol during the neonatal admission was not associated with adverse early neurodevelopment at 3–4 months corrected age in this cohort of extreme preterm infants.The findings from this observational study is consistent with the small body of literature supporting the lack of association between neonatal paracetamol exposure and adverse neurodevelopmental outcomes in preterm infants.

Blood spinal cord barrier disruption recovers in patients with degenerative cervical myelopathy after surgical decompression: a prospective cohort study

The pathophysiology of degenerative cervical myelopathy (DCM) is characterized by chronic compression-induced damage to the spinal cord leading to secondary harm such as disruption of the blood spinal cord barrier (BSCB). It is therefore the purpose of this study to analyze BSCB disruption in pre- and postoperative DCM patients and to correlate those with the clinical status and postoperative outcome. This prospectively controlled cohort included 50 DCM patients (21 female; 29 male; mean age: 62.9 ± 11.2 years). As neurological healthy controls, 52 (17 female; 35 male; mean age 61.8 ± 17.3 years) patients with thoracic abdominal aortic aneurysm (TAAA) and indication for open surgery were included. All patients underwent a neurological examination and DCM-associated scores (Neck Disability Index, modified Japanese Orthopaedic Association Score) were assessed. To evaluate the BSCB status, blood and cerebrospinal fluid (CSF) samples (lumbar puncture or CSF drainage) were taken preoperatively and in 15 DCM patients postoperatively (4 female; 11 male; mean age: 64.7 ± 11.1 years). Regarding BSCB disruption, CSF and blood serum were examined for albumin, immunoglobulin (Ig) G, IgA and IgM. Quotients for CSF/serum were standardized and calculated according to Reiber diagnostic criteria. Significantly increased preoperative CSF/serum quotients were found in DCM patients as compared to control patients: AlbuminQ (p < .001), IgAQ (p < .001) and IgGQ (p < .001). IgMQ showed no significant difference (T = − 1.15, p = .255). After surgical decompression, neurological symptoms improved in DCM patients, as shown by a significantly higher postoperative mJOA compared to the preoperative score (p = .001). This neurological improvement was accompanied by a significant change in postoperative CSF/serum quotients for Albumin (p = .005) and IgG (p = .004) with a trend of a weak correlation between CSF markers and neurological recovery. This study further substantiates the previous findings, that a BSCB disruption in DCM patients is evident. Interestingly, surgical decompression appears to be accompanied by neurological improvement and a reduction of CSF/serum quotients, implying a BSCB recovery. We found a weak association between BSCB recovery and neurological improvement. A BSCB disruption might be a key pathomechanism in DCM patients, which could be relevant to treatment and clinical recovery.

Role of narrative medicine-based education in cultivating empathy in residents

ObjectiveTo explore the role of narrative medicine-based education in standardized empathy training for residents.MethodsAmong the 2018–2020 residents at the First Affiliated Hospital of Xinxiang Medical University, 230 receiving neurology training were enrolled in this study and randomly divided into study and control groups. The study group received narrative medicine-based education and standardized routine resident training. The Jefferson Scale of Empathy–Medical Student version (JSE–MS) was used to evaluate empathy in the study group, and the neurological professional knowledge test scores of the two groups were also compared.ResultsIn the study group, the empathy score was higher than the preteaching score (P < 0.01). The neurological professional knowledge examination score was higher in the study group than in the control group, albeit not significantly.ConclusionThe addition of narrative medicine-based education in standardized training improved empathy and may have improved the professional knowledge of neurology residents.

Early Electroencephalogram as Predictor of Neuromotor Outcome in a Term Newborn with Hypoxic Ischaemia Encephalopathy- A Prospective Cohort Study

Introduction: Birth asphyxia is the leading cause of neonatal morbidity and mortality globally. Early prediction of neuromotor problems in babies with Hypoxic Ischaemic Encephalopathy (HIE) allows parental counseling regarding treatment continuation, better utilisation of limited resources and prompt referral for early intervention services to so that the best possible outcomes can be achieved. Aim: To investigate the role of early Electroencephalogram (EEG) background activity in prediction of cerebral palsy, epilepsy and developmental delay in term newborn with HIE and also to check the association of EEG background activity with Hammersmith Neonatal Neurological Examination (HNNE), Hammersmith Infant Neurological Examination (HINE) score and various short-term and long-term outcome. Materials and Methods: This prospective clinical study was conducted in Neonatal Intensive Care Unit (NICU) of Department of Paediatrics, Shyam Shah Medical college and associated Gandhi Memorial Hospital, Rewa (MP) during April 2019 to June 2020. Total 81 term neonates with HIE underwent conventional EEG within six hours of birth using International 10-20 system, (modified for neonate) for electrode placement. At the time of discharge HNNE was performed. On follow-up (6 to 12 months), cerebral palsy was evaluated using HINE and modified Ashworth score, Developmental delay by Development Assessment Scale for Indian Infant (DASII) scale and for epilepsy by clinical history and EEG background activity. Association between EEG background activity, HNNE, HINE score and various short-term and long-term outcome was calculated using Chi-square test and Fisher’s-exact test. Results: In present study, out of 81 cases, 58 (71.6%) cases were male and 23 (28.4%) cases were female with mean Gestational Age (GA) of 39 weeks. A total of 57 neonates were discharged successfully after stay of mean duration 13.8 days in NICU. All neonates with normal/mildly abnormal EEG background activity had 100% survival rates whereas only 68.2% (15) and 37% (10) could be discharged from moderately abnormal and severely abnormal EEG background activity group. Out of discharged patients none with severely abnormal background had optimal HNNE whereas 75% (24) of mildly abnormal EEG had optimal HNNE score. Mean HNNE and HINE score was significantly lower in newborns with severely abnormal EEG (mean score 20.8) as compared to normal EEG group (mean score 30.3). Severely abnormal EEG at birth had significant association with cerebral palsy (p-value&lt;0.0001), epilepsy (p-value= 0.003) and developmental delay (p-value&lt;0.001) as compared to moderately and mildly abnormal EEG background activity in newborns with HIE. Conclusion: EEG within six hours of birth in term neonate with HIE has very high sensitivity and Negative Predictive Value (NPV) in predicting short-term outcome as death, poor neurological outcome and long-terms outcome as cerebral palsy, epilepsy and developmental delay.

Effect of decompressive hemicraniectomy performed within the first 48 hours on mortality in the treatment of malignant infarction of the middle cerebral artery

To evaluate the clinical outcomes of patients with malignant middle cerebral artery (MCA) infarction who underwent wide decompressive hemicraniectomy. Fifty-two patients with large MCA infarcts were retrospectively evaluated, and 12 patients who underwent extensive decompressive hemicraniectomy and duraplasty with a bone flap extending beyond the infarct borders were included in the study. The degree of stroke (stroke volume), preoperative and postoperative midline shift of the craniectomy area and improvement in midline shift were calculated on computed tomography. Preoperative and postoperative neurologic examination scores were determined using the modified Rankin Scale. The mean age was 63 (min: 41, max: 79) years. There was left MCA infarction in one patient and right MCA infarction in 11 patients. The postoperative follow-up period of the operated patients ranged from 14 to 90 days. The neurological examination of seven patients (58.3%) showed significant improvement. Eight (66.6%) patients survived. Postoperatively, the mean midline deviation value of 12 patients decreased from 11.04 to 4.8. It is considered that wide decompressive craniectomy performed in young patients in the early period with strict radiological and clinical follow-up can increase survival and functional recovery.

Association of electrochemical skin conductance with neuropathy in chemotherapy-treated patients

PurposeChemotherapy-induced peripheral neuropathy (CIPN) is an adverse event of cancer treatment that can affect sensory, motor, or autonomic nerves. Assessment of autonomic neuropathy is challenging, with limited available tools. Accordingly, it is not routinely assessed in chemotherapy-treated patients. In this study, we aimed to examine whether electrochemical skin conductance (ESC) via Sudoscan, a potential measure of autonomic function, associates with subjective and objective measures of CIPN severity and autonomic neuropathy.MethodsA cross-sectional assessment of patients who completed neurotoxic chemotherapy 3–24 months prior was undertaken using CIPN patient-reported outcomes (EORTC-QLQ-CIPN20), clinically graded scale (NCI-CTCAE), neurological examination score (TNSc), autonomic outcome measure (SAS), and Sudoscan. Differences in CIPN severity between participants with or without ESC dysfunction were investigated. Linear regression analyses were used to identify whether ESC values could predict CIPN severity.ResultsA total of 130 participants were assessed, with 93 participants classified with CIPN according to the clinically graded scale (NCI-CTCAE/grade ≥ 1), while 49% demonstrated hands or feet ESC dysfunction (n = 46). Participants with ESC dysfunction did not significantly differ from those with no dysfunction on multiple CIPN severity measures (clinical-grade, patient-report, neurological examination), and no differences on the autonomic outcome measure (SAS) (all p > 0.0063). Linear regression analyses showed that CIPN could not be predicted by ESC values.ConclusionsThe inability of ESC values via Sudoscan to predict clinically-graded and patient-reported CIPN or autonomic dysfunction questions its clinical utility for chemotherapy-treated patients. The understanding of autonomic neuropathy with chemotherapy treatment remains limited and must be addressed to improve quality of life in cancer survivors.

Biomarkers for brain injury and copper toxicity in Wilson’s disease: a diffusion tensor imaging study

IntroductionChelation therapy is used to ‘de-copper’ patients with Wilson’s disease but neurological outcomes are unpredictable and biomarkers of end-organ damage are needed.MethodsWe prospectively performed clinical assessments, venepuncture and 3T MRI in 40 patients with Wilson’s disease. Using tract-based spatial statistics, we compared diffusion tensor imaging parameters across white matter tracts between patients with neurological (n=23) and hepatic (n=17) presentations and active (n=5) and stable (n=35) disease. We also tested associations with neurological examination scores and serum free copper concentrations in stable patients.ResultsThere were no differences in DTI parameters between patients with neurological and hepatic presentations, however examination scores were negatively associated with axial diffusivity in subcortical tracts in stable patients. Patients with active disease had widespread increases in mean, axial and, to a lesser extent, radial diffusivity. In contrast, increasing serum copper concentrations in stable patients were associated with widespread increases in mean, radial and, to a lesser extent, axial diffusivity.ConclusionsWe demonstrate that distinct patterns of abnormal white matter diffusivity are associated with brain injury and copper toxicity in Wilson’s disease. Decreases in axial diffusivity in chronically-treated patients likely reflect axonal loss. Increases in axial and radial diffusivity are a promising biomarker for monitoring treatment response.s.shribman@ucl.ac.uk

Plasma neurofilament light levels as a novel biomarker for neurological involve- ment in Wilson’s disease

BackgroundSerum and urine copper levels are used to monitor chelation therapy for Wilson’s disease (WD) but do not reflect neurological involvement. Biomarkers of end-organ damage are needed to optimise chelation therapy and prepare for clinical trials for gene therapy.MethodsWe measured neurofilament light (NfL) levels in plasma samples, Unified WD Rating Scale (UWDRS) scores and brain volumes on T1-weighted MRI at baseline visits for a longitudinal, observational study. Participants were divided into neurological (n=25) and hepatic (n=15) groups according to UWDRS neurological examination (UWDRS-N) scores. Those with new diagnoses (n=2) or recent neurological dete- rioration associated with non-adherence (n=3) were subcategorised as having active disease. Plasma NfL levels were measured in age-matched healthy controls (n=38) for comparison.ResultsPlasma NfL levels were higher in neurological compared with hepatic and healthy control groups (8.6 vs 7.6 vs 7.6 ng/L, p=0.046) and higher in neurological cases with active disease (22.2 vs 8.3 ng/L, p=0.004). In cases without active disease, they correlated with UWDRS-N scores (p=0.003) and cortical grey matter (p&lt;0.001) but not subcortical grey matter (p=0.071) or white matter volumes (p=0.895).ConclusionsPlasma NfL is a promising biomarker for neurological involvement that, in a small cohort, appears to be associated with neurological disease activity and clinical severity and reflects neuroaxonal injury in cortical grey matter.s.shribman@ucl.ac.uk

IDF21-0671 Identification of neurophysiological abnormalities in nerve in patients with type 1 diabetes

Background: The progression of neuropathy is a continuum from subclinical neuropathy detectable with electrophysiological tests to clinically evident one recognizable on neurological examination. Although there has been considerable research of peripheral neuropathy in type 1 diabetes, still there is controversy regarding the pattern of nerve involvement in diabetic neuropathy. Detection of the pattern of nerve involvement can help to establish a protocol for screening of diabetic peripheral neuropathy (DPN) in its subclinical stage. Aim: The aim of this study was to describe the pattern of nerve involvement in type 1 diabetic patients with subclinical neuropathy using nerve conduction studies. Method: 65 patients with type 1 diabetes, ages from 18 to 55 years, and 34 controls without diabetes matched to the patient group were included into the study. All patients and controls underwent a standard neurological examination (NSS and NDS (the variant used in Belarus) score) and bilateral nerve conduction study (NCS) of the sensory and motor lower limb nerves using NeuroSoft machine. Interpretation of the results was based on the following normal reference ranges: n. Peroneus superficialis A> 10μV, v> 45 m/s; n Suralis A> 5 μV, v> 45 m / s; n Peroneus profundus A> 3mV, v> 40 m/s; n Tibialis A> 3.5mV, v> 40m/s. Data was analysed using IBM SPSS statistics v 20.0. Results: Clinically evident DPN was detected in 14 patients with type 1 diabetes from 65 examined. The use of NCS allowed to identify the subclinical stage of polyneuropathy (abnormalities in at least two of the electrophysiological parameters) additionally in 35 patients who had no symptoms and clinical signs of DPN. In all the patients of the control group, the NCS parameters were in the reference range. Regarding abnormal NCS parameters in patients with subclinical diabetic neuropathy (conduction velocity vs. amplitude of the compound action potential), amplitudes reduction for the n. Peroneus superficialis and n. Peroneus profundus predominated. Median of the amplitude for n. Peroneus superficialis in patients with subclinical diabetic polyneuropathy was 7.22 [5.25; 8.54] μV vs. 12.4 [10.57; 16.2] μV in the control group, p <0.001; for n. Peroneus profundus 2.21 [1.68; 2.68]mV vs. 3.15 [3.04; 3.42]mV in the control group, p <0.001. Significant differences in the amplitudes for n.Suralis and n.Tibialis has not been identified. Nerve conduction velocity (NCV) in both groups – in patients with the subclinical diabetic polyneuropathy and the control group – was within the reference range. However, the medians of NCV for n. Suralis and n. Tibialis were significantly lower in the group of patients with neuropathy compared with the controls – 47.08 [44.35; 50.13]m/s vs. 51.3 [47.6; 59.05]m/s, p = 0.025 and 44.8 [42.15; 48.2]m/s vs. 49.35 [48.30; 53.45]m/s, p = 0.004, respectively. Conclusion: In patients with type 1 diabetes with a subclinical stage of diabetic polyneuropathy the most frequent neurophysiological abnormalities were reduced amplitudes (the axonal damage) for sensory and motor fibers of n. Peroneus.

Turkish Validity and Reliability of the Hammersmith Infant Neurological Examination (HINE) with High-Risk Infant Group: A Preliminary Study.

Objective: The purpose of this study was to establish the reliability of the Turkish translation of the Hammersmith Infant Neurological Examination in infants at 8-12 months corrected age and compare Hammersmith Infant Neurological Examination scores to other predictive assessments.Materials and Methods: Perinatal risk factors, term-age magnetic resonance imaging, general movements at 3-month corrected age, and 12-month corrected age The Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III) scores were obtained in 35 high-risk infants. The Hammersmith Infant Neurological Examination was evaluated using intra-rater and inter-rater reliability. Hammersmith Infant Neurological Examination scores were compared to the findings from the three other assessments.Results: Intra-rater and inter-rater reliability was high (intraclass correlation coefficient = 1.00; intraclass correlation coefficient = 0.969, P < .001, 95% CI = 0.939-0.984, respectively). Global Hammersmith Infant Neurological Examination scores were significantly lower in infants with magnetic resonance imaging evidence of brain injury than without (P < .05) and in infants without general movements Fidgety movements (P < .05), than with. There was a significant positive correlation between global Hammersmith Infant Neurological Examination scores and Bayley Scales-III cognitive (P < .001), language (P < .001), and motor composite scores (P < .001).Conclusion: This study strongly supports the use of the Turkish translation of the Hammersmith Infant Neurological Examination. Users found it readily understandable and easy to use, and the scores were consistent with 3 different methods of predicting neurodevelopmental outcomes. These findings will aid the early diagnosis, management, and support for children with neurodevelopmental problems.

Reference Material for Hammersmith Infant Neurologic Examination Scores Based on Healthy, Term Infants Age 3-7 Months

To provide a valid, continuous reference interval, including a 10th percentile cut-off, for Hammersmith Infant Neurological Examination (HINE) scores based on 3- to 7-month-old term infants with weight appropriate for gestational age. In a prospective study, we examined 168 Norwegian infants at one timepoint with HINE at 3-7months of age. In 134 of these infants Ages and Stages Questionnaire was completed by their parents at 2years of age to ensure typical motor development. We calculated a reference interval for HINE scores with the 10th percentile as cut-off for age-dependent optimal scores. The best fitting mean model for HINE total score was 78.1358+9659.231∗1/age in weeks2-5104.174∗natural logarithm(age in weeks)/age in weeks2, which explained 49.8% of the variance. The HINE total score 10th percentile cut-off corresponded to 52.1 points at age 12weeks, 55.6 points at 16weeks, 59.0 points at 20weeks, 61.8 points at 24weeks, and 63.8 points at 28weeks. We found an excellent intraclass correlation coefficient of 0.953 (0.931-0.968) between 2 examiners. The infants had a typical motor development at 2years follow-up. We have presented a valid, continuous reference interval and a 10th percentile cut-off for HINE scores for infants age 3-7months.

Factors associated with the Dubowitz neurological examination in preterm new-borns

Background: This article aims to explore the factors associated with Dubowitz neurological examination scores in preterm new-borns. Methods: This is a cross-sectional study in which forty preterm new-borns were evaluated by using the Dubowitz neurological examination on their first outpatient visit following hospital discharge. A questionnaire was also deployed to explore parental variables. Results: Preterm new-borns with low scores in the Dubowitz neurological examination were born at an even lower gestational age, required longer hospitalisation, and had more indicators for orotracheal intubation. Positive correlations between the subdomains of the Dubowitz neurological examination with birthweight, mother's age, Apgar score at 5 min, maternal and paternal education, and income were also significant. Linear regression analyses explained 26% of the of the Dubowitz neurological examination global score, retaining birthweight, income, and father’s age as significant predictors. Conclusions: Early, appropriated neurological assessments of preterm infants are critical to detect risk factors that may underpin developmental delays.

Associations Between Early Structural Magnetic Resonance Imaging, Hammersmith Infant Neurological Examination, and General Movements Assessment in Infants Born Very Preterm

To evaluate the prevalence and associations between structural magnetic resonance imaging (sMRI) injury/abnormality at term-equivalent age and absent fidgety General Movements Assessment (GMA) and abnormal Hammersmith Infant Neurological Examination (HINE) scores among infants born very preterm at 3-4months of corrected age. This prospective cohort study enrolled 392 infants born ≤2weeks of gestation from 5 neonatal intensive care units in the greater Cincinnati area between September 2016 and October 2019. Infants completed sMRI at term-equivalent age and GMA and HINE at 3-4months of corrected age. All assessors were blinded. Of 392 infants, 375 (96%) had complete data. Of these, 44 (12%) exhibited moderate or severe brain abnormalities, 17 (4.5%) had abnormal GMA, and 77 (20.3%) had abnormal HINE. Global and regional abnormality scores on sMRI were significantly correlated with GMA (R2 range 0.05-0.17) and HINE at 3-4months of corrected age (R2 range 0.01-0.17). These associations remained significant in multivariable analyses after adjusting for gestational age and sex. There was a significant but low correlation (R2 0.14) between GMA and HINE. We observed a low prevalence of moderate or severe brain abnormalities in survivors born very preterm in this geographically defined cohort. The much greater prevalence of abnormal motor examination on the HINE compared with GMA and their low correlation suggests that these tests evaluate different constructs and, thus, should be used in combination with sMRI rather than interchangeably.

Pre-ataxic Changes of Clinical Scales and Eye Movement in Machado-Joseph Disease: BIGPRO Study.

The pathological burden of spinocerebellar ataxia type 3, also known as Machado-Joseph disease (SCA3/MJD), accumulates before the beginning of symptoms. Our study aims at validating biomarkers for disease progression since pre-ataxic periods. We report on baseline findings of clinical scales and oculomotor neurophysiology. Ataxic (Scale for the Assessment and Rating of Ataxia > 2.5) and at 50% risk subjects were included. The latter were subdivided into noncarriers, pre-ataxic carriers near (PAN), or pre-ataxic carriers far from (PAFF) ataxia onset (AO), with 4 years from the predicted age at onset being the cutoff. The subjects were assessed by Neurological Examination Score for Spinocerebellar Ataxia (NESSCA), International Cooperative Ataxia Rating Scale (ICARS), Inventory of Non-Ataxic Signs (INAScount), Composite Cerebellar Functions Score and SCA Functional Index, and video-oculography, including the regression slope of vestibulo-ocular reflex gain (VORr), main sequence of volitional and reflexive vertical saccades, slow-phase velocity of central and gaze-evoked (SPV-GE) nystagmus, and vertical pursuit gain. Correction for multiple comparisons was performed; the threshold for statistical significance was P < 0.05. A total of 35 ataxic, 14 PAN, 24 PAFF, and 22 noncarriers were included. All variables showed significant differences between groups and correlated to time to onset or time after onset, among all 73 SCA3/MJD carriers; none significantly changed with age in controls. NESSCA, ICARS, INAScount, VORr, main sequence of volitional saccades, and SPV-GE not only distinguished PAN from controls but also correlated with time left to AO. Clinical scales and video-oculography variables were already altered in pre-ataxic SCA3/MJD carriers and worsened with time. NESSCA, ICARS, INAScount, VORr, main sequence of vertical volitional saccades, and SPV-GE are good candidates to measure preclinical changes in SCA3/MJD. © 2021 International Parkinson and Movement Disorder Society.

Effectiveness of Exposure in Vivo for Patients with Painful Diabetic Neuropathy: a Pilot Study of Effects on Physical Activity and Quality of Life.

ObjectiveTo evaluate the effects of personalized exposure in vivo on level of physical activity and quality of life in patients with painful diabetic neuropathy.DesignRandomized, single-case, ABC design.SubjectsTwelve patients with painful diabetic neuropathy, age > 18 years, diabetes mellitus type II, Clinical Neurological Examination score > 5, Diabetic Neuropathy Symptom Score ≥ 1 and Douleur Neuropathique 4 Questions score ≥ 3.MethodsThe treatment consists of an Intensive screening, followed by an 8-week exposure in vivo intervention specifically adapted to the needs/risks of patients with painful diabetic neuropathy, and 6-months follow-up. Outcome measures included daily and non-daily measures of physical activity, quality of life, metabolic parameters, disability, depression, general and painful diabetic neuropathy-related anxiety, pain intensity and pain catastrophizing.ResultsDue to high drop-out rates (n = 6 during screening, n = 2 during treatment, n = 1 after treatment), only 3 participants completed the study. Slight, but non-significant, changes in physical activity and disability were observed. In quality of life, no changes were observed.ConclusionAnalysis of the reasons for the high drop-out rate indicate that exposure in vivo may have added value in patients with painful diabetic neuropathy only for those patients: (i) whose daily life functioning is impaired mainly by the painful diabetic neuropathy; (ii) in whom painful diabetic neuropathy-related fears are exaggerated and irrational; (iii) in whom specific activities evoke the painful diabetic neuropathy-related fears; (iv) whose spouse and healthcare providers are involved in the treatment; and (v) who are willing to change their daily behaviour. Further research is needed into this subject.LAY ABSTRACTPainful diabetic neuropathy places a high burden on patients’ physical and emotional wellbeing. Patients with painful diabetic neuropathy may have several fears related to diabetes and pain (e.g. fear of pain, fear of falling), which can limit their physically activity in daily life. This study investigated the effects of a personalized rehabilitation treatment, exposure in vivo, which aimed to help people with painful diabetic neuropathy to overcome their fears, so that they could become more active in daily life and improve their quality of life. Slight improvements in physical activity and disability were seen. There were no changes in quality of life. The results of this study should be interpreted with caution, as there was a large number of drop-outs.

Neuroprotective Effects of Milrinone on Experimental Acute Spinal Cord Injury: Rat Model

Spinal cord injury (SCI) disrupts nerve axons with devastating neurological consequences, but there is no effective clinical treatment. The secondary damage mechanism is a mainstay process, and it starts within a few minutes after trauma. We aim to investigate the neuroprotective effects of milrinone on the SCI model. A total of 36 Wistar albino rats, each weighing 300-400 g, were randomly split into 4 groups that received different treatments: in group 1 (sham) (n= 9) control, only a laminectomy was performed; in group 2 (SCI) (n= 9), SCI was imitated after laminectomy; in group 3 (SCI+ saline) (n= 9), physiological saline solution was injected intraperitoneally immediately after the SCI; and in group 4 (SCI+ milrinone), milrinone was administered intraperitoneally on lateral decubitus position immediately after the SCI. Spinal cord contusion was established by the weight-drop technique after laminectomy. Neurological examination scores were recorded, and rats were killed 72 hours later. Serum and spinal cord tissue glutathione peroxidase, total antioxidant status, total oxidant status, 8-hydroxiguanosine, interleukin-6 and interleukin-10 levels, histopathological spinal cord damage score, and apoptotic index were examined and compared between groups. Neurological examination scores were significantly better in the milrinone-treated group compared with groups 2 and 3. SCI significantly increased serum and spinal cord tissue glutathione peroxidase, total oxidant status, 8-hydroxiguanosine, and interleukin-6 levels that were successfully reduced with milrinone treatment. Interleukin-10 and total antioxidant status levels decreased as a result of SCI increased with milrinone treatment. Increased histopathological spinal cord damage score and apoptotic index in groups 2 and 3 significantly decreased in group4. Milrinone could reduce apoptosis and increase anti-inflammatory and antioxidative mediators, thus playing a protective role in secondary nerve injury after SCI in rats.