Intracranial Hypertension in Multisystem Inflammatory Syndrome in Children.

Multisystem inflammatory syndrome in children (MIS-C) is characterized by fever and multiorgan system dysfunction. Neurologic complications of MIS-C are not well described. We present 4 patients with MIS-C who had intracranial hypertension and discuss the unique management considerations when this occurs concurrently with significant myocardial dysfunction.

Type I AV fistula of the thoracic spinal cord.

Type I AV fistulas of the spinal cord are exceedingly rare. The average age at diagnosis is 50. Clinical presentation is often very non-specific, and sensory deficits and sphincter dysfunction may also occur. Neurological deterioration is generally gradual. Thus, failure to diagnosis frequently results in permanent disability. A 22-year-old female complained of a "muscle spasm" in the midline thoracic area with no history of trauma or prior occurrence. She also experienced bilateral lower extremity weakness/numbness and perianal anesthesia. She is a healthy female with no medical problems. Exam revealed lower extremity motor function of 1/5 as well as diminished sensation. A foley catheter was placed for urinary retention. The remainder of the neurological exam was normal. MRI demonstrated a lesion at the fourth thoracic level with significant cord compression. The patient was taken emergently to the operating room by neurosurgery. This case demonstrates a rare disorder occurring in a 22-year old female, far younger than the typical 50-year old patient. Moreover, the lesion was located in the thoracic rather than the typical lumbar cord. Symptoms may be misinterpreted as a peripheral nerve lesion and delay time to diagnosis. Early diagnosis remains critical to prevent permanent neurologic sequelae. AV fistula should remain high on the differential of patients presenting with back pain and focal neurologic complaints.

Evaluation and treatment of Langerhans cell histiocytosis patients with central nervous system abnormalities: Current views and new vistas.

Central nervous system (CNS) involvement in Langerhans cell histiocytosis (LCH) can include mass lesions of the hypothalamic pituitary axis, choroid plexus, cerebrum, and cerebellum or magnetic resonance imaging (MRI) signal abnormalities of the cerebellum, pons, and basal ganglia. The term neurodegenerative (ND) CNS-LCH has been given to the MRI signal abnormalities and neurologic dysfunction, although initially patients may have no clinical symptoms. Standardized evaluations to better understand the natural history and response to therapy are needed. We propose guidelines for clinical, radiologic, and physiologic tests as a framework for developing the best methods of evaluation, which can then be tested in prospective treatment protocols.

Is eculizumab efficacious in Shigatoxin-associated hemolytic uremic syndrome? A narrative review of current evidence.

Severe complications due to Shigatoxin-associated hemolytic uremic syndrome (STEC-HUS) currently present a serious challenge since no specific treatment for this condition is available. Eculizumab, a terminal complement inhibitor, has been used especially in STEC-HUS patients with severe neurological involvement, but the efficacy remains undetermined. In order to determine its efficacy, we searched the databases Pubmed, Web of Science, Embase, and LiLACS for reports describing outcomes of eculizumab administration in STEC-HUS. We retrieved 11 reports ranging from case reports to cohort studies with the largest study population emanating from the 2011 German outbreak. Outcomes were variable and difficult to interpret in light of the absence of high-quality studies but seemed to point towards potential efficacy of eculizumab if administered early in the course. CONCLUSION: The efficacy of eculizumab in STEC-HUS could not be established nor disproven based on current data, and there is a desperate need for randomized controlled trials. What is known? • Eculizumab has been used in complicated cases of Shigatoxin-associated hemolytic uremic syndrome but the efficacy remains unknown? What is new? • Eculizumab might be efficacious if given early in selected cases of Shigatoxin-associated hemolytic uremic syndrome; however, randomized trials are needed to assess this.

Conservative and surgical treatment of pediatric asymptomatic lumbosacral lipoma: a meta-analysis.

The aim of this study is to compare the outcomes of surgical and conservative treatments of pediatric asymptomatic lumbosacral lipomas, and to address whether the patients can benefit from prophylactic surgeries. The literature reports of surgical and conservative treatments of child asymptomatic lumbosacral lipomas were reviewed and collected, and a meta-analysis of the reports regarding the incidence of sphincter and lower limb dysfunctions was performed. A total of five literatures were collected, containing a total of 403 patients, among which 124 patients received conservative treatments with 32 (25.81%) cases developing neurological dysfunctions during follow-up, and 279 received prophylactic surgical treatments with 30 (10.75%) patients developing neurological dysfunctions in follow-up, the difference being statistically significant (P ≤ 0.05). For pediatric asymptomatic lumbosacral lipomas of the three major subtypes, the limited source of literature so far suggests that prophylactic surgery is superior to conservative strategy in preventing the patients from neurological deterioration. Larger patient cohorts, randomized studies, and longer length of follow-ups are needed for further corroboration.

Rituximab therapy for patients with Langerhans cell histiocytosis-associated neurologic dysfunction.

OBJECTIVE: Since patients with langerhans cell histiocytosis and neurologic dysfunction (LCH-ND) often have incomplete treatment responses we sought a new treatment regimen. Because of clinical benefit from rituximab in multiple sclerosis patients with neurodegeneration, we evaluated its use in patients with LCH-ND. PARTICIPANTS: Eight LCH-ND patients who had failed prior therapies. METHODS: Charts of the 8 patients treated with rituximab were reviewed. Signs/symptoms and MRI responses were assessed. RESULTS: Seven of eight patients experienced some clinical improvement: gait abnormalities and tremors in four children, proprioceptive deficits in 2, and dysarthria/dysphagia in 2. Five of eight patients demonstrated improvement in intellectual/behavioral/psychological symptoms. CONCLUSION: These findings suggest that prospective studies are warranted to define safety and efficacy of rituximab for patients with LCH-ND.

Prediction of 3- to 5-Month Outcomes from Signs of Acute Bilirubin Toxicity in Newborn Infants.

OBJECTIVE: To evaluate the ability of the bilirubin-induced neurologic dysfunction (BIND) score to predict residual neurologic and auditory disability and to document the relationship of BIND score to total serum bilirubin (TSB) concentration. STUDY DESIGN: The BIND score (assessing mental status, muscle tone, and cry patterns) was obtained serially at 6- to 8-hour intervals in 220 near-term and full-term infants with severe hyperbilirubinemia. Neurologic and/or auditory outcomes at 3-5 months of age were correlated with the highest calculated BIND score. The BIND score was also correlated with TSB. RESULTS: Follow-up neurologic and auditory examinations were performed for 145/202 (72%) surviving infants. All infants with severe acute bilirubin encephalopathy (BIND scores 7-9) either died or suffered residual neurologic and auditory impairment. Of 24 cases with moderate encephalopathy (BIND 4-6), 15 (62.5%) resolved following aggressive intervention and were normal at follow-up. Three of 73 infants with mild encephalopathy (BIND scores 1-3) but severe jaundice (TSB ranging 33.5-38 mg/dL; 573-650 µmol/L) had residual neurologic and/or auditory impairment. A BIND score ≥4 had a specificity of 87.3% and a sensitivity of 97.4% for predicting poor neurologic outcomes (receiver operating characteristic analysis). BIND scores trended higher with severe hyperbilirubinemia (r2 = 0.54, P < .005), but 5/39 (13%) infants with TSB ≥36.5 mg/dL (624 µmol/L) had BIND scores ≤3, and normal outcomes at 3-5 months. CONCLUSIONS: The BIND score can be used to evaluate the severity of acute bilirubin encephalopathy and predict residual neurologic and hearing dysfunction.

Quantitative Romberg's test in acute carbon monoxide poisoning treated by hyperbaric oxygen.

OBJECTIVE: The aim of this study was to evaluate whether monitoring of acute carbon monoxide-poisoned (COP) patients by means of quantitative Romberg's test (QR-test) during a hyperbaric oxygen (HBO2) therapy regimen could be a useful supplement in the evaluation of neurological status. METHODS: We conducted a retrospective study (2000-2014) in which we evaluated data containing quantitative sway measurements of acute COP patients (n = 58) treated in an HBO2 regimen. Each patient was tested using QR-test before and after each HBO2 treatment. Data were analyzed using linear mixed models (LMM). In each LMM, sway prior to HBO2 therapy was set as the fixed effect and change in sway after HBO2 therapy was set as the response variable. Patient, treatment number, weight and age were set as random effects for all LMMs. RESULTS: From the LMMs we found that larger values of sway prior to HBO2 produced a negative change in sway. We found no correlation between CO level and sway (P=0.1028; P=0.8764; P=0.4749; P=0.5883). Results showed that loss of visual input caused a significant increase in mean sway (P=0.028) and sway velocity (P⟨0.0001). CONCLUSIONS: The Quantitative Romberg's test is a fast, useful supplement to neurological evaluation and a potential valuable tool for monitoring postural stability during the course of treatment in acute COP patients.

Evaluation of a New Brain Tissue Probe for Intracranial Pressure, Temperature, and Cerebral Blood Flow Monitoring in Patients with Aneurysmal Subarachnoid Hemorrhage.

OBJECTIVE: To evaluate an intraparenchymal probe for intracranial pressure (ICP) and temperature (TEMP) monitoring as well as determination of cerebral hemodynamics using a near-infrared spectroscopy (NIRS) and indocyanine green (ICG) dye dilution method (NIRS-ICP probe). METHODS: The NIRS-ICP probe was applied after aneurysmal subarachnoid hemorrhage if multimodal monitoring was established due to poor neurological condition. ICP and TEMP values were obtained from ventricular catheters and systemic temperature sensors. Repeated NIRS-ICG measurements (2 injections within 30 min) were performed daily for determination of cerebral blood flow (CBF), cerebral blood volume (CBV), and mean transit time of ICG (mttICG). Secondary neurologic dysfunction was defined as brain tissue oxygen tension <20 mmHg and/or lactate/pyruvate ratio >35 obtained from cerebral probing. RESULTS: A total of 128 NIRS-ICG measurements were performed in ten patients. The correlation coefficients between ICP and TEMP values obtained with the NIRS-ICP probe and values from routine monitoring were r = 0.72 and r = 0.96, respectively. The mean values were 30.3 ± 13.6 ml/100 g/min for CBF, 3.3 ± 1.2 ml/100 g for CBV, and 6.8 ± 1.6 s for mttICG. The coefficients of variation from repeated NIRS-ICG measurements were 10.9 % for CBF, 11.7 % for CBV, and 3.8 % for mttICG. The sensitivity for detection of secondary neurologic dysfunction was 85 % and the specificity 83 % using a CBF-threshold of 25 ml/100 g/min. CONCLUSION: Multimodal monitoring using the NIRS-ICP probe is feasible with high reproducibility of measurement values and the ability to detect secondary neurologic dysfunction. No safety concerns exist for the routine clinical use of the NIRS-ICP probe.

Monitoring the Response of Hyperbilirubinemia in the Mouse Brain by In Vivo Bioluminescence Imaging.

Increased levels of unconjugated bilirubin are neurotoxic, but the mechanism leading to neurological damage has not been completely elucidated. Innovative strategies of investigation are needed to more precisely define this pathological process. By longitudinal in vivo bioluminescence imaging, we noninvasively visualized the brain response to hyperbilirubinemia in the MITO-Luc mouse, in which light emission is restricted to the regions of active cell proliferation. We assessed that acute hyperbilirubinemia promotes bioluminescence in the brain region, indicating an increment in the cell proliferation rate. Immunohistochemical detection in brain sections of cells positive for both luciferase and the microglial marker allograft inflammatory factor 1 suggests proliferation of microglial cells. In addition, we demonstrated that brain induction of bioluminescence was altered by pharmacological displacement of bilirubin from its albumin binding sites and by modulation of the blood-brain barrier permeability, all pivotal factors in the development of bilirubin-induced neurologic dysfunction. We also determined that treatment with minocycline, an antibiotic with anti-inflammatory and neuroprotective properties, or administration of bevacizumab, an anti-vascular endothelial growth factor antibody, blunts bilirubin-induced bioluminescence. Overall the study supports the use of the MITO-Luc mouse as a valuable tool for the rapid response monitoring of drugs aiming at preventing acute bilirubin-induced neurological dysfunction.

New frontiers in aortic therapy: focus on deep hypothermic circulatory arrest.

There is currently a paradigm shift in the conduct of adult aortic arch repair. Although deep hypothermic circulatory arrest has been the classic perfusion platform for adult aortic arch repair, recent developments have challenged this aortic arch paradigm. There has been a gradual clinical drift towards moderate, and even mild, hypothermic circulatory arrest combined with antegrade cerebral perfusion. This paradigm shift appears to be associated with equivalent clinical outcomes, and in certain settings, with improved outcomes. The advent of endovascular therapy has challenged even further the concept that circulatory arrest is required for adult aortic arch repair. These dramatic advances have resulted in the emergence of an international aortic arch surgery study group that aims to advance this dynamic field through consensus statements, meta-analysis, clinical database analysis, prospective registries, and randomized controlled trials.

Acupuncture, an effective treatment for post-stroke neurologic dysfunction.

Stroke episodes represent a significant subset of cerebrovascular diseases globally, often resulting in diverse neurological impairments such as hemiparesis, spasticity, dysphagia, sensory dysfunction, cognitive impairment, depression, aphasia, and other sequelae. These dysfunctions markedly diminish patients' quality of life and impose substantial burdens on their families and society. Consequently, the restoration of neurological function post-stroke remains a primary objective of clinical treatment. Acupuncture, a traditional Chinese medicine technique, is endorsed by the World Health Organization (WHO) for stroke treatment due to its distinct advantages in managing cerebrovascular diseases, including ischemic stroke. Numerous clinical studies have substantiated the efficacy of acupuncture in ameliorating neurological dysfunctions following stroke. This review systematically examines the improvements in post-stroke neurological dysfunction attributable to acupuncture treatment and elucidates potential mechanisms of action proposed in recent years. Additionally, this article aims to present novel therapeutic concepts and strategies for the clinical management of post-stroke neurological dysfunction.

Effects of temperature on transient neurologic dysfunction after total arch replacement.

OBJECTIVES: The relationship between cooling status during aortic surgery with hypothermic circulatory arrest and postoperative neurologic dysfunction remains unknown. In the present study, we evaluated the effect of cooling status on transient neurologic dysfunction after total arch replacement. METHODS: We studied patients who underwent elective total arch replacement with hypothermic circulatory arrest and antegrade selective cerebral perfusion from December 2011 to January 2021. Changes in tympanic temperature trends recorded during surgery were plotted. Several parameters, including the nadir temperature, cooling speed, and degree of cooling (cooling area, or the area under the curve of inverted temperature trends from cooling to rewarming as calculated by the integral method), were analyzed. The relationships between these variables and transient neurologic dysfunction were evaluated. RESULTS: Transient neurologic dysfunction was observed in 33 (14.5%) of the 228 included patients. In the transient neurologic dysfunction group, the cooling area was larger (2417.3 vs. 1920.8 °C min; P < 0.001) and the cooling speed was higher (0.68 vs. 0.51 °C/min; P < 0.001) than in the non-transient neurologic dysfunction group. A multivariate logistic model revealed that both the cooling area (odds ratio = 1.13 per 100 °C min; P < 0.001) and cooling speed (odds ratio = 3.69 per °C/min; P = 0.041) were independent risk factors for transient neurologic dysfunction. CONCLUSIONS: Both the cooling area, which indicates the degree of cooling, and cooling speed had significant relationships with transient neurologic dysfunction after total arch replacement. Together, these findings indicate that overcooling and rapid cooling may contribute to brain injury.

Erectile dysfunction after acetabular fracture.

Objectives: To determine the rate of erectile dysfunction in male patients who have sustained an acetabular fracture with no previously identified urogenital injury. Design: Cross-sectional survey. Setting: Level 1 Trauma Center. Patients/Participants: All male patients treated for acetabular fracture without urogenital injury. Intervention: The International Index of Erectile Function (IIEF), a validated patient-reported outcome measure for male sexual function, was administered to all patients. Main Outcome Measurements: Patients were asked to complete the International Index of Erectile Function score for both preinjury and current sexual function, and the erectile function (EF) domain was used to quantify the degree of erectile dysfunction. Fractures were classified according the OTA/AO classification schema, fracture classification, injury severity score, race, and treatment details, including surgical approach were collected from the database. Results: Ninety-two men with acetabular fractures without previously diagnosed urogenital injury responded to the survey at a minimum of 12 months and an average of 43 ± 21 months postinjury. The mean age was 53 ± 15 years. 39.8% of patients developed moderate-to-severe erectile dysfunction after injury. The mean EF domain score decreased 5.02 ± 1.73 points, which is greater than the minimum clinically important difference of 4. Increased injury severity score and associated fracture pattern were predictive of decreased EF score. Conclusion: Patients with acetabular fractures have an increased rate of erectile dysfunction at intermediate-term follow-up. The orthopaedic trauma surgeon treating these injuries should be aware of this as a potential associated injury, ask their patients about their function, and make appropriate referrals. Level of Evidence: III.

Cognitive Impairment Is Associated with AMPAR Glutamatergic Dysfunction in a Mouse Model of Neuronal Methionine Synthase Deficiency.

Impairment of one-carbon metabolism during pregnancy, either due to nutritional deficiencies in B9 or B12 vitamins or caused by specific genetic defects, is often associated with neurological defects, including cognitive dysfunction that persists even after vitamin supplementation. Animal nutritional models do not allow for conclusions regarding the specific brain mechanisms that may be modulated by systemic compensations. Using the Cre-lox system associated to the neuronal promoter Thy1.2, a knock-out model for the methionine synthase specifically in the brain was generated. Our results on the neurobehavioral development of offspring show that the absence of methionine synthase did not lead to growth retardation, despite an effective reduction of both its expression and the methylation status in brain tissues. Behaviors were differently affected according to their functional outcome. Only temporary retardations were recorded in the acquisition of vegetative functions during the suckling period, compared to a dramatic reduction in cognitive performance after weaning. Investigation of the glutamatergic synapses in cognitive areas showed a reduction of AMPA receptors phosphorylation and clustering, indicating an epigenomic effect of the neuronal deficiency of methionine synthase on the reduction of glutamatergic synapses excitability. Altogether, our data indicate that cognitive impairment associated with methionine synthase deficiency may not only result from neurodevelopmental abnormalities, but may also be the consequence of alterations in functional plasticity of the brain.

Intranasal Orexin After Cardiac Arrest Leads to Increased Electroencephalographic Gamma Activity and Enhanced Neurologic Recovery in Rats.

OBJECTIVES: Prolonged cardiac arrest is known to cause global ischemic brain injury and functional impairment. Upon resuscitation, electroencephalographic recordings of brain activity begin to resume and can potentially be used to monitor neurologic recovery. We have previously shown that intrathecal orexin shows promise as a restorative drug and arousal agent in rodents. Our goal is to determine the electrophysiology effects of orexin in a rodent model of asphyxial cardiac arrest, focusing on the electroencephalographic activity in the gamma and super-gamma bands (indicative of return of higher brain function). DESIGN: Experimental animal study. SETTING: University-based animal research laboratory. SUBJECTS: Adult male Wistar rats. INTERVENTIONS: In an established model of asphyxial cardiac arrest (n = 24), we treated half of Wistar rats with orexin administered intranasally by atomizer 30 minutes post return of spontaneous circulation in one of two dose levels (10 and 50 µM); the rest were treated with saline as control. Continuous electroencephalographic recording was obtained and quantitatively analyzed for the gamma fraction. Gamma and high-frequency super-gamma band measures were compared against clinical recovery according to Neuro-Deficit Score. MEASUREMENTS AND MAIN RESULTS: Compared with the control cohort, the high-dose orexin cohort showed significantly better Neuro-Deficit Score 4 hours after return of spontaneous circulation (55.17 vs 47.58; p < 0.02) and significantly higher mean gamma fraction (0.251 vs 0.177; p < 0.02) in cerebral regions surveyed by rostral electrodes for the first 170 minutes after administration of orexin. CONCLUSIONS: Our findings support early and continuous monitoring of electroencephalography-based gamma activity as a marker of better functional recovery after intranasal administration of orexin as measured by Neuro-Deficit Score in an established animal model of asphyxial cardiac arrest.

Laminectomy with instrumented fusion vs. laminoplasty in the surgical treatment of cervical ossification of the posterior longitudinal ligament: A multicenter retrospective study.

Laminectomy with instrumented fusion (LF) has demonstrated better prevention of ossification of posterior longitudinal ligament (OPLL) growth compared to laminoplasty (LP). There remains uncertainty, however, as to which surgical approach is more beneficial with respect to clinical outcomes and complications. We retrospectively reviewed 273 cervical OPLL patients of more than 3 levels, from the two institutions' databases, who underwent LF or LP between January 1998 and January 2016. Each 273 patient (85 with LF, 188 with LP) was assessed for postoperative neurologic and radiologic outcomes, complications and reoperations. The mean length of follow-up was 40.11 months. There were baseline differences between cohorts. Overall, postoperative JOA recovery rate at last follow up was significantly better in the LP group with similar improvement in visual analog neck score. Postoperative C2-7 Cobb angle was decreased compared to baseline for both LF and LP cohorts, but there was no significant difference between groups. Complications occurred in 19 (22.35%) LF patients, and 11 (5.85%) LP patients, with higher incidence of C5 palsy and instrumentation failure in the LF group. Four LF patients (4.71%) and five LP patients (2.66%) underwent reoperation during the follow up period.

Postural neurologic deficits after decompressive craniectomy: A case series of sinking skin flap syndrome in traumatic brain injury.

BACKGROUND: Hemicraniectomy to manage raised intracranial pressure following traumatic brain injury (TBI) has improved survival but may increase the incidence of Sinking Skin Flap Syndrome (SSFS). SSFS is a clinical syndrome in which patients with craniectomy develop objective neurologic abnormalities due to the pressure of the atmosphere on the unprotected brain, often presenting with postural headaches and neurologic deficits that localize to the craniectomy site. Previously thought to be a rare complication of craniectomy after TBI, evidence suggests SSFS is under-recognized. OBJECTIVE: To describe the clinical and radiographic features leading to diagnosis and the impact of temporizing and definitive management of SSFS on outcomes in inpatients with moderate/severe TBI. METHODS: Two patients' symptoms, qualitative behaviour observation, physical and cognitive outcome measures, and neuroimaging pre- and post-temporizing measures and cranioplasty are presented. RESULTS: Both patients demonstrated partial improvements with temporizing measures and substantial improvements in functional, cognitive, physical, and rehabilitation outcomes from the cranioplasty and resolution of SSFS. CONCLUSIONS: Rehabilitation care providers are critical to the timely diagnosis and management of SSFS, including the use of temporizing measures and advocacy for definitive treatment with cranioplasty. These cases highlight the diverse clinical presentations and importance of SSFS diagnosis to improve patient outcomes.

Neurologic Dysfunction Associated With Mechanically Assisted Crevice Corrosion and Elevated Cobalt Ion Levels After Total Hip Arthroplasty.

Adverse local tissue reactions secondary to mechanically assisted crevice corrosion (MACC) at the trunnion is a complication of total hip arthroplasty known to cause local soft-tissue damage. However, what is not as well appreciated is that MACC in metal-on-polyethylene (MOP) articulations can lead to cobalt ion serum elevations with associated neurological dysfunction just as in metal-on-metal articulations. We report a compelling case for the association of neurologic dysfunction tied to metal ion elevations secondary to MACC at two distinct MOP tapers in a 58-year-old intensive care unit nurse with two hips implanted 3 years apart. This report further raises awareness about the potential of MACC-generated elevated ion levels to produce neurological symptoms that might otherwise be overlooked in patients with MOP articulations.

The spectrum of bilirubin neurotoxicity in term and near-term babies with hyperbilirubinemia: Does outcome improve with time?

BACKGROUND: While neonatal jaundice is generally a common benign condition; severe hyperbilirubinemia has a devastating potential for brain injury. AIM: To detect the impact of severe neonatal hyperbilirubinemia on motor and mental development and its progress over time in the first year of life using the Bayley scales of infant development (BSID) II. STUDY DESIGN AND PATIENTS: 177 term/near-term infants admitted for neonatal hyperbilirubinemia to the NICU of Cairo University Children's Hospital were enrolled. Clinical examination, BIND score and laboratory tests were performed at admission. Neurodevelopmental assessment using BSIDΙΙ was performed at 3 months for 147/177 neonates, and at 6 months and 12 months for 139/177 neonates. Auditory brainstem evoked potential was recorded at 3 months of age and repeated if abnormal. OUTCOME MEASURES: Psychomotor (PDI) and mental developmental indices (MDI) using BSIDII. Auditory impairment using Auditory Brainstem Response (ABR). RESULTS: TSB levels ranged from 10 to 63 mg/dL (179.6-1077 µmol/L) with a mean of 25.52 ±â€¯6.5 mg/dL (436 ±â€¯112.9 µmol/L) and BIND scores ranged from 0 to 7. By one year of age, 19/139 patients were affected; 8 had classic kernicterus, 3 had isolated auditory impairment, 1 had severe motor and mild mental delay and 7 had mild motor delay. TSB level and BIND score had positive correlation with auditory impairment and lower scores for PDI (which improved with time) and MDI (which remained stationary). Duration of exposure to hyperbilirubinemia negatively affected neurodevelopmental scores. CONCLUSION: The impact of severe hyperbilirubinemia is mainly on motor and auditory impairment. Mild mental delay was detected by BSIDII in few patients. Neurodevelopmental outcome improves over time.