Abnormal Brain Magnetic Resonance Imaging Findings Research Articles

Correlation between Persistent Serum Lactate Elevation and Brain Magnetic Resonance Imaging Abnormalities in Children with Status Epilepticus

Purpose: This study was conducted to evaluate the correlation between persistent serum lactate elevation and brain magnetic resonance imaging (MRI) in children with status epilepticus (SE). Methods: In this prospective analytical study, serum lactate levels were measured 24 hours after episodes of SE, and brain MRI was performed within 7 to 14 days after SE termination. MRI abnormalities were classified as acute encephalopathy (AE) grade I to III. The Kruskal-Wallis test was utilized for statistical analysis. Results: The study included 42 participants with SE, of whom 85.70% were boys, with a mean age of 4.94 years. Viral encephalitis was the most common diagnosis, accounting for 47.60% of cases. Elevated serum lactate levels were detected in 71.40% of patients, and approximately 47.60% exhibited abnormal MRI findings consistent with AE grade I. The median serum lactate levels for AE grades I, II, and III were 1.50, 3.10, and 0.78 mmol/L, respectively. Two patients died, and 66.70% experienced neurologic sequelae. A significant correlation (<i>P</i>=0.021) was observed between persistent serum lactate elevation and abnormal brain MRI findings. Conclusion: In children with SE, AE grade I was the most common neuroimaging pattern observed, and persistently elevated serum lactate level was correlated with abnormal brain MRI findings.

Magnetic Resonance Imaging Findings and Genetic Testing Results in Children With Congenital Corneal Opacities

This study investigates brain and globe abnormalities identified on magnetic resonance imaging (MRI) in children with congenital corneal opacities (CCO). Retrospective cohort study. Clinical notes, radiology records, and genetic testing results were reviewed for patients diagnosed with corneal opacification within the first 6 months of life at a tertiary referral academic center between August 2008 and January 2018. Ocular findings, systemic anomalies, neuroimaging, and genetic testing results were summarized. A total of 135 patients presenting at age 1 day to 12 years (mean age, 1 year) were identified. Children with bilateral CCO were more likely to have systemic disease (P=0.018). Of the entire cohort, 43 (31.8%) patients received MRI, of whom 27 (62.8%) had abnormal brain findings and 30 (69.7%) had abnormal orbital findings. The most common abnormal brain findings were ventriculomegaly (n=16, 59.2%) and corpus callosum abnormalities (n=10, 37.0%) followed by brainstem/pons anomalies (n=5, 18.5%), and cerebellar anomalies (n=2, 7.4%). Abnormal brain MRI findings were associated with the presence of neurologic (P=.003) and craniofacial (P=.034) disease. A total of 44 (32.1%) patients underwent genetic testing, of whom 29 (65.9%) had pathogenic results. More than 60% of the children with CCO who underwent MRI had abnormal brain and orbit findings that were correlated with significant neurologic disease. Furthermore, almost two-thirds of patients with CCO who underwent genetic testing had pathogenic results. These data demonstrate the value of systemic workup in children with CCO, and highlight the role of ophthalmologists in facilitating the diagnosis of systemic comorbidities associated with CCO.

Brain magnetic resonance imaging findings among children with epilepsy in two urban hospital settings, Kampala-Uganda: a descriptive study

BackgroundEpilepsy is one of the most common neurological conditions in children worldwide. Its presentation is heterogeneous, with diverse underlying aetiology, clinical presentation, and prognosis. Structural brain abnormalities are among the recognized causes of epilepsy. Brain Magnetic Resonance Imaging (MRI) is the imaging modality of choice for epilepsy workup.We aimed to determine the prevalence and describe the structural abnormalities identified in the brain MRI studies performed on children with epilepsy from two urban hospitals in Kampala, Uganda.MethodsThis was a cross-sectional descriptive study performed at two urban hospital MRI centres. The study population was 147 children aged 1 day to 17 years with confirmed epilepsy. Brain MRI was performed for each child and a questionnaire was used to collect clinical data.ResultsThe prevalence of structural abnormalities among children with epilepsy was 74.15% (109 out of 147). Of these, 68.81% were male, and the rest were female. Among these, the majority, 40.14% (59 of 144) were aged 1 month to 4 years. Acquired structural brain abnormalities were the commonest at 69.22% with hippocampal sclerosis (HS) leading while disorders of cortical development were the most common congenital causes.An abnormal electroencephalogram (EEG) was significant for brain MRI abnormalities among children with epilepsy with 95% of participants with an abnormal EEG study having epileptogenic structural abnormalities detected in their brain MRI studies.Conclusion and recommendationTwo-thirds of children with epilepsy had structural brain abnormalities. Abnormal activity in the EEG study was found to positively correlate with abnormal brain MRI findings. As such, EEG study should be considered where possible before MRI studies as a determinant for children with epilepsy who will be having imaging studies done in the Ugandan setting.

Radiological characteristics of neuromyelitis optica spectrum disorder in Egypt

AbstractBackgroundNeuromyelitis optica spectrum disorder (NMOSD) is an autoimmune demyelinating disease of the central nervous system, which mainly affects the optic nerves and the spinal cord. Magnetic resonance imaging (MRI) plays an important role in the diagnosis of NMOSD based on the 2015 consensus criteria.MethodsThe objective of this study was to describe the MRI characteristics of NMOSD patients in Egypt. This were a prospective, observational study on patients fulfilling the 2015 diagnostic criteria of NMOSD. Patients were recruited consecutively from the Neurology, Psychiatry and Neurosurgery hospital, Assiut University Hospitals, Egypt from March 2019 until August 2020. Data on demographics, clinical and laboratory features were collected. MRI scan results were reviewed for each patient and compared according to Aquaporin4‐seropositivity.ResultsNinety patients fulfilled NMOSD diagnostic criteria. The mean age at onset was 30.7±11.8 with a female predominance of 3:1. AQP4 antibodies were positive in 77/90 patients (85.6%). Seventy‐two patients (80%) had acute myelitis, 44 cases (61.1%) had longitudinally extensive transverse myelitis (LETM) and 61 cases (87.1%) had a central position in the cord. Bright spotty lesions (BSL) were found in 44 cases (61.1%). The most common site of cord involvement was the cervical region. Abnormal MRI brain scans were found in 81.1% of patients. No statistically significant differences could be found between the two groups.ConclusionThere was a high frequency of abnormal spine and brain MRI findings in the Egyptian population. Even though no specific patterns were typical for AQP4‐specific serostatus, certain findings may suggest NMOSD diagnosis and warrant AQP4‐IgGs testing.

Coexistence of neuronal intranuclear inclusion disease and amyotrophic lateral sclerosis: an autopsy case

BackgroundNeuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease. Pathologically, it is characterized by eosinophilic hyaline intranuclear inclusions in the cells of the visceral organs as well as central, peripheral, and autonomic nervous system cells. Recently, a GGC repeat expansion in the NOTCH2NLC gene has been identified as the etiopathological agent of NIID. Interestingly, this GGC repeat expansion was also reported in some patients with a clinical diagnosis of amyotrophic lateral sclerosis (ALS). However, there are no autopsy-confirmed cases of concurrent NIID and ALS.Case presentationA 60-year-old Taiwanese woman reported a four-month history of progressive weakness beginning in the right foot that spread to all four extremities. She was diagnosed with ALS because she met the revised El Escorial diagnostic criteria for definite ALS with upper and lower motor neuron involvement in the cervical, thoracic, and lumbosacral regions. She died of respiratory failure at 22 months from ALS onset, at the age of 62 years. Brain magnetic resonance imaging (MRI) revealed lesions in the medial part of the cerebellar hemisphere, right beside the vermis (paravermal lesions). The subclinical neuropathy, indicated by a nerve conduction study (NCS), prompted a potential diagnosis of NIID. Antemortem skin biopsy and autopsy confirmed the coexistence of pathology consistent with both ALS and NIID. We observed neither eccentric distribution of p62-positive intranuclear inclusions in the areas with abundant large motor neurons nor cytopathological coexistence of ALS and NIID pathology in motor neurons. This finding suggested that ALS and NIID developed independently in this patient.ConclusionsWe describe a case of concurrent NIID and ALS discovered during an autopsy. Abnormal brain MRI findings, including paravermal lesions, could indicate the coexistence of NIID even in patients with ALS showing characteristic clinical phenotypes.

Short-term developmental outcomes in neonates born to mothers with COVID-19 from Wuhan, China.

BackgroundCoronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is an emerging disease. The consequences of SARS-CoV-2 exposure in infants remain unknown. Therefore, this study aims to investigate whether neonates born to mothers with COVID-19 have adverse brain development.MethodsThis multicenter observational study was conducted at two designated maternal and children’s hospitals in Hubei Province, mainland China from February 1, 2020 to May 15, 2020. Neonates born to mothers with COVID-19 were enrolled. Brain magnetic resonance imaging (MRI) findings, and volumes of grey and white matters, and physical growth parameters were observed at 44 weeks corrected gestational age.ResultsOf 72 neonates born to mothers with COVID-19, 8 (11%) were diagnosed with COVID-19, 8 (11%) were critically ill, and no deaths were reported. Among the eight neonates that underwent brain MRI at corrected gestational age of 44 weeks, five neonates were diagnosed with COVID-19. Among these five neonates, three presented abnormal MRI findings including abnormal signal in white matter and delayed myelination in newborn 2, delayed myelination and brain dysplasia in newborn 3, and abnormal signal in the bilateral periventricular in newborn 5. The other three neonates without COVID-19 presented no significantly changes of brain MRI findings and the volumes of grey matter and white matter compared to those of healthy newborns at the equivalent age (P > 0.05). Physical growth parameters for weight, length, and head circumference at gestational age of 44 weeks were all above the 3rd percentile for all neonates.ConclusionsSome of the neonates born to mothers with COVID-19 had abnormal brain MRI findings but these neonates did not appear to have poor physical growth. These findings may provide the information on the follow-up schedule on the neonates exposed to SARS-CoV-2, but further study is required to evaluate the association between the abnormal MRI findings and the exposure to SARS-CoV-2.Supplementary InformationThe online version contains supplementary material available at 10.1007/s12519-021-00426-z.

The prognosis of brain magnetic resonance imaging injury pattern for outcomes of hypothermia-treated infants.

Magnetic resonance imaging (MRI) can be a tool that allows the observation of structural injury patterns after cooling. The aim of this study was to determine the early pattern of brain injury in the MRIs of infants with hypoxic ischemic encephalopathy (HIE) after cooling and to search for any clinical factors related to abnormal MRI findings.The study retrospectively recruited 118 infants who were treated with therapeutic hypothermia (TH) between 2013 and 2016.Forty-three patients had normal brain MRI, and 75 had abnormal brain MRI findings. The TH-treated infants with abnormal brain MRI readings showed significantly more clinical seizures and the use of additional antiepileptic drugs (AEDs) than the normal MRI group. As a long-term outcome, more lesions in the basal ganglia and thalamus, posterior limb of internal capsule, or severe white matter lesions were associated with abnormal neurodevelopmental outcomes at 18 to 24 months of age.A higher frequency of clinical seizures and AED use were related to abnormal brain injury on MRI. A significant risk for poor long-term outcomes was found in the abnormal brain MRI group.

Is Brain MRI Needed in Diagnostic Evaluation of Mild Intellectual Disability?

The purpose of our study was to suggest an imaging strategy and guidelines for the selection of the children with mild intellectual disability (ID) for magnetic resonance imaging (MRI), to avoid unnecessary imaging. The brain MRIs and patient reports of 471 children were reviewed for the imaging findings and ID severity. The correlation between the clinical and brain MRI findings was analyzed in the 305 children with mild ID. Thirty-eight (12.5%) of the children with mild ID had significant abnormal brain MRI findings. Thirty-five of these had other neurological symptoms or diseases in addition to ID, which were an indication for brain MRI. In the logistic regression analysis, seizures (in patients without an epilepsy diagnosis), epilepsy, movement disorders, dysmorphia, encephalitis, traumatic brain injury, and abnormal head size were statistically significant symptoms or comorbidities associated with abnormal MRI findings. Only three children (1.0%) with mild ID had a significant MRI finding without any other clinical symptoms or disease. Routine MRI in children with mild ID without specific neurological symptoms, dysmorphic features, or related diseases is not suggested for revealing an etiology of mild ID. Since children with ID usually need to be sedated for MRI, routine imaging in the diagnostic evaluation of mild ID should be carefully considered. Clinical examination, other symptoms, and related diseases should be carefully assessed to decide the need for MRI.

Neurodevelopmental impairment at 3 years of age after fetoscopic laser surgery for twin-to-twin transfusion syndrome.

Data on neurodevelopmental outcomes of children surviving after fetoscopic laser surgery (FLS) for twin-to-twin transfusion syndrome (TTTS) are scarce. We retrospectively investigated children surviving after FLS for TTTS at 16 to 26 weeks' gestation between 2003 and 2014. Children were evaluated by standardized neurologic examinations using the Kyoto Scale 2001 at a corrected age of 3 years ± 6 months. Neurodevelopmental impairment (NDI) was defined as cerebral palsy (CP), bilateral blindness, bilateral deafness or a developmental quotient (DQ) < 70 points. Brain magnetic resonance imaging (MRI) was performed at term-equivalent age. A total of 188 children from 110 twin pregnancies were evaluated. NDI was detected in 16/188 (8.5%) children, including six cases of CP (3.2%). No children had bilateral blindness or deafness. An earlier gestational age at delivery was associated with a higher incidence of NDI (P < .001). Abnormal brain MRI findings were detected in 9/16 (56%) of children with NDI, including 6/6 (100%) with CP. The incidence of NDI in children following FLS at 3 years old was 8.5%. Prematurity is a strong risk factor for NDI. Brain MRI may predict the development of CP.

Probable progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome with immunosuppressant dose reduction following lung transplantation: a case report and literature review

BackgroundProgressive multifocal leukoencephalopathy (PML) is a rapidly developing demyelinating disease in the cerebral white matter and is often caused by JC polyomavirus (JCV). PML after lung transplantation is rare and has a poor prognosis, with no established therapies. Reducing the patient’s immunosuppressant doses, thereby restoring immunity, could be used to treat PML. However, some patients develop immune reconstitution inflammatory syndrome (IRIS) with this treatment, an immune-induced inflammatory response to JCV that results in serious neuronal damage. We herein report a case of a 60-year-old female who suffered from PML 5 years after lung transplantation, had worsened brain lesions thought to be related to PML-IRIS at the time of immunosuppressant reduction, and missed treatment opportunities.Case presentationA 60-year-old female developed PML 5 years after lung transplantation. Fluid-attenuated inversion recovery and diffusion-weighted brain magnetic resonance imaging (MRI) revealed multiple high-signal lesions, mainly in the cerebral white matter. Polymerase chain reaction found 0.32 million copies/mL of JCV in the cerebrospinal fluid. Thus, she was given a diagnosis of PML. Mycophenolate mofetil and tacrolimus dosages were reduced, and CD4-positive cell counts and the blood concentration of each immunosuppressant were monitored. Mefloquine was also orally administered at a daily dose of 275 mg for 3 days and was then administered at a dose of 275 mg per week. Although the patient’s CD4-positive cell counts increased and her immune system recovered, her symptoms and brain MRI findings worsened. We suspected PML progression or a transition to PML-IRIS. Steroid pulse therapy to suppress the inflammatory lesions was not possible but was retrospectively indicated. The patient rapidly began to exhibit akinetic mutism and died 4 months after the onset of neurologic symptoms.ConclusionsWhen neurologic symptoms and abnormal brain MRI findings are noted during immune recovery, it is often difficult to distinguish between progressed PML and PML-IRIS. However, the pathogenesis of brain lesions usually involves inflammation and immune-reactive mechanisms for JCV. Steroid pulse therapy, which can reduce inflammation, should thus be administered in organ transplantation cases with differential diagnoses including PML-IRIS.

Utility of brain MRI in children with sleep-disordered breathing.

To investigate the utility of a brain magnetic resonance imaging (MRI) in children with sleep-disordered breathing (SDB), classified as isolated obstructive sleep apnea (OSA) in the absence of adenotonsillar hypertrophy, persistent OSA following adenotonsillectomy, isolated central sleep apnea (CSA) of unclear etiology, OSA with coexisting CSA of unclear etiology, or unexplained nocturnal hypoventilation (NH). Retrospective chart review of polysomnography (PSG) and brain MRI data. Children with PSG evidence of SDB, as described above, and who subsequently had their first brain MRI, were included. PSG, MRI data, and subsequent interventions were recorded. A total of 59 of 6,087 (1%) children met inclusion criteria. Of those, 28 of 59 (47%) were nonsyndromic children and 31 of 59 (53%) were syndromic children with an underlying medical disorder. Abnormal brain MRI findings were observed in 19 of 59 (32%) children, where eight of 19 (42%) were nonsyndromic and 11 of 19 (58%) were syndromic. Abnormal brain MRI findings were most common in syndromic children with combined OSA and CSA without adenotonsillar hypertrophy. Isolated OSA was also a common PSG finding associated with an abnormal brain MRI. Of the nonsyndromic children with an abnormal brain MRI, the most common abnormal brain MRI finding was Chiari malformation (CM), observed in 88% of the group. A brainstem tumor was identified in one nonsyndromic child. Interventions following brain MRI included neurosurgery, chemotherapy, and noninvasive positive pressure ventilation (NiPPV). A brain MRI is an important diagnostic tool in syndromic and nonsyndromic children, especially in children with either isolated OSA or combined OSA and CSA without a clear etiology. 4. Laryngoscope, 2016 127:513-519, 2017.

Serum Lactate, Brain Magnetic Resonance Imaging and Outcome of Neonatal Hypoxic Ischemic Encephalopathy after Therapeutic Hypothermia

Serum lactate was used to predict the severity and outcome of neonatal hypoxic ischemic encephalopathy (HIE) before the era of therapeutic hypothermia (TH). There is no report on neurodevelopment (ND) outcome of neonates with HIE treated with TH in Taiwan. Between April 2011 and December 2012, newborn infants admitted to Chang Gung Memorial Hospital (CGMH), with gestational age > 35 weeks and birth weight ≥ 1800 g, who had acute perinatal events, evidence of significant fetal compromise, and ongoing clinical encephalopathy were prospectively enrolled for TH. Whole body cooling method was used to maintain the affected neonate's esophageal temperature at 33.5 ± 0.5 °C for 72 hours. Demographic data were recorded and hemogram, biochemical parameters, serum lactate, and creatine kinase (CK) were measured as well. Brain magnetic resonance imaging (MRI) was performed between 7 and 14 days of life. ND outcome of infants was evaluated by Bayley Scales of Infant Development, third edition (BSID-III) at 24 months of corrected age. Poor ND (PND) outcome was defined as infants surviving with either disability or ND delay. Seventeen patients were enrolled. Fifty-nine percent of babies (10/17) were born through cesarean section and 77% of babies (13/17) were transferred from outside hospitals. Six babies were moderate HIE and 11 babies were severe HIE. Among the 14 surviving patients, eight infants had PND outcome. There was no difference in demographic data between infants with and without PND. Serum level of lactate (mg/dL) after 72 hours of TH was higher (35.6 vs. 13.8, p = 0.042) in infants with PND. Neonates with abnormal brain MRI findings were also associated with PND (p = 0.01). This is the first report on ND outcome of neonates with HIE treated with TH in Taiwan. Higher serum level of lactate following TH and abnormal results of brain MRI are associated with poor ND outcome.

Subacute Sclerosing Panencephalitis Case Presenting with Cortical Blindness: Early Diagnosis with MRI and MR Spectroscopy

subacute sclerosing panencephalitis (sspe), chronic progressive inflammatory disorder of the central nervous system, is associated with the presence of persistent and aberrant measles virus infection. The diagnosis is based on characteristic clinical features, periodic electroencephalographic complexes, and elevated measles antibody titer in cerebrospinal fluid. Ocular changes occur approximately in 50 % of cases, but rarely presenting as a first symptom [1]. Visual system abnormalities in SSPE include retinal vasculitis, maculopathy, chorioretinitis, optic neuritis, papillitis, papilledema, optic atrophy, nystagmus, cranial nerve palsies and transient hemianopia, and acute vision loss because of cortical involvement. The most common ocular manifestations are because of macular retinitis and macular pigment disturbances [2–4]. In the beginning, during brain conventional magnetic resonance imaging (MRI) findings are normal, though magnetic resonance spectroscopy (MRS) of SSPE patient may show some abnormalities [1, 5]. Our patient’s neurologic and fundoscopic examination and laboratory findings were normal except loss of visual acuity and abnormal brain MRI and MRS findings. Herein, we report a case of cortical blindness diagnosed as SSPE with the help of neuroimaging without the classical clinical presentation of central nervous system involvement initially.

Letters to the Editor

Letters to the Editor

Brain Magnetic Resonance Imaging After High-Dose Chemotherapy and Radiotherapy for Childhood Brain Tumors

Brain necrosis or other subacute iatrogenic reactions has been recognized as a potential complication of radiotherapy (RT), although the possible synergistic effects of high-dose chemotherapy and RT might have been underestimated. We reviewed the clinical and radiologic data of 49 consecutive children with malignant brain tumors treated with high-dose thiotepa and autologous hematopoietic stem cell rescue, preceded or followed by RT. The patients were assessed for neurocognitive tests to identify any correlation with magnetic resonance imaging (MRI) anomalies. Of the 49 children, 18 (6 of 25 with high-grade gliomas and 12 of 24 with primitive neuroectodermal tumors) had abnormal brain MRI findings occurring a median of 8 months (range, 2-39 months) after RT and beginning to regress a median of 13 months (range, 2-26 months) after onset. The most common lesion pattern involved multiple pseudonodular, millimeter-size, T1-weighted unevenly enhancing, and T2-weighted hyperintense foci. Four patients with primitive neuroectodermal tumors also had subdural fluid leaks, with meningeal enhancement over the effusion. One-half of the patients had symptoms relating to the new radiographic findings. The MRI lesion-free survival rate was 74%+/-6% at 1 year and 57%+/-8% at 2 years. The number of marrow ablative courses correlated significantly to the incidence of radiographic anomalies. No significant difference was found in intelligent quotient scores between children with and without radiographic changes. Multiple enhancing cerebral lesions were frequently seen on MRI scans soon after high-dose chemotherapy and RT. Such findings pose a major diagnostic challenge in terms of their differential diagnosis vis-à-vis recurrent tumor. Their correlation with neurocognitive results deserves further investigation.