A Girl from Qatar with Post-Infantile Acquired Cerebral Palsy Caused By Submersion Injury: A Rare Etiology and a Therapeutic-Challenge.

Background: Cerebral palsy is a heterogeneous disorder that can cause a lifelong disability that is associated with a non-progressive damage in the brain. It is commonly caused by antenatal, perinatal, early postnatal and neonatal conditions. However, post-neonatal cases of acquired cerebral palsy have also been reported, and were commonly caused by infection. Patients and Methods: The family of a girl from Qatar, who developed severe cerebral palsy caused by submersion injury, consulted us about the possible therapies for her condition. Clinical picture and brain imaging abnormalities are described, and the relevant literatures were reviewed with the aim of suggesting possible evidence-based therapies. Results: At the age of 23 months, a previously healthy girl developed anoxic encephalopathy after experiencing submersion injury. MRI showed evidence of significant hypoxic ischemic injury primarily affecting the deep grey matter, hippocami, mid-brain and the posterior cortex. EEG showed diffuse slowness of cerebral activity and diffuse attenuation of the background without no epileptic abnormalities suggesting diffuse encephalopathy resulting from diffuse cortical injury. At the about age of three and half years, her family consulted us about her condition as she was still showing no awareness to the environment, showing no significant spontaneous movements. She had poor head control. Unable to sit or stand alone, and had a flexed posture. She was on levetiracetam (Keppra), diazepam, and baclofen 30 mg daily. She was still having tracheotomy, and was fed through gastrostomy tube. Conclusion: In this paper, the rare occurrence of severe post-infantile cerebral palsy is described. Emphasis is made on the possibility of using evidence-based multi-factorial therapies in cerebral palsy.

A Girl from Qatar with Post-Infantile Acquired Cerebral Palsy Caused By Submersion Injury: A Rare Etiology and a Therapeutic Challenge

Background: Cerebral palsy is a heterogeneous disorder that can cause a lifelong disability that is associated with a non-progressive damage in the brain. It is commonly caused by antenatal, perinatal, early postnatal and neonatal conditions. However, post-neonatal cases of acquired cerebral palsy have also been reported, and were commonly caused by infection. Patients and methods: The family of a girl from Qatar, who developed severe cerebral palsy caused by submersion injury, consulted us about the possible therapies for her condition. Clinical picture and brain imaging abnormalities are described, and the relevant literatures were reviewed with the aim of suggesting possible evidence-based therapies. Results: At the age of 23 months, a previously healthy girl developed anoxic encephalopathy after experiencing submersion injury. MRI showed evidence of significant hypoxic ischemic injury primarily affecting the deep grey matter, hippocami, mid-brain and the posterior cortex. EEG showed diffuse slowness of cerebral activity and diffuse attenuation of the background without no epileptic abnormalities suggesting diffuse encephalopathy resulting from diffuse cortical injury. At the about age of three and half years, her family consulted us about her condition as she was still showing no awareness to the environment, showing no significant spontaneous movements. She had poor head control. Unable to sit or stand alone, and had a flexed posture. She was on levetiracetam (Keppra), diazepam, and baclofen 30 mg daily. She was still having tracheotomy, and was fed through gastrostomy tube. Conclusion: In this paper, the rare occurrence of severe post-infantile cerebral palsy is described. Emphasis is made on the possibility of using evidence-based multi-factorial therapies in cerebral palsy.

Analysis of deep grey nuclei susceptibility in early childhood: a quantitative susceptibility mapping and R2* study at 3 Tesla

Purpose Aging is the most significant determinant for brain iron accumulation in the deep grey matter. Data on brain iron evolution during brain maturation in early childhood are limited. The purpose of this study was to investigate age-related iron deposition in the deep grey matter in children using quantitative susceptibility (QSM) and R2* mapping. Methods We evaluated brain MRI scans of 74 children (age 6–154 months, mean 40 months). A multi-echo gradient-echo sequence obtained at 3 Tesla was used for the QSM and R2* calculation. Susceptibility of the pallidum, head of caudate nucleus, and putamen was correlated with age and compared between sexes. Results Susceptibility changes in all three nuclei correlated with age (correlation coefficients for QSM/R2*: globus pallidus 0.955/0.882, caudate nucleus 0.76/0.65, and putamen 0.643/0.611). During the first 2 years, the R2* values increased more rapidly than the QSM values, indicating a combined effect of iron deposition and myelination, followed by a likely dominating effect of iron deposition. There was no significant gender difference. Conclusion QSM and R2* can monitor myelin maturation processes and iron accumulation in the deep grey nuclei of the brain in early life and may be a promising tool for the detection of deviations of this normal process. Susceptibility in the deep nuclei is almost similar early after birth and increases more quickly in the pallidum. The combined use of QSM and R2* analysis is beneficial.

Automated quantification of deep grey matter structures and white matter lesions using magnetic resonance imaging in relapsing remission multiple sclerosis

Background Brain volume loss (BVL) is widespread in MS and occurs throughout the disease course at a rate considerably greater than in the general population. In MS, brain volume correlates with and predicts future disability, making BVL a relevant measure of diffuse CNS damage leading to clinical disease progression, as well as serving as a useful outcome in evaluating MS therapies. The aim of our study was to evaluate the role of automated segmentation and quantification of deep grey matter structures and white matter lesions in Relapsing Remitting Multiple Sclerosis patients using MR images and to correlate the volumetric results with different degrees of disability based on expanded disability status scale (EDSS) scores. Results All the patients in our study showed relative atrophy of the thalamus and the putamen bilaterally when compared with the normal control group. Statistical analysis was significant for the thalamus and the putamen atrophy (P value < 0.05). On the other hand, statistical analysis was not significant for the caudate and the hippocampus (P value > 0.05); there was a significant positive correlation between the white matter lesions volume and EDSS scores (correlation coefficient of 0.7505). On the other hand, there was a significant negative correlation between the thalamus and putamen volumes, and EDSS scores (correlation coefficients < − 0.9), while the volumes of the caudate and the hippocampus had a very weak and non-significant correlation with the EDSS scores (correlation coefficients > − 0.35). Conclusions The automated segmentation and quantification tools have a great role in the assessment of brain structural changes in RRMS patients, and that it became essential to integrate these tools in the daily medical practice for the great value they add to the current evaluation measures.

An automatic multi-tissue human fetal brain segmentation benchmark using the Fetal Tissue Annotation Dataset

It is critical to quantitatively analyse the developing human fetal brain in order to fully understand neurodevelopment in both normal fetuses and those with congenital disorders. To facilitate this analysis, automatic multi-tissue fetal brain segmentation algorithms are needed, which in turn requires open datasets of segmented fetal brains. Here we introduce a publicly available dataset of 50 manually segmented pathological and non-pathological fetal magnetic resonance brain volume reconstructions across a range of gestational ages (20 to 33 weeks) into 7 different tissue categories (external cerebrospinal fluid, grey matter, white matter, ventricles, cerebellum, deep grey matter, brainstem/spinal cord). In addition, we quantitatively evaluate the accuracy of several automatic multi-tissue segmentation algorithms of the developing human fetal brain. Four research groups participated, submitting a total of 10 algorithms, demonstrating the benefits the dataset for the development of automatic algorithms.

Contrasting the brain imaging features of MOG-antibody disease, with AQP4-antibody NMOSD and multiple sclerosis

Background: Identifying magnetic resonance imaging (MRI) markers in myelin-oligodendrocytes-glycoprotein antibody-associated disease (MOGAD), neuromyelitis optica spectrum disorder-aquaporin-4 positive (NMOSD-AQP4) and multiple sclerosis (MS) is essential for establishing objective outcome measures. Objectives: To quantify imaging patterns of central nervous system (CNS) damage in MOGAD during the remission stage, and to compare it with NMOSD-AQP4 and MS. Methods: 20 MOGAD, 19 NMOSD-AQP4, 18 MS in remission with brain or spinal cord involvement and 18 healthy controls (HC) were recruited. Volumetrics, lesions and cortical lesions, diffusion-imaging measures, were analysed. Results: Deep grey matter volumes were lower in MOGAD ( p = 0.02) and MS ( p = 0.0001), compared to HC and were strongly correlated with current lesion volume (MOGAD R = −0.93, p < 0.001, MS R = −0.65, p = 0.0034). Cortical/juxtacortical lesions were seen in a minority of MOGAD, in a majority of MS and in none of NMOSD-AQP4. Non-lesional tissue fractional anisotropy (FA) was only reduced in MS ( p = 0.01), although focal reductions were noted in NMOSD-AQP4, reflecting mainly optic nerve and corticospinal tract pathways. Conclusion: MOGAD patients are left with grey matter damage, and this may be related to persistent white matter lesions. NMOSD-AQP4 patients showed a relative sparing of deep grey matter volumes, but reduced non-lesional tissue FA. Observations from our study can be used to identify new markers of damage for future multicentre studies.

Abstract MP24: Sedentary Behavior (SB) In Mid-life And Structural Brain Magnetic Resonance Imaging (MRI) Markers Of Cerebrovascular Disease And Neurodegeneration In Late-life: The Atherosclerosis Risk In Communities Neurocognitive Study (ARIC-NCS)

Introduction: Few prospective studies have examined the associations of SB on brain MRI markers. We tested the hypotheses that higher levels of, and persistence of mid-life television viewing, a cognitively passive SB, are associated with structural brain MRI markers in late-life, and that these associations are independent from physical activity (PA). Methods: ARIC participants (n=1,601, mean age: 76.2 years, 60.5% female, 27.2% Black) with reported television viewing at visits 1 (1987-89) and 3 (1993-95), and brain MRI in 2011-13 were included. Participants were categorized as low [never/seldom], medium [sometimes], or high [often/very often] television viewing. Persistent pattern of television viewing was quantified as the same frequency of reported television viewing at visits 1 and 3 (n=971). Imaging using 3T brain MRI quantified the presence of cerebrovascular lesions, white matter microstructural integrity and disease, and grey matter volumes using a standardized protocol. Models were adjusted for age, race-center, sex, education, APOE-ε4, smoking status, and total intracranial volume in volumetric analysis. Sampling weights were included to generalize MRI sample to the visit 5 cohort. Interactions by meeting (or not) 2018 PA guidelines were tested. Results: Compared to low television viewing, medium and high television viewing in midlife was significantly associated with smaller deep grey matter volumes in late-life after multivariable adjustment; associations were stronger for persistent television viewing ( Table ). All other associations of midlife, or persistent midlife, television viewing with structural brain MRI markers were statistically null. Interactions with meeting PA guidelines were also non-significant. Conclusions: Findings suggest an inverse association of mid-life television viewing with later-life deep grey matter volumes. Studies examining the associations of daily accumulated SB, and differences by SB type (active versus passive), with brain MRI markers are needed.

Abstract P120: Association Of Carotid Intima-Media Thickness With Brain MRI Markers In The Atherosclerosis Risk In Communities Neurocognitive Study (ARIC-NCS)

Background: Elevated carotid intima-media thickness (cIMT) and presence of carotid plaque are markers of atherosclerosis, which may be linked to structural brain injury. Using data from the ARIC-NCS cohort, we tested the hypotheses that greater cIMT and presence of carotid plaque at late midlife were each associated with presence of infarcts (lacunar and cortical) and cerebral microbleeds (lobar and subcortical), greater white matter hyperintensity (WMH) volume, and smaller regional brain volumes in late life. Methods: We included 1,795 ARIC participants who had carotid ultrasounds in 1990-92 and brain MRI scans in 2011-13. Weighted linear regression was used for continuous (brain volume) outcomes, and weighted logistic regression for dichotomous outcomes (infarcts and cerebral microbleeds). Results: Participants were aged 57±6 years at baseline, 57% were female, and 23% were Black. Mean ± SD follow-up was 21±1 years. After multivariable adjustments, the highest quintile of cIMT in late midlife was associated with smaller deep grey matter volume (β [95% CI]: -0.11 [-0.22, -0.01]) and cortical volume in a temporal-parietal meta region of interest (ROI) (β [95% CI]: -0.10 [-0.20, -0.01]) in late life (Table). At baseline, 473 (26%) participants had carotid plaque present on their carotid ultrasound. Similar to cIMT, those with carotid plaque had smaller regional brain volumes than those without carotid plaque present (βs [95% CIs]: -0.05 [-0.12, 0.03] and -0.06 [-0.13, 0.01] for deep grey matter and temporal-parietal meta ROI volumes, respectively). No significant relations were observed with WMH volume, or the presence of any infarcts or cerebral microbleeds. Conclusion: Greater midlife cIMT and presence of carotid plaque were associated with smaller deep grey matter volume and cortical volume in a temporal-parietal meta ROI in later life. Contrary to our hypothesis, associations between midlife subclinical atherosclerosis and markers of vascular brain injury in later life were null.