Periventricular White Matter Research Articles

PRE- AND POST- SHUNT CEREBRAL PERFUSION MEASURED BY DYNAMIC SUSCEPTIBILITY CONTRAST MRI IN PATIENTS WITH NORMAL PRESSURE HYDROCEPHALUS.

Background: NPH-related dementia is the only surgically manageable dementia. Response to ventricular shunting in NPH is variable. This study aims to establish a possible correlation between the changes in cerebral perfusion with clinical response to shunting. The study group consisted of Methods: 20 patients with NPH diagnoses who underwent ventricular shunting. Pre- and 3-month post- shunt DSC-MRI was done to assess the change in relative cerebral blood ow (rCBF). Mean ± SD values of rCBF in frontal grey matter, frontal periven Results: tricular white matter, parietal periventricular white matter and hippocampus were 0.91 ± 0.11, 0.61 ± 0.09, 0.47 ± 0.11 and 0.93 ± 0.06 before shunt surgery and 0.98 ± 0.06, 0.70 ± 0.07, 0.54 ± 0.07 and 0.98 ± 0.06 after shunt surgery in NPH patients who responded to shunt surgery; and 0.78 ± 0.17, 0.37 ± 0.14, 0.41 ± 0.11 and 0.89 ± 0.10 before shunt surgery and 0.79 ± 0.14, 0.37 ± 0.19, 0.38 ± 0.09 and 0.84 ± 0.07 after shunt surgery in NPH patients who did not respond to shunt surgery. Therefore, regional rCBF in these regions increased in responders after shunt surgery, with a signicant statistical difference (p-value < 0.05) while no signicant change was seen in shunt non-responders (p-value > 0.05). There is a correlation between a si Conclusion: gnicant increase in regional rCBF and clinical improvement after shunt surgery.

Effect of right hemispheric damage on structured spoken conversation.

Patients with right hemisphere damage (RHD) occasionally complain of difficulties in conversation. A conversation is a type of communication between the speaker and listener, and several elements are required for a conversation to take place. However, it is unclear which of those elements affect communication in patients with RHD. Therefore, we prospectively enrolled 11 patients with right hemispheric damage due to acute cerebral infarction, within 1 week of onset. To evaluate patients’ conversational abilities, we used a structured conversation task, namely, the “Hallym Conversation and Pragmatics Protocol”. The topics of conversation were “family”, “leisure”, and “other/friends”. The conversation characteristics were classified according to three indices: the “conversational participation index”, “topic manipulation index”, and “conversational breakdown index”. Patients with RHD were compared with 11 age-, sex-, and years of education-matched healthy adults. The most common site of damage in the patients with RHD was the periventricular white matter. There was no significant difference in performance between the two groups according to the conversation participation index and in the discontinuance rate assessed with the conversational breakdown index. However, patients with RHD showed a lower topic maintenance rate and higher topic initiation and topic switching rates, according to the topic manipulation index. Therefore, we explored the characteristics of impaired conversation abilities in patients with RHD by assessing their ability to converse and manage topics during structured conversations, and found difficulties with pragmatics and communication discourse in these patients.

Different mechanisms in periventricular and deep white matter hyperintensities in old subjects.

ObjectiveAlthough multiple pieces of evidence have suggested that there are different mechanisms in periventricular white matter hyperintensities (PWMHs) and deep white matter hyperintensities (DWMHs), the exact mechanism remains uncertain.MethodsWe reviewed clinical and imaging data of old participants from a local She Ethnic group. We assessed the cerebral blood flow of white matter (WM-CBF) on arterial spin-labeling, deep medullary veins (DMVs) visual score on susceptibility-weighted imaging, and index for diffusion tensor image analysis along the perivascular space (ALPS index), indicating glymphatic function on diffusion tensor imaging. Furthermore, we investigated their relationships with volumes of PWMHs and DWMHs.ResultsA total of 152 subjects were included, with an average age of 63 ± 8 years old. We found that higher age and history of hypertension were independently related to higher volumes of both PWMHs and DWMHs (all p < 0.05). Lower ALPS index was independently associated with higher PWMHs volumes (β = 0.305, p < 0.001), and this relationship was accounted for by the indirect pathway via DMVs score (β = 0.176, p = 0.017). Both lower ALPS index and WM-CBF were independent risk factors for higher DWMHs volumes (β = −0.146, p = 0.041; β = −0.147, p = 0.036).ConclusionsOur study indicated that there were different mechanisms in PWMHs and DWMHs. PWMHs were mainly attributed to the damage of veins due to the dysfunction of the glymphatic pathway, while DWMHs could be affected by both ischemia-hypoperfusion and dysfunction of the glymphatic pathway.Advances in knowledgeThe relationship between glymphatic dysfunction and PWMHs might be accounted for by the indirect pathway via venous abnormalities, a glymphatic dysfunction, and lower CBF in white matter were independent risk factors for DWMHs.

Association of Serum GFAP with Functional and Neurocognitive Outcome in Sporadic Small Vessel Disease.

Cerebrospinal fluid (CSF) and serum biomarkers are critical for clinical decision making in neurological diseases. In cerebral small vessel disease (CSVD), white matter hyperintensities (WMH) are an important neuroimaging biomarker, but more blood-based biomarkers capturing different aspects of CSVD pathology are needed. In 42 sporadic CSVD patients, we prospectively analysed WMH on magnetic resonance imaging (MRI) and the biomarkers neurofilament light chain (NfL), glial fibrillary acidic protein (GFAP), chitinase3-like protein 1 (CHI3L1), Tau and Aβ1-42 in CSF and NfL and GFAP in serum. GFAP and CHI3L1 expression was studied in post-mortem brain tissue in additional cases. CSVD cases with higher serum NfL and GFAP levels had a higher modified Rankin Scale (mRS) and NIHSS score and lower CSF Aβ1-42 levels, whereas the CSF NfL and CHI3L1 levels were positively correlated with the WMH load. Moreover, the serum GFAP levels significantly correlated with the neurocognitive functions. Pathological analyses in CSVD revealed a high density of GFAP-immunoreactive fibrillary astrocytic processes in the periventricular white matter and clusters of CHI3L1-immunoreactive astrocytes in the basal ganglia and thalamus. Thus, besides NfL, serum GFAP is a highly promising fluid biomarker of sporadic CSVD, because it does not only correlate with the clinical severity but also correlates with the cognitive function in patients.

Periventricular white matter hyperintensities are associated with gait and balance in patients with minor stroke.

ObjectiveCerebral small vessel disease (CSVD) is associated with gait and balance deficits in older adults. However, the effect of CSVD-related brain injury on post-stroke mobility is unknown. This study aimed to investigate the association of CSVD with gait and balance impairment after a minor stroke.MethodsA total of 273 patients with a minor stroke (NIHSS ≤ 5 points) who were hospitalized at the Affiliated Hospital of Qingdao University were enrolled. The manifestations of white matter hyperintensities (WMH), lacunes, enlarged perivascular spaces (EPVS), and cerebral microbleeds (CMB) were statistically analyzed according to magnetic resonance imaging results, and the total burden score of CSVD was calculated. Gait function was assessed by a 6-m walking speed test, and balance function was assessed by the timed-up-and-go (TUG) test. Linear regression analysis was applied to determine the association after adjusting for key variables.ResultsThe correlation results showed that in patients with minor stroke, age, sex, smoking history, and the infarct site were associated with gait speed, and age and the infarct site were associated with the TUG test. In the univariate linear regression model, periventricular white matter hyperintensities (PVWMH), deep white matter hyperintensities (DWMH), and the total burden of CSVD were correlated with gait speed, while only PVWMH correlated with the TUG test. After adjusting for confounders, only PVWMH were independent predictors of gait speed (β = −0.089, p < 0.05) and the TUG test (β = 0.517, p < 0.05).ConclusionsOur study confirmed that CSVD is associated with gait and balance disorders after a minor stroke. PVWMH are independent predictors of gait and balance disorders in patients with minor stroke. These findings should be confirmed in larger prospective studies.

3D finite-element brain modeling of lateral ventricular wall loading to rationalize periventricular white matter hyperintensity locations

Aging-related periventricular white matter hyperintensities (pvWMHs) are a common observation in medical images of the aging brain. The underlying tissue damage is part of the complex pathophysiology associated with age-related microstructural changes and cognitive decline. PvWMH formation is linked to blood–brain barrier dysfunction from cerebral small vessel disease as well as the accumulation of cerebrospinal fluid in periventricular tissue due to progressive denudation of the ventricular wall. In need of a unifying theory for pvWMH etiology, image-based finite-element modeling is used to demonstrate that ventricular expansion from age-related cerebral atrophy and hemodynamic loading leads to maximum mechanical loading of the ventricular wall in the same locations that show pvWMHs. Ventricular inflation, induced via pressurization of the ventricular wall, creates significant ventricular wall stretch and stress on the ependymal cells lining the wall, that are linked to cerebrospinal fluid leaking from the lateral ventricles into periventricular white matter tissue. Eight anatomically accurate 3D brain models of cognitively healthy subjects with a wide range of ventricular shapes are created. For all models, our simulations show that mechanomarkers of mechanical wall loading are consistently highest in pvWMHs locations (p < 0.05). Maximum principal strain, the ependymal cell thinning ratio, and wall curvature are on average 14%, 8%, and 24% higher in pvWMH regions compared to the remaining ventricular wall, respectively. Computational modeling provides a powerful framework to systematically study pvWMH formation and growth with the goal to develop pharmacological interventions in the future.

Symptomatic care of late-onset Alexander disease presenting with area postrema-like syndrome with prednisolone; a case report

BackgroundAlexander disease (AxD) is classified into AxD type I (infantile) and AxD type II (juvenile and adult form). We aimed to determine the potential genetic cause(s) contributing to the AxD type II manifestations in a 9-year-old male who presented area postrema-like syndrome and his vomiting and weight loss improved after taking prednisolone.Case presentationA normal cognitive 9-year-old boy with persistent nausea, vomiting, and a significant weight loss at the age of 6 years was noticed. He also experienced an episode of status epilepticus with generalized atonic seizures. He showed non-febrile infrequent multifocal motor seizures at the age of 40 days which were treated with phenobarbital. He exhibited normal physical growth and neurologic developmental milestones by the age of six. Occasionally vomiting unrelated to feeding was reported. Upon examination at 9 years, a weak gag reflex, prominent drooling, exaggerated knee-deep tendon reflexes (3+), and nasal tone speech was detected. All gastroenterological, biochemical, and metabolic assessments were normal. Brain magnetic resonance imaging (MRI) revealed bifrontal confluent deep and periventricular white matter signal changes, fine symmetric frontal white matter and bilateral caudate nucleus involvements with garland changes, and a hyperintense tumefactive-like lesion in the brain stem around the floor of the fourth ventricle and area postrema with contrast uptake in post-contrast T1-W images. Latter MRI at the age of 8 years showed enlarged area postrema lesion and bilateral middle cerebellar peduncles and dentate nuclei involvements. Due to clinical and genetic heterogeneities, whole-exome sequencing was performed and the candidate variant was confirmed by Sanger sequencing. A de novo heterozygous mutation, NM_001242376.1:c.262 C > T;R88C in exon 1 of the GFAP (OMIM: 137,780) was verified. Because of persistent vomiting and weight loss of 6.0 kg, prednisolone was prescribed which brought about ceasing vomiting and led to weight gaining of 3.0 kg over the next 3 months after treatment. Occasional attempts to discontinue prednisolone had been resulting in the reappearance of vomiting.ConclusionsThis study broadens the spectrum of symptomatic treatment in leukodystrophies and also shows that R88C mutation may lead to a broad range of phenotypes in AxD type II patients.

Age related increase in internal jugular vein size parallels temporal development of periventricular white matter hyperintensities.

Age-related white matter hyperintensities are associated with cognitive impairment and dementia. Venous insufficiency has recently been proposed as a potential mechanism for the development of periventricular white matter hyperintensities based on the neuroanatomic distribution. The current study assesses age related changes of the internal jugular veins and its association with white matter hyperintensities. A retrospective study was performed assessing patients with computed tomography angiography (CTA) and magnetic resonance imaging (MRI) within a 4-week window. The size of the internal jugular veins, straight sinus, vein of Galen and internal cerebral veins were measured on the CT angiography. A normalized neck venous ratio was developed. Burden of white matter hyperintensities were quantified on MRI using periventricular/deep Fazekas scores. Association was assessed using correlation analysis and multrivariate linear modeling, and differences between groups were assessed using t test, ANOVA or Kruskal-Wallis test, using p < 0.05 for significance. One hundred eighty-two patients were included with a mean age of 65.2 ± 16.8 (51.6% females). Age was correlated with the normalized neck venous ratio (rs =0.25, p < 0.001), and, with both, the periventricular Fazekas (rs =0.63, p < 0.001) and the deep Fazekas (rs =0.57, p < 0.001) grades. The periventricular Fazekas score was positively correlated with the normalized neck venous ratio (rs =0.21, p=0.003), but not significant on multivariate analysis accounting for age. The internal jugular veins demonstrate age related increase in size, paralleling the progression of periventricular white matter hyperintensities. Age remains the strongest predictor of white matter hyperintensities. Further work is needed to evaluate any causal role of venous changes on white matter hyperintensities.

Combined Score of Perivascular Space Dilatation and White Matter Hyperintensities in Patients with Normal Cognition, Mild Cognitive Impairment, and Dementia.

Background and Objectives: Cerebral perivascular spaces (PVS) are part of the cerebral microvascular structure and play a role in lymphatic drainage and the removal of waste products from the brain. White matter hyperintensities (WMH) are hyperintense lesions on magnetic resonance imaging that are associated with cognitive impairment, dementia, and cerebral vascular disease. WMH and PVS are direct and indirect imaging biomarkers of cerebral microvascular integrity and health. In our research, we evaluated WMH and PVS enlargement in patients with normal cognition (NC), mild cognitive impairment (MCI), and dementia (D). Materials and Methods: In total, 57 participants were included in the study and divided into groups based on neurological evaluation and Montreal Cognitive Assessment results (NC group 16 participants, MCI group 29 participants, D group 12 participants). All participants underwent 3T magnetic resonance imaging. PVS were evaluated in the basal ganglia, centrum semiovale, and midbrain. WMHs were evaluated based on the Fazekas scale and the division between deep white matter (DWM) and periventricular white matter (PVWM). The combined score based on PVS and WMH was evaluated and correlated with the results of the MoCA. Results: We found statistically significant differences between groups on several measures. Centrum semiovale PVS dilatation was more severe in MCI and dementia group and statistically significant differences were found between D-MCI and D-NC pairs. PVWM was more severe in patients with MCI and dementia group, and statistically significant differences were found between D-MCI and D-NC pairs. Furthermore, we found statistically significant differences between the groups by analyzing the combined score of PVS dilatation and WMH. We did not find statistically significant differences between the groups in PVS dilation of the basal ganglia and midbrain and DWM hyperintensities. Conclusions: PVS assessment could become one of neuroimaging biomarkers for patients with cognitive decline. Furthermore, the combined score of WMH and PVS dilatation could facilitate diagnostics of cognitive impairment, but more research is needed with a larger cohort to determine the use of PVS dilatation and the combined score.

Association of sudden sensorineural hearing loss and its prognosis with the brain white matter hyperintensity

Objective:To explore the relationship between white matter hyperintensity（WMH） and sudden sensorineural hearing loss（SSHL） and evaluate the influence of WMH on the prognosis of SSHL. Methods:Fifty hospitalized unilateral SSHL patients and 50 age and gender matched routine physical examination individuals without SSHL history from June 2019 to June 2020 were included for a case-control study. All included subjects underwent 3.0 Tesla cranial magnetic resonance examination, and the Fazekas scale was applied to evaluate periventricular white matter hyperintense（PVWMH） and deep white matter hyperintense（DWMH）. Fazekas score and distribution proportions of Fazekas score was compared between SSHL and control. Ordered logistic regression was used to study the relationship between prognosis of SSHL and WMH. Results:The Fazekas score of WMH in SSHL group was significantly higher than that of control group（PVWMH: P=0.004, DWMH: P=0.010）; There was a significant difference in the distribution proportions of Fazekas scores between SSHL and control（PVWMH: P=0.036, DWMH: P=0.047）; The results of ordered logistic regression showed that patients without WMH（Fazekas=0） is an independent predictor of good prognosis in SSHL（P=0.025, OR=12.779）. Conclusion:The prevalence of WMH in SSHL patients was higher than that of control. SSHL patients without WMH has a better prognosis than those with WMH.

Acute Stroke Caused by Large Vessel Vasculitis in a Patient with Systemic Lupus Erythematosus

Systemic lupus erythematosus is an uncommon cause of ischemic stroke. The subcortical and periventricular white matter are most commonly involved in the central nervous system. Although vasculitis is the most frequently mentioned etiology of stroke, it has been mainly documented in the vicinity of small-diameter vessels. In the present case, we report a young women with lupus nephritis who presented with stroke caused by large-vessel vasculitis, which was confirmed by vessel wall magnetic resonance imaging.

Relations of impaired blood flow and cerebrospinal fluid flow with damage of strategic for cognitive impairment brain regiones in cerebral small vessel disease

Introduction. Cerebral small vessel disease (CSVD), associated with age and vascular risk factors, as well as the main cause of vascular and degenerative mixed cognitive impairment (CI). Previously established microstructural predictors of CI (axial diffusion in normal-appearing periventricular white matter of the posterior left frontal lobe, the right midcingulate cortex, and the middle posterior part of the corpus callosum) can be used to calculate an integrative factor, exceeding the threshold value for which indicates the presence of CI. The use of this factor in the diagnosis of CI in CSVD is supported by the fact that leading mechanisms of CSVD are involved in the damage to areas of the brain that are strategic for CI. The aim of this study was to clarify the link between the known microstructural predictors of CI in CSVD and MRI findings that correspond to the main mechanisms of CSVD. Materials and methods. Patients (n = 74; including 48 women; average age 60.6 6.9 years) with CSVD and CI of varying severity underwent phase-contrast MRI and voxel-based morphometry (3T) to assess arterial, venous and CSF flow, as well as atrophy. Results. The established microstructural predictors of CI correlated with measures of arterial and venous blood flow, as well as atrophy. Linear regression models allow us to estimate cognitive impairment (CI) predictors in cerebral small vessel disease (CSVD), based on increased arterial velocity pulse index, CSF flow at the level of the cerebral aqueduct, cerebral aqueduct area and lateral ventricles volume, when there is reduced blood flow in the superior sagittal sinus and the overall arterial blood flow. Conclusion. The ability to calculate microstructural predictors of CI due to CSVD, based on MRI findings, indicates the validity of using an integrative measure of microstructural predictors of CI as a diagnostic tool of CI in CSVD.

Association Between Enlarged Perivascular Spaces and Cognition in a Memory Clinic Population.

Although enlarged perivascular spaces (EPVS) have been suggested as an emerging measure of small vessel disease (SVD) in the brain, their association with cognitive impairment is not yet clearly understood. We aimed to examine the relationship between each EPVS in the basal ganglia (BG-EPVS) and centrum semiovale (CSO-EPVS) with cognition in a memory clinic population. Participants with a diverse cognitive spectrum were recruited from a university hospital memory clinic. They underwent comprehensive clinical and neuropsychological assessments and brain MRI. BG-EPVS and CSO-EPVS were measured on T2-weighted MRI and then dichotomized into low and high degrees for further analyses. Other SVD markers were assessed using validated rating scales. A total of 910 participants were included in this study. A high degree of BG-EPVS was significantly associated with poorer scores on the executive function domain, but not with other cognitive domains, when age, sex, education, MRI scanner type, and cognitive diagnosis were controlled as covariates. However, the association between BG-EPVS and executive function was no longer significant after controlling for other markers of SVD, such as lacunar infarcts and periventricular white matter hyperintensities, as additional covariates. CSO-EPVS did not have a significant relationship with any cognitive scores, regardless of the covariates. Our findings from a large memory clinic population suggest that EPVS, regardless of the topographical location, may not be used as a specific SVD marker for cognitive impairment, although an apparent association was observed between a high degree of BG-EPVS and executive dysfunction before controlling other SVD markers that share a common pathophysiologic process with BG-EPVS.

Human Brain Lipidomics: Investigation of Formalin Fixed Brains.

Human brain lipidomics have elucidated structural lipids and lipid signal transduction pathways in neurologic diseases. Such studies have traditionally sourced tissue exclusively from brain bank biorepositories, however, limited inventories signal that these facilities may not be able to keep pace with this growing research domain. Formalin fixed, whole body donors willed to academic institutions offer a potential supplemental tissue source, the lipid profiles of which have yet to be described. To determine the potential of these subjects in lipid analysis, the lipid levels of fresh and fixed frontal cortical gray matter of human donors were compared using high resolution electrospray ionization mass spectrometry. Results revealed commensurate levels of specific triacylglycerols, diacylglycerols, hexosyl ceramides, and hydroxy hexosyl ceramides. Baseline levels of these lipid families in human fixed tissue were identified via a broader survey study covering six brain regions: cerebellar gray matter, superior cerebellar peduncle, gray and subcortical white matter of the precentral gyrus, periventricular white matter, and internal capsule. Whole body donors may therefore serve as supplemental tissue sources for lipid analysis in a variety of clinical contexts, including Parkinson’s disease, Alzheimer’s disease, Lewy body dementia, multiple sclerosis, and Gaucher’s disease.

A Rare Case Report on Opsoclonus Myoclonus Syndrome Associated with a Developmental Regression

Published on:June 2022 Journal of Young Pharmacists, 2022; 14(2):256-257 Case Report | doi:10.5530/jyp.2022.14.49 Authors: Neha Suratiya*, Khevna Shah, Preya Soni, Sarvajeet Khare Department of Pharmacy Practice, Sumandeep Vidyapeeth (Deemed University), Vadodara, Gujarat, INDIA. Abstract: Developmental regression can be described as the progressive loss of previously acquired skills which is relatively rare. Opsoclonus myoclonus syndrome is an uncommon autoimmune disorder in which a patient’s body attacks its own nervous system. It usually affects young children. Complicated developmental regression may lead to diagnosis of Opsoclonus Myoclonus Syndrome (OMS). A 3 years and 1 month old, the female child presented the history of convulsion, tonic contraction of upper and lower limb, up rolling of eye ball, frothing from mouth and post ictal drowsiness at the age of 1.5 years. On examination her general condition was unstable and systemic examination was not normal (Temp- 102.2 F). Her hematological and hepatic tests were done. Magnetic Resonance Imaging (MRI) report of her brain conclude that few T2 and FLAIR hyper intense foci are noted in bilateral periventricular deep white matter possibility of myelin pallor likely. We show that Opsoclonus myoclonus syndrome can be associated with developmental regression. Neurologist must be aware of such presentation. Our findings highlighted the need for increased recognition of developmental regression leading OMS as an urgent and treatable condition.Key words: Opsoclonus myoclonus syndrome (OMS), Developmental regression, Paraneoplastic, Myelin pallor, Ataxia, Cerebellar nuclei degeneration.

Imaging Characteristics for Predicting Cognitive Impairment in Patients With Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy.

ObjectivesPatients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) show various clinical symptoms, including migraine, recurrent stroke, and cognitive impairment. We investigated the associations between magnetic resonance imaging (MRI) markers of small vessel disease and neuropsychological tests and identified the MRI characteristics for predicting cognitive impairment in patients with CADASIL.MethodsSubjects included 60 CADASIL patients diagnosed with genetic tests and registered in the Japanese CADASIL REDCap database between June 2016 and December 2020. Patient information including clinical data, modified Rankin Scale (mRS); MRI findings of small vessel disease including periventricular and deep white matter lesions (WML), lacunar infarcts, and cerebral microbleeds (CMBs); and neuropsychological tests, including the Japanese version of the Mini-Mental State Examination (MMSE), the Japanese version of the Montreal Cognitive Assessment (MoCA-J), and the Frontal Assessment Battery (FAB), were evaluated.ResultsData from 44 CADASIL patients were eligible for this study, compared between patients with and without dementia. Regarding the neuroimaging findings, the Fazekas score of periventricular and deep WML was higher in patients with dementia (periventricular, p = 0.003; deep, p = 0.009). The number of lacunar infarcts was higher in patients with dementia (p = 0.001). The standardized partial regression coefficient (SPRC) in MoCA-J was 0.826 (95% CI, 0.723–0.942; p = 0.005) for the number of CMBs. The SPRC in MMSE was 0.826 (95% CI, 0.719–0.949; p = 0.007) for the number of CMBs. The SPRC for FAB decreased significantly to 0.728 (95% CI, 0.551–0.960; p = 0.024) for the number of lacunar infarcts. Receiver operating characteristic (ROC) curves for dementia showed that in the number of lacunar infarcts, a cut-off score of 5.5 showed 90.9% sensitivity and 61.1% specificity. For the number of CMBs, a cut-off score of 18.5 showed 45.5% sensitivity and 100% specificity.ConclusionThe characteristic MRI findings were that CADASIL patients with dementia had severe WML, both periventricular and deep, and a larger number of lacunar infarcts than those without dementia. The risk of dementia may be associated with ≥ 6 lacunar infarcts, ≥19 CMBs, or a Fazekas scale score of 3 in periventricular and deep WML.

Quantitative Analysis of White Matter Hyperintensity: Comparison of Magnetic Resonance Imaging Image Analysis Software

ObjectiveWhite matter hyperintensity (WMH), defined as abnormal signals on magnetic resonance imaging (MRI), is an important clinical indicator of aging and dementia. Although MRI image analysis software can automatically detect WMH, the quantitative accuracy of periventricular hyperintensity (PVH) and deep white matter hyperintensity (DWMH) is unknown. Materials and MethodsThis study was a sub-analysis of MRI data from an ongoing hospital-based prospective cohort study (the Gimlet study). Between March 2016 and March 2017, we enrolled patients who visited our memory clinic and agreed to undergo medical assessments of cognitive function and fecal examination to study the gut microbiome. Participants with a history of stroke were excluded. WMH was independently quantitatively analyzed using two MRI imaging analysis software modalities: SNIPER and FUSION. Intraclass correlation coefficients and the mean difference in volume were calculated and compared between modalities. ResultsThe data of 87 patients (49 women, mean age 74.8 ± 7.9 years) were analyzed. Both total WMH and DWMH volumes obtained using FUSION were greater (p < 0.001), and PVH volume was smaller (p < 0.001) than those obtained using SNIPER. Intraclass correlation coefficients for the lesion measurements of WMH, PVH, and DWMH between the different software were 0.726 (p < 0.001), 0.673 (p < 0.001), and 0.048 (p = 0.231), respectively. ConclusionsThere were significant differences in the quantitative data of WMH between the two MRI imaging analysis software modalities. Thus, care should be taken for quantitative assessments of WMH.

Mania With Psychotic Symptoms a Rare Clinical Presentation of Fahr's Disease- a Case Report

AimsFahr's disease is a rare neurodegenerative disease with radiological findings of symmetrical and bilateral idiopathic calcifications of the cerebellum, periventricular white matter, and basal ganglia, characterized by the presence of neuropsychiatric symptoms.MethodsWe report a case of a 46-year-old male who presented with psychomotor agitation, aggression, restlessness, irritability, decrease need for sleep and psychotic symptoms including grandeur and mystical delusions. He had a previous history of an admission 4 years prior with a similar presentation. Computed tomographic scan of the patient demonstrated a bilateral calcification of globus pallidus. Laboratory investigation was unremarkable. Due to agitation, the patient started treatment with Haloperidol 10 mg and Levomepromazine 25 mg presenting resulting in important extra-pyramidal symptoms (EPS), namely marked motor rigidity. Subsequently, a switch was made to Olanzapine 5 mg with persistence of clinically significant EPS. A final switch was made to Aripiprazol 15 mg (gradually titrated) and a mood stabilizer was started (Sodium Valproate), with full clinical remission within a month and no signs of EPS.ResultsThe age of onset of manic symptoms in this patient is not suggestive of bipolar disorder (average age onset 25). On the other hand, Fahr's disease usually presents within the 4th and 5th decade of life. The clinical presentation usually involves motor symptoms (movement disorder and Parkinson like symptoms) and dementia, but purely psychiatric presentations have been described. The localization of calcifications also seems to have a clinical correlation, as Pallidal calcifications as the ones identified in our patient have been associated with manic symptoms. Idiopathic forms in which no metabolic or other underlying causes are identified, treatment is usually symptomatic, but one has to be cautious because these patients have an increased sensitivity to neuroleptics and can thus easily develop EPS.ConclusionPsychiatrists should consider Fahr's disease as a differential diagnosis in a manic episode, especially with a late age of onset, which is not suggestive of a bipolar disorder. This case also further emphasizes the importance of neuro-imaging in psychiatry and underlines the importance of a careful treatment approach in this type of patients because of an higher risk of developing EPS.

C.1 Cerebral venous sinus thrombosis in preterm infants

Background: Previous studies of neonatal cerebral venous sinus thrombosis (CVST) have focused on term infants, and studies of preterm infants are lacking. In this study, we examined the clinical and radiological features, treatment and outcome of CVST in preterm infants. Methods: This was a retrospective cohort study of preterm infants (gestational age &lt;37 weeks) with radiologically confirmed CVST. All MRI/MRV and CT/CTV scans were re-reviewed. Clinical and radiological data were analysed using descriptive statistics, ANOVA and chi-square tests. Results: A total of 26 preterm infants with CVST were included. Of these, 65% were late preterm, 27% very preterm and 8% extreme preterm. Most (73%) were symptomatic at presentation with seizures or abnormal exam. Transverse (85%) and superior sagittal (42%) sinus were common sites of thrombosis. Parenchymal brain injury was predominantly periventricular (35%) and deep white matter (31%) in location. Intraventricular hemorrhage occurred in 46%. Most infants (69%) were treated with anticoagulation. None of the treated infants had hemorrhagic complications. Outcome at follow-up ranged from no impairment (50%), mild impairment (25%) and severe impairment (25%). Conclusions: Preterm infants with CVST are often symptomatic and have white matter brain lesions. Anticoagulation treatment of preterm CVST appeared to be safe and was not associated with hemorrhagic complications.

P.086 Risk factors for term born periventricular white matter injury in children with cerebral palsy: a case control study

Background: The aim of this study was to identify possible risk factors associated with term-born newborns with cerebral palsy and PWMI on imaging. Methods: This is a case-controlled study with cases from the Canadian Cerebral Palsy Registry and controls from Alberta Pregnancy Outcomes and Nutrition Study. PWMI was diagnosed based on MRI reports and 160 cases were compared to 1950 controls. Risk factor were selected a priori; including pregnancy complications, toxin exposure, perinatal infection, sex, small for gestational age, and perinatal adversity. Multivariate regression binomial model was used to calculate odds ratios(OR) and 95% confidence intervals(CI). Results: Multivariable analyses suggested PWMI was associated with pregnancy complications(OR=3.35; 95% CI=2.23-4.94), antenatal toxin exposure(OR=2.45; 95% CI=1.67-3.55), perinatal infection(OR=3.61; 95% CI=1.96-6.29) and perinatal adversity(OR=2.03; 95% CI=1.42-2.94). Term born males were not more likely to have PWMI compared to females(OR=1.37; 95% CI=0.98-1.93). Multiple regression analyses suggested independent associations between PWMI and pregnancy complications(OR=3.63; 95% CI 2.40-5.40), antenatal toxin exposure(OR=2.62; 95% CI 1.77-3.84), perinatal infection(OR=3.42; 95% CI 1.83-6.05) and perinatal adversity(OR=2.49; 95% CI= 1.71-3.69). Conclusions: Risk factors such as pregnancy complications, toxin exposure, perinatal infection and perinatal adversity are associated with PWMI in term-borns, suggesting a ‘two-hit’ model that could involve an interaction among both antenatal and perinatal variables.