Cognitive and Behavioral Functioning in Childhood Acquired Demyelinating Syndromes

2016 ◽  
Vol 22 (10) ◽  
pp. 1050-1060 ◽  
Author(s):  
Christine Till ◽  
Austin Noguera ◽  
Leonard H. Verhey ◽  
Julia O’Mahony ◽  
E. Ann Yeh ◽  
...  
Keyword(s):  
Time Course ◽  
Binary Classification ◽  
Brain Mri ◽  
Poor Performance ◽  
Brain Lesions ◽  
Cognitive Outcome ◽  
Normative Values ◽  
Visuomotor Integration ◽  
Mri Scans

AbstractObjectives:The aim of this study was to describe cognitive, academic, and psychosocial outcomes after an incident demyelinating event (acquired demyelinating syndromes, ADS) in childhood and to investigate the contribution of brain lesions and confirmed MS diagnosis on outcome.Methods:Thirty-six patients with ADS (mean age=12.2 years,SD=2.7, range: 7–16 years) underwent brain MRI scans at presentation and at 6-months follow-up. T2-weighted lesions on MRI were assessed using a binary classification. At 6-months follow-up, patients underwent neuropsychological evaluation and were compared with 42 healthy controls.Results:Cognitive, academic, and behavioral outcomes did not differ between the patients with ADS and controls. Three of 36 patients (8.3%) were identified with cognitive impairment, as determined by performance falling ≤1.5SDbelow normative values on more than four independent tests in the battery. Poor performance on a visuomotor integration task was most common, observed among 6/32 patients, but this did not differ significantly from controls. Twelve of 36 patients received a diagnosis of MS within 3 years post-ADS. Patients with MS did not differ from children with monophasic ADS in terms of cognitive performance at the 6-months follow-up. Fatigue symptoms were reported in 50% of patients, irrespective of MS diagnosis. Presence of brain lesions at onset and 6 months post-incident demyelinating event did not associate with cognitive outcome.Conclusions:Children with ADS experience a favorable short-term neurocognitive outcome, even those confirmed to have MS. Longitudinal evaluations of children with monophasic ADS and MS are required to determine the possibility of late-emerging sequelae and their time course. (JINS, 2016,22, 1050–1060)

scholarly journals Ventricular volume expansion in presymptomatic genetic frontotemporal dementia

Neurology ◽  
2019 ◽  
Vol 93 (18) ◽  
pp. e1699-e1706 ◽  
Author(s):  
Tamara P. Tavares ◽  
Derek G.V. Mitchell ◽  
Kristy Coleman ◽  
Christen Shoesmith ◽  
Robert Bartha ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Volume Expansion ◽  
Time Course ◽  
Automatic Segmentation ◽  
Disease Onset ◽  
Onset Time ◽  
Ventricular Volume ◽  
Mutation Carriers ◽  
Mri Scans

ObjectiveTo characterize the time course of ventricular volume expansion in genetic frontotemporal dementia (FTD) and identify the onset time and rates of ventricular expansion in presymptomatic FTD mutation carriers.MethodsParticipants included patients with a mutation in MAPT, PGRN, or C9orf72, or first-degree relatives of mutation carriers from the GENFI study with MRI scans at study baseline and at 1 year follow-up. Ventricular volumes were obtained from MRI scans using FreeSurfer, with manual editing of segmentation and comparison to fully automated segmentation to establish reliability. Linear mixed models were used to identify differences in ventricular volume and in expansion rates as a function of time to expected disease onset between presymptomatic carriers and noncarriers.ResultsA total of 123 participants met the inclusion criteria and were included in the analysis (18 symptomatic carriers, 46 presymptomatic mutation carriers, and 56 noncarriers). Ventricular volume differences were observed 4 years prior to symptom disease onset for presymptomatic carriers compared to noncarriers. Annualized rates of ventricular volume expansion were greater in presymptomatic carriers relative to noncarriers. Importantly, time-intensive manually edited and fully automated ventricular volume resulted in similar findings.ConclusionsVentricular volume differences are detectable in presymptomatic genetic FTD. Concordance of results from time-intensive manual editing and fully automatic segmentation approaches support its value as a measure of disease onset and progression in future studies in both presymptomatic and symptomatic genetic FTD.

The neuropsychological sequelae of delirium in elderly patients with hip fracture three months after hospital discharge

2013 ◽  
Vol 25 (9) ◽  
pp. 1521-1531 ◽  
Author(s):  
Joost Witlox ◽  
Chantal J. Slor ◽  
René W.M.M. Jansen ◽  
Kees J. Kalisvaart ◽  
Mireille F.M. van Stijn ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Hip Fracture ◽  
Episodic Memory ◽  
Hospital Discharge ◽  
Depressive Symptomatology ◽  
Poor Performance ◽  
Cognitive Outcome ◽  
Neuropsychological Sequelae ◽  
Global Cognition

ABSTRACTBackground: Delirium is a risk factor for long-term cognitive impairment and dementia. Yet, the nature of these cognitive deficits is unknown as is the extent to which the persistence of delirium symptoms and presence of depression at follow-up may account for the association between delirium and cognitive impairment at follow-up. We hypothesized that inattention, as an important sign of persistent delirium and/or depression, is an important feature of the cognitive profile three months after hospital discharge of patients who experienced in-hosptial delirium.Methods: This was a prospective cohort study. Fifty-three patients aged 75 years and older were admitted for surgical repair of acute hip fracture. Before the surgery, baseline characteristics, depressive symptomatology, and global cognitive performance were documented. The presence of delirium was assessed daily during hospital admission and three months after hospital discharge when patients underwent neuropsychological assessment.Results: Of 27 patients with in-hospital delirium, 5 were still delirious after three months. Patients with in-hospital delirium (but free of delirium at follow-up) showed poorer performance than patients without in-hospital delirium on tests of global cognition and episodic memory, even after adjustment for age, gender, and baseline cognitive impairment. In contrast, no differences were found on tests of attention. Patients with in-hospital delirium showed an increase of depressive symptoms after three months. However, delirium remained associated with poor performance on a range of neuropsychological tests among patients with few or no signs of depression at follow-up.Conclusion: Elderly hip fracture patients with in-hospital delirium experience impairments in global cognition and episodic memory three months after hospital discharge. Our results suggest that inattention, as a cardinal sign of persistent delirium or depressive symptomatology at follow-up, cannot fully account for the poor cognitive outcome associated with delirium.

Diagnostic performance of brain MRI in pharmacovigilance of natalizumab-treated MS patients

2016 ◽  
Vol 22 (9) ◽  
pp. 1174-1183 ◽  
Author(s):  
Mike P Wattjes ◽  
Martijn T Wijburg ◽  
Anke Vennegoor ◽  
Birgit I Witte ◽  
Stefan D Roosendaal ◽  
...  
Keyword(s):  
Sensitivity And Specificity ◽  
Diagnostic Performance ◽  
Brain Mri ◽  
Lesion Detection ◽  
Interrater Agreement ◽  
Diagnostic Study ◽  
Mri Scans ◽  
Interrater Variability ◽  
Magnetic Resonance Imaging Mri

Background: In natalizumab-treated multiple sclerosis (MS) patients, magnetic resonance imaging (MRI) is considered as a sensitive tool in detecting both MS disease activity and progressive multifocal leukoencephalopathy (PML). Objective: To investigate the performance of neuroradiologists using brain MRI in detecting new MS lesions and asymptomatic PML lesions and in differentiating between MS and PML lesions in natalizumab-treated MS patients. The secondary aim was to investigate interrater variability. Methods: In this retrospective diagnostic study, four blinded neuroradiologists assessed reference and follow-up brain MRI scans of 48 natalizumab-treated MS patients with new asymptomatic PML lesions ( n = 21) or new MS lesions ( n = 20) or no new lesions ( n = 7). Sensitivity and specificity for detection of new lesions in general (MS and PML lesions), MS and PML lesion differentiation, and PML detection were determined. Interrater agreement was calculated. Results: Overall sensitivity and specificity for the detection of new lesions, regardless of the nature of the lesions, were 77.4% and 89.3%, respectively; for PML-MS lesion differentiation, 74.2% and 84.7%, respectively; and for asymptomatic PML lesion detection, 59.5% and 91.7%, respectively. Interrater agreement for the tested categories was fair to moderate. Conclusion: The diagnostic performance of trained neuroradiologists using brain MRI in pharmacovigilance of natalizumab-treated MS patients is moderately good. Interrater agreement among trained readers is fair to moderate.

scholarly journals Association of White Matter Hyperintensity Markers on MRI and Long-term Risk of Mortality and Ischemic Stroke

Neurology ◽  
2021 ◽  
Vol 96 (17) ◽  
pp. e2172-e2183 ◽  
Author(s):  
Rashid Ghaznawi ◽  
Mirjam I. Geerlings ◽  
Myriam Jaarsma-Coes ◽  
Jeroen Hendrikse ◽  
Jeroen de Bresser ◽  
...  
Keyword(s):  
Ischemic Stroke ◽  
White Matter ◽  
Cox Regression ◽  
Brain Mri ◽  
Arterial Disease ◽  
White Matter Hyperintensity ◽  
Risk Of Mortality ◽  
Mri Scans

ObjectiveTo determine whether white matter hyperintensity (WMH) markers on MRI are associated with long-term risk of mortality and ischemic stroke.MethodsWe included consecutive patients with manifest arterial disease enrolled in the Second Manifestations of Arterial Disease–Magnetic Resonance (SMART-MR) study. We obtained WMH markers (volume, type, and shape) from brain MRI scans performed at baseline using an automated algorithm. During follow-up, occurrence of death and ischemic stroke was recorded. Using Cox regression, we investigated associations of WMH markers with risk of mortality and ischemic stroke, adjusting for demographics, cardiovascular risk factors, and cerebrovascular disease.ResultsWe included 999 patients (59 ± 10 years; 79% male) with a median follow-up of 12.5 years (range 0.2–16.0 years). A greater periventricular or confluent WMH volume was independently associated with a greater risk of vascular death (hazard ratio [HR] 1.29, 95% confidence interval [CI] 1.13–1.47) for a 1-unit increase in natural log-transformed WMH volume and ischemic stroke (HR 1.53, 95% CI 1.26–1.86). A confluent WMH type was independently associated with a greater risk of vascular (HR 1.89, 95% CI 1.15-3.11) and nonvascular death (HR 1.65, 95% CI 1.01–2.73) and ischemic stroke (HR 2.83, 95% CI 1.36-5.87). A more irregular shape of periventricular or confluent WMH, as expressed by an increase in concavity index, was independently associated with a greater risk of vascular (HR 1.20, 95% CI 1.05–1.38 per SD increase) and nonvascular death (HR 1.21, 95% CI 1.03–1.42) and ischemic stroke (HR 1.28, 95% CI 1.05–1.55).ConclusionsWMH volume, type, and shape are associated with long-term risk of mortality and ischemic stroke in patients with manifest arterial disease.

scholarly journals Value of screening and follow‐up brain MRI scans in patients with metastatic melanoma

2021 ◽  
Author(s):  
Annemarie C. Eggen ◽  
Thijs T. Wind ◽  
Ingeborg Bosma ◽  
Miranda C. A. Kramer ◽  
Peter Jan Laar ◽  
...  
Keyword(s):  
Metastatic Melanoma ◽  
Brain Mri ◽  
Mri Scans

Chronic paroxysmal headache secondary to an orbital metastatic leiomyosarcoma: A case report

Cephalalgia ◽  
2017 ◽  
Vol 38 (2) ◽  
pp. 389-392 ◽  
Author(s):  
Hyun Ah Choi ◽  
Mi Ji Lee ◽  
Chin-Sang Chung
Keyword(s):  
Case Report ◽  
Brain Mri ◽  
Paroxysmal Hemicrania ◽  
Oblique Muscle ◽  
Beneficial Effects ◽  
Contrast Enhanced ◽  
Mri Scans ◽  
Chronic Paroxysmal Hemicrania ◽  
One Year

Background Intraorbital lesions associated with symptomatic trigeminal autonomic cephalalgias (TACs) are rarely reported. We present a case of orbital metastatic leiomyosarcoma, presenting with chronic paroxysmal hemicrania-like headache. Case report A 43-year-old man presented with a severe paroxysmal headache in his left periocular and frontal area for a year. The attacks occurred 10–12 times per day, lasting 10–15 minutes with ipsilateral lacrimation and conjunctival injection. Neurological examination and brain MRI without contrast were unremarkable. Different medications were tried, without beneficial effects. A follow-up contrast-enhanced brain MRI performed one year after the baseline MRI revealed an enhancing mass in the left superior oblique muscle. Orbital metastatic leiomyosarcoma arising from the thigh was revealed. He received gamma knife surgery, which completely resolved the headache. Discussion Intraorbital lesion should be considered a possibility in patients with headache mimicking TACs. Baseline contrast-enhanced MRI is essential, and repeated MRI scans might be needed if clinically indicated.

scholarly journals Exploratory Investigation of Brain MRI Lesions According to Whole Sample and Visual Function Subtyping in Children With Cerebral Visual Impairment

2022 ◽  
Vol 15 ◽  
Author(s):  
Hanna Sakki ◽  
Naomi J. Dale ◽  
Kshitij Mankad ◽  
Jenefer Sargent ◽  
Giacomo Talenti ◽  
...  
Keyword(s):  
Visual Impairment ◽  
Brain Mri ◽  
Visual Pathways ◽  
Brain Lesions ◽  
Total Brain ◽  
Variable Nature ◽  
Mri Scans ◽  
Group A ◽  
Group B ◽  
Cerebral Visual Impairment

Background: There is limited research on brain lesions in children with cerebral visual impairment (CVI) of heterogeneous etiologies and according to associated subtyping and vision dysfunctions. This study was part of a larger project establishing data-driven subtypes of childhood CVI according to visual dysfunctions. Currently there is no consensus in relation to assessment, diagnosis and classification of CVI and more information about brain lesions may be of potential diagnostic value.Aim: This study aimed to investigate overall patterns of brain lesions and associations with level of visual dysfunction and to compare the patterns between the classification subgroups in children with CVI.Methods: School-aged children with CVI received ophthalmological and neuro-psychological/developmental assessments to establish CVI-related subtyping. Other pediatric information was collected from medical records. MRI scans were coded according to a semi-quantitative template including brain regions (right hemisphere, left hemisphere, visual pathways) and summed for total scores. Non-parametric analyses were conducted.Results: 28 children had clinical brain MRI scans available [44% of total sample, Group A (lower severity of visual dysfunctions) n = 16, Group B (higher severity) n = 12]. Total brain scores ranged between 0 and 18 (Group A mdn = 7, IQR = 0.8–10.0, Group B mdn = 10, IQR = 6.5–11.8) and were widespread across regions. 71 per cent had post-geniculate visual pathway damage. The median total brain and hemisphere scores of Group B were higher than subgroup A but differences did not reach statistical significance. No statistically significant associations were found between brain scores and vision variables (acuity, contrast sensitivity).Conclusion: This study found a spread of lesions across all regions on the brain scans in children with congenital CVI. The majority had damage in the postgeniculate visual pathways and visual cortex region suggesting this is an area of interest and potentially informative for diagnosis. However the subtyping classification did not show differences in number or region of lesions though the trend was higher toward Group B. This study confirms the complex diffuse and variable nature of brain lesions in children with congenital CVI, many of whom have other neurological impairments.

Incidence of interattack asymptomatic brain lesions in NMO spectrum disorder

Neurology ◽  
2020 ◽  
Vol 95 (23) ◽  
pp. e3124-e3128
Author(s):  
Min Young Lee ◽  
Kok Pin Yong ◽  
Jae-Won Hyun ◽  
Su-Hyun Kim ◽  
Sang-Hyun Lee ◽  
...  
Keyword(s):  
Immunosuppressive Therapy ◽  
Brain Mri ◽  
Brain Lesion ◽  
Brain Lesions ◽  
Spectrum Disorder ◽  
Cancer Center ◽  
Conventional Mri ◽  
Monitoring Strategies ◽  
Mri Scans ◽  
Aqp4 Antibody

ObjectiveTo determine whether aquaporin-4 (AQP4) antibody–seropositive patients with neuromyelitis optica spectrum disorder (NMOSD) develop new asymptomatic brain lesions during the interattack period.MethodsOf 296 consecutive AQP4 antibody–seropositive patients in the NMOSD database of the National Cancer Center from May 2005 to November 2019, 145 patients, who had serial brain MRI scans over an interval of at least 1 year during relapse-free period after immunosuppressive therapy, with 370 longitudinally assessed brain MRI scans were included in this study. We retrospectively analyzed them for presence of new subclinical brain lesions during the relapse-free period.ResultsFive of 145 patients (3.4%) had detectable new, asymptomatic brain lesions in the deep white matter over a total observed relapse-free period of 708 person-years. All the lesions were smaller than 6 mm and assessed to be nonspecific. No brain lesion characteristic of NMOSD or gadolinium-enhancing lesion was identified.ConclusionsAsymptomatic brain lesions are rarely observed on conventional MRI in clinically stable AQP4 antibody–seropositive patients with NMOSD after immunosuppressive therapy and brain MRI lesions characteristic of NMOSD are not seen in the relapse-free period. These findings may provide further insight regarding currently known diagnostic and disease-monitoring strategies in NMOSD.

scholarly journals Recovery Nystagmus in Vestibular Neuritis with Minimal Canal Paresis. Clinical Observation and Interpretation

2022 ◽  
Vol 12 (1) ◽  
pp. 110
Author(s):  
Eleni Zoe Gkoritsa
Keyword(s):  
Time Course ◽  
Brain Mri ◽  
Vestibular Neuritis ◽  
Spontaneous Nystagmus ◽  
Vestibular Evoked Myogenic Potentials ◽  
Head Impulse Test ◽  
Slow Phase ◽  
Canal Paresis ◽  
Directional Preponderance

Recovery nystagmus in vestibular neuritis patients is a reversal of spontaneous nystagmus direction, beating towards the affected ear, observed along the time course of central compensation. It is rarely registered due either to its rarity as a phenomenon per se, or to the fact that it is missed between follow-up appointments. The aim of the manuscript is to describe in detail a case of recovery nystagmus found in an atypical case of vestibular neuritis and discuss pathophysiology and clinical considerations regarding this rare finding. A 26-year-old man was referred to our Otorhinolaryngology practice reporting “dizziness” sensation and nausea in the last 48 h. Clinical examination revealed left beating spontaneous nystagmus (average slow phase velocity aSPV 8.1°/s) with absence of fixation. The head impulse test (H.I.T.) was negative. Cervical vestibular evoked myogenic potentials (cVEMP) and Playtone audiometry (PTA) were normal. Romberg and Unterberger tests were not severely affected. A strong directional preponderance to the left was found in caloric vestibular test with minimal canal paresis (CP 13%) on the right. The first follow-up consultation took place on the 9th day after the onset of symptoms. Right beating weak (aSPV 2.4°/s) spontaneous nystagmus was observed with absence of fixation, whereas a strong right directional preponderance (DP) was found in caloric vestibular test. A brain MRI scan was ordered to exclude central causes of vertigo, which was normal. The patient was seen again completely free of symptoms 45 days later. He reported feeling dizzy during dynamic movements of the head and trunk for another 15 days after his second consultation. The unexpected observation of nystagmus direction reversal seven days after the first consultation is a typical sign of recovery nystagmus. Recovery nystagmus (RN) is centrally mediated and when found, it should always be carefully assessed in combination with the particularities of vestibular neuritis.

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