scholarly journals Rapid Progression of White Matter Signal Changes and Frontotemporal Atrophy in Globular Glial Tauopathy

2020 ◽  
Author(s):  
Yuya Ohno ◽  
Toshimasa Ikeda ◽  
Keita Sakurai ◽  
Kentaro Yamada ◽  
Tatsuya Tomonari ◽  
...  
Keyword(s):  
White Matter ◽  
Rapid Progression ◽  
Globular Glial Tauopathy ◽  
Signal Changes

Clinical and Neuroimaging Changes of Subacute Sclerosing Panencephalitis – Experience 30 Cases in Tertiary Care Center in Bangladesh

2021 ◽  
Vol 2 (3) ◽  
pp. 1-4
Author(s):  
Gopen Kumar Kundu ◽  
Mohammad Monir Hossain
Keyword(s):  
Early Childhood ◽  
White Matter ◽  
Subacute Sclerosing Panencephalitis ◽  
Tertiary Care ◽  
Stage Ii ◽  
Cortical Atrophy ◽  
Periventricular White Matter ◽  
History Of ◽  
Signal Changes ◽  
Intense Signal

Background: Subacute sclersing panencephalitis (SSPE) is a very rare progressive, fatal neurodegenerative disease of the control nervous system of childhood and early adolescence. It is a slow virus disease caused by persistent defective measles virus infection of the brain Objective: To see the clinical andneuro-imaging findings in children with Subacute sclerosing panencephalitis. Methods: This retrospective study was conducted at Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, a tertiary care premier Postgraduate Medical Institution in Bangladesh. Thirty (30) Subacute sclerosing panencephalitis (SSPE) children were evaluated at paediatric neurology ward during the period January 2010 to December 2017. Diagnosis was based on typical clinical characteristic features, the presence of periodic discharges on EEG, demonstration of raised antibody titer against measles in the plasma and cerebrospinal fluid Detected by ELISA in all patients. Results: Total number of studied children were 30. Mean age was 10.2±3.1 year and Male female ratio was 5:1. Most of the patient arrived from poor socio-economic (83.33%) background of rural area (66.67%) of Bangladesh. Among them 46.67% had history of measles infection during early childhood. Progressive deterioration of school performance (50%), gait disturbance (70%), myoclonus (83%) dysarthria (43%) and Ocular manifestations like optic atrophy & papilledema (83.33%) were the main presenting feature of our studied children. All of the patients (100%) showed positive measles specific antibody IgG in CSF and On electroencephalographic findings showed periodic burst suppression in 90.90% cases. Most of the children (56.6%) were in stage II category and other 3.3%, 33.3%,6.6%, were stage I, stage III, stage IV category respectively. Neuroimaging study showed abnormalities in 45.83% cases included periventricular white matter hyper intense signal changes, cortical atrophy and ischaemic change. Conclusion: In our study most of the SSPE patient were in stage II.About half of the patient had history of measles infection during early childhood. Neuroimaging abnormalities found in about half of the cases and majority cases were in stage II. Common neuroimaging abnormalities were periventricular white matter hyper intense signal changes and cortical atrophy.

scholarly journals Bilateral Telencephalic Gliomatosis Cerebri in a Dog

2014 ◽  
Vol 2014 ◽  
pp. 1-5
Author(s):  
Mario Ricciardi ◽  
Antonio De Simone ◽  
Pasquale Giannuzzi ◽  
Maria Teresa Mandara ◽  
Alice Reginato ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
White Matter ◽  
Mass Effect ◽  
Gliomatosis Cerebri ◽  
Imaging Findings ◽  
Mri Findings ◽  
Intact Male ◽  
Presumptive Diagnosis ◽  
Atypical Cells ◽  
Signal Changes

An 8-year-old intact male Lagotto Romagnolo was presented with forebrain signs. Neuroanatomic localization was diffuse prosencephalon. MRI revealed diffuse, bilateral, and symmetric T2 and FLAIR hyperintensities in the parieto-occipital white matter and corpus callosum. No mass effect or contrast enhancement was noted. Analysis of cerebrospinal fluid revealed normal protein content and mild mononuclear pleocytosis. Atypical cells were not identified. 15 days later because of the worsening of clinical condition the patient was euthanized upon owner’s request. Neuropathological investigations were consistent with gliomatosis cerebri (GC). Such an unusual imaging pattern appeared similar to some cases of human GC and to a previous reported case in a dog, suggesting a possible repeatable imaging findings for this rare brain neoplasm. GC should be included in the MRI differentials for diffuse bilateral white matter signal changes and specific MRI findings described in this report may help in reaching a presumptive diagnosis of this tumor.

scholarly journals Symptomatic Bilateral Xanthogranuloma of the Choroid Plexus

2017 ◽  
Vol 08 (S 01) ◽  
pp. S123-S126 ◽  
Author(s):  
Selin Tural Emon ◽  
Erek Ozturk ◽  
Kaan Meric ◽  
Fugen Aker ◽  
Metin Orakdogen
Keyword(s):  
White Matter ◽  
Choroid Plexus ◽  
Brain Edema ◽  
Ventricular Dilatation ◽  
Benign Lesions ◽  
Additional Information ◽  
Signal Changes ◽  
Enhanced Imaging

ABSTRACTXanthogranulomas (XGRs) of the choroid plexus are rare, asymptomatic, and benign lesions usually found incidentally. Here, we present a case of a 47-year-old male with bilateral XGR of the choroid plexus with periventricular edema and discuss our case in relation to a review of existing literature pertaining to the radiology of XGRs. To the best of our knowledge, this is the first reported case of bilateral trigonal XGR causing brain edema without ventricular dilatation. Despite the fact that they can cause hydrocephalus, XGRs are silent and benign lesions. Although the etiopathology of XGRs remains poorly understood, enhanced imaging analyses may provide additional information regarding edema and focal white matter signal changes.

Abstract T P362: Prevalence and Clinicoradiological Profile of Japanese Patients with Alzheimer Disease Coexisting Multiple Microbleeds

Stroke ◽  
2014 ◽  
Vol 45 (suppl_1) ◽  
Author(s):  
Ken Ikeda ◽  
Takehisa Hirayama ◽  
Takanori Takazawa ◽  
Testuhito Kiyozuka ◽  
Masaru Yanagihashi ◽  
...  
Keyword(s):  
White Matter ◽  
Brain Mri ◽  
Japanese Patients ◽  
Brain Parenchyma ◽  
Rapid Progression ◽  
Amyloid Angiopathy ◽  
Mri Findings ◽  
Age Related ◽  
Magnetic Resonance Imaging Mri ◽  
Severe Degree

Background: Pathological findings of cerebral amyloid angiopathy (CAA) and Alzheimer’s disease (AD) coexist frequently. Both diseases are associated with β-amyloid deposition and dementia. We aimed to evaluate frequency and clinicoradiological profile of AD patients with multiple microbleeds (MBs) Methods: We reviewed clinical charts and magnetic resonance imaging (MRI) findings in patients with probable AD from 2009 to 2012, according to the DSM-IV. Brain MRI was performed at 1.5 T using protocols, including T2*-weighted gradient-echo or susceptibility-weighted imaging. MBs were defined as rounded, hypointense foci ≤ 10 mm in size in the brain parenchyma. MBs topography was divided into the lobar (L) and the deep/infratentorial (D/I) region. Multiple MBs were defined as the number ≥ 8 in the L or the D/I territory. White matter hyperintensities (WMH) were assessed using the age-related white matter changes scale. Clinicoradiological findings were examined for ≥ 1 year. Prevalence and clinicoradiological profiles were studied in patients with multiple L MBs or multiple D/I MBs. Results: Five hundred fifty patients (238 men and 312 women) were participated in the present study. Mean age (SD) was 78.4 (7.7) years, 78.3 (8.1) in men and 78.6 (7.5) in woman. A total of 132 patients (55 men and 78 women) had at least MB. Prevalence of total MBs was 24.0%, 23.1 in men and 25.0 in women. Multiple L MBs existed in 49 patients (8.9%), 15 men (6.3) and 34 women (10.9). Multiple D/I MBs were found in 44 patients (8.0%), 23 men (9.7) and 21 women (6.7). Clinical profiles of multiple L MBs revealed sudden deterioration, rapid progression, non-hypertension, higher rates of symptomatic subcortical hemorrhage and onset of CAA-associated leukoencephalopathy. Radiological hallmarks exhibited increased number and size of MBs, high score of WMH and asymptomatic subcortical hemorrhage. Clinicoradiological features of multiple D/I MBs exhibited higher rates of hypertension and variable degrees of WMH. Conclusions: The present study indicated that prevalence of multiple MBs was 16.9% in Japanese patients with AD. The clinicoradiological profile suggested severe degree of CAA in patients with multiple L MBs (8.9%) and hypertensive changes in patients with multiple D/I MBs (8.0%).

Isolated mild white matter signal changes in preterm infants: a regional approach for comparison of cranial ultrasound and MRI findings

2014 ◽  
Vol 34 (6) ◽  
pp. 476-482 ◽  
Author(s):  
M Weinstein ◽  
D Ben Bashat ◽  
V Gross-Tsur ◽  
Y Leitner ◽  
I Berger ◽  
...  
Keyword(s):  
White Matter ◽  
Preterm Infants ◽  
Cranial Ultrasound ◽  
Mri Findings ◽  
Regional Approach ◽  
Signal Changes

MRI signal changes in the white matter after corpus callosotomy

1999 ◽  
Vol 21 (4) ◽  
pp. 691-695 ◽  
Author(s):  
Divya S Khurana ◽  
Richard H Strawsburg ◽  
Richard L Robertson ◽  
Joseph R Madsen ◽  
Sandra L Helmers
Keyword(s):  
White Matter ◽  
Corpus Callosotomy ◽  
Signal Changes

scholarly journals Natural history of Krabbe disease – a nationwide study in Germany using clinical and MRI data

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Sarah Isabel Krieg ◽  
Ingeborg Krägeloh-Mann ◽  
Samuel Groeschel ◽  
Stefanie Beck-Wödl ◽  
Ralf A. Husain ◽  
...  
Keyword(s):  
White Matter ◽  
Natural History ◽  
Neurodegenerative Disorder ◽  
Tube Feeding ◽  
Krabbe Disease ◽  
First Year ◽  
Rapid Progression ◽  
Disease Course ◽  
History Of ◽  
First Year Of Life

Abstract Background Krabbe disease or globoid cell leukodystrophy is a severe neurodegenerative disorder caused by a defect in the GALC gene leading to a deficiency of the enzyme ß-galactocerebrosidase. The aim of this work was to describe the natural disease course covering the whole spectrum of the disease. Methods Natural history data were collected with a standardized questionnaire, supplemented by medical record data. We defined different forms of the disease according to Abdelhalim et al. (2014). Developmental and disease trajectories were described based on the acquisition and loss of milestones as well as the time of first clearly identifiable symptoms and needs such as spasticity, seizures and tube feeding. MRI was assessed using the scoring system by Loes et al. (1999) and in addition a pattern recognition approach, based on Abdelhalim et al. (2014). Results Thirty-eight patients were identified, from 27 of these patients 40 MRIs were available; 30 (79%) had an infantile onset, showing first symptoms in their first year of life, almost all (27 out of 30) starting in the first six months. A later onset after the first year of life was observed in 8 patients (21%, range 18 months to 60 years). Irritability, abnormalities in movement pattern as well as general developmental regression were the first symptoms in the infantile group; disease course was severe with rapid progression, e.g. loss of visual fixation, need for tube feeding and then an early death. Gait disorders were the first symptoms in all patients of the later onset groups; progression was variable. The different forms of the disease were characterized by different MRI patterns (infantile: diffuse white matter involvement and cerebellar structures specifically affected, later onset: parieto-occipital white matter and splenium affected, adult: motor tracts specifically affected). Conclusion This is the first description of the natural history of Krabbe disease in a larger European cohort using developmental, clinical and MRI data. We would like to highlight the very different clinical and MRI characteristics of the later onset forms. These data are important for counselling affected patients and families and may serve as a basis for future treatment trials.

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