scholarly journals Dementia with Leukoencephalopathy in Systemic Lupus Erythematosus

1991 ◽  
Vol 18 (3) ◽  
pp. 344-348 ◽  
Author(s):  
Andrew Kirk ◽  
Andrew Kertesz ◽  
Marsha J. Polk
Keyword(s):  
White Matter ◽  
Lupus Erythematosus ◽  
Memory Loss ◽  
Cerebral Atrophy ◽  
Hepatic Function ◽  
Subcortical White Matter ◽  
Oligoclonal Bands ◽  
Mri Findings ◽  
Recent Onset ◽  
Blood Brain Barrier Disruption

ABSTRACT:Neurologic manifestations, afflicting up to 70% of SLE patients, include psychosis, seizures, chorea, neuropathies, and stroke. MRI is useful in evaluation of lupus patients and several reports have documented cerebral atrophy or focal hyperintensities. We report an unusual MRI appearance in a 56-year-old woman with SLE, diagnosed on the basis of pleuritis, lymphopenia, anti-DNA antibodies, and neurologic involvement. She reported recent onset of Raynaud's phenomenon and generalized macular rash. She presented after two months of gradual deterioration with memory loss, flattened affect, dysphagia, dysarthria, anomia, and somnolence, without focal neurologic signs. Investigations included elevated ESR, reduced complement, normal CSF without oligoclonal bands, negative viral serology, normal hormone and vitamin levels, normal renal and hepatic function. Neuropsychologic testing showed widespread impairment (WAIS-R: FSIQ-63; WMS-69; DRS-98; RCPM-14; WAB AQ-78.8). CT was normal but MRI showed strikingly symmetric, confluent hyperintensities extensively involving cerebral and cerebellar white matter on Tl and T2 weighted scans. Basal ganglia and subependymal and subcortical white matter were spared. Treated with prednisone, the patient made a gradual, but incomplete, recovery. These MRI findings may reflect widespread vasculopathy or direct immunologic brain insult with or without imunologic blood-brain barrier disruption.

scholarly journals Jaw Dystonia and Reversible Basal Ganglia Changes as an Initial Presentation of Systemic Lupus Erythematosus

2017 ◽  
Vol 8 (1) ◽  
pp. 31-34 ◽  
Author(s):  
Meghan Romba ◽  
Yujie Wang ◽  
Shu-Ching Hu ◽  
Sandeep Khot
Keyword(s):  
Systemic Lupus Erythematosus ◽  
White Matter ◽  
Basal Ganglia ◽  
Brain Imaging ◽  
Lupus Erythematosus ◽  
Subcortical White Matter ◽  
High Dose ◽  
Neuropsychiatric Lupus ◽  
Systemic Lupus ◽  
Traditional Treatment

Dystonia as a manifestation of neuropsychiatric lupus erythematosus (NPSLE) is uncommon. We report a 25-year-old woman who experienced progressive confusion, reduced speech, and difficulty opening her mouth approximately 2 weeks after development of a facial rash. Brain imaging showed bilateral, symmetric signal abnormalities within the basal ganglia and subcortical white matter. Despite treatment with high-dose steroids, she continued to have difficulty speaking with evidence of jaw dystonia on examination. Jaw dystonia rapidly improved with the initiation of levodopa. Repeat evaluation 3 months later exhibited the absence of jaw dystonia and near resolution of the imaging abnormalities. Our patient demonstrated a unique presentation with jaw dystonia refractory to traditional treatment for NPSLE. Such a presentation likely represents a severe variant of NPSLE requiring both immunosuppressive and symptomatic therapies.

scholarly journals PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY IN AN ADULT PATIENT WITHSYSTEMICLUPUSERYTHEMATOSUS

2020 ◽  
Vol 8 (11) ◽  
pp. 791-798
Author(s):  
Khalid Abdullah Alghamdi ◽  
◽  
Ahmed Saeed Almaqati ◽  
Nuha Adnan Meraiani ◽  
Nawal Bassuni ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Lupus Erythematosus ◽  
Brain Mri ◽  
Immunosuppressive Agents ◽  
Clinical Manifestations ◽  
White Matter Lesions ◽  
Subcortical White Matter ◽  
Systemic Lupus ◽  
Mri Findings

We report a case of 40-year-old female who is known to have systemic lupus erythematosus (SLE) presenting with severe cognitive impairment with lymphopenia, proteinuria, and evidence of SLE serological activity. Initially, she was managed as a case of neuropsychiatric systemic lupus erythematosus (NPSLE) with Cyclophosphamide and pulse steroids. However, she has been deteriorating clinically in forms of right sided hemiparesis, blindness, and aphasia despite normalization of complements and anti-double stranded DNA antibodies levels. Diagnosis of progressive multifocal leukoencephalopathy (PML) was made based on her clinical manifestations with brain MRI findings of subcortical white matter lesions and detection of John Cunningham virus (JCV) in cerebrospinal fluid analysis. Immunosuppressive agents were discontinued aiming for immune system restoration.

Cognitive Dysfunction and White Matter Abnormalities in Systemic Lupus Erythematosus

2011 ◽  
Vol 17 (3) ◽  
pp. 385-392 ◽  
Author(s):  
Elizabeth Kozora ◽  
Christopher M. Filley
Keyword(s):  
Systemic Lupus Erythematosus ◽  
White Matter ◽  
Cognitive Dysfunction ◽  
Processing Speed ◽  
Lupus Erythematosus ◽  
Neuronal Damage ◽  
Cerebral Atrophy ◽  
Periventricular White Matter ◽  
Systemic Lupus ◽  
Neuroimaging Techniques

AbstractBrain abnormalities have been documented by neuropsychological assessment as well as a variety of neuroimaging techniques in patients with systemic lupus erythematosus (SLE). Conventional neuroimaging in patients with neuropsychiatric disease (NPSLE) typically discloses periventricular white matter (WM) hyperintensities, infarcts, hemorrhages, and cerebral atrophy. In SLE patients with none of these findings, sophisticated neuroimaging techniques have recently supported associations between microstructural WM abnormalities and abnormal attention, executive function, and processing speed. This mild cognitive dysfunction in SLE (MCD-SLE), which may result from early myelinopathy, precedes the more severe cognitive dysfunction of NPSLE, related to more obvious WM and neuronal damage. (JINS, 2011, 17, 385–392)

Magnetic Resonance Image Abnormality in Migraine with Aura

Cephalalgia ◽  
1991 ◽  
Vol 11 (3) ◽  
pp. 147-150 ◽  
Author(s):  
Dewey K Ziegler ◽  
Solomon Batnitzky ◽  
Ruth Barter ◽  
John H McMillan
Keyword(s):  
Magnetic Resonance ◽  
Cerebral Atrophy ◽  
White Matter Lesions ◽  
Subcortical White Matter ◽  
Resonance Imaging ◽  
Mri Findings ◽  
Cerebral Magnetic Resonance Imaging ◽  
Small Areas ◽  
Magnetic Resonance Imaging Mri ◽  
Age Range

Cerebral magnetic resonance imaging (MRI) was performed on 18 patients with migraine characterized by aura consisting of both visual symptoms and paresthesias. Fifteen headache-free individuals of the same age range were used as controls. Records were randomized and read in blind fashion by two neuroradiologists. Small subcortical white matter lesions were seen in three migraine cases and two controls. In one migraine case cortical infarctions were seen. In two controls, small areas of increased density similar to those in migraine were seen. No consistent correlation of migraine or its duration with cerebral atrophy was found. It is concluded that identification of both these MRI findings (small subcortical white lesions and cerebral atrophy) as significantly associated with migraine is doubtful.

What Has Direct Cortical and Subcortical Electrostimulation Taught Us about Neurolinguistics?

2019 ◽  
pp. 185-211
Author(s):  
Hugues Duffau
Keyword(s):  
White Matter ◽  
Large Scale ◽  
Functional Neuroimaging ◽  
Great Accuracy ◽  
Subcortical White Matter ◽  
Cerebral Lesions ◽  
Physiological Basis ◽  
Postoperative Mri ◽  
The Brain

Investigating the neural and physiological basis of language is one of the most important challenges in neurosciences. Direct electrical stimulation (DES), usually performed in awake patients during surgery for cerebral lesions, is a reliable tool for detecting both cortical and subcortical (white matter and deep grey nuclei) regions crucial for cognitive functions, especially language. DES transiently interacts locally with a small cortical or axonal site, but also nonlocally, as the focal perturbation will disrupt the entire subnetwork sustaining a given function. Thus, in contrast to functional neuroimaging, DES represents a unique opportunity to identify with great accuracy and reproducibility, in vivo in humans, the structures that are actually indispensable to the function, by inducing a transient virtual lesion based on the inhibition of a subcircuit lasting a few seconds. Currently, this is the sole technique that is able to directly investigate the functional role of white matter tracts in humans. Thus, combining transient disturbances elicited by DES with the anatomical data provided by pre- and postoperative MRI enables to achieve reliable anatomo-functional correlations, supporting a network organization of the brain, and leading to the reappraisal of models of language representation. Finally, combining serial peri-operative functional neuroimaging and online intraoperative DES allows the study of mechanisms underlying neuroplasticity. This chapter critically reviews the basic principles of DES, its advantages and limitations, and what DES can reveal about the neural foundations of language, that is, the large-scale distribution of language areas in the brain, their connectivity, and their ability to reorganize.

scholarly journals Preliminary Evidence for a Relationship between Elevated Plasma TNFα and Smaller Subcortical White Matter Volume in HCV Infection Irrespective of HIV or AUD Comorbidity

2021 ◽  
Vol 22 (9) ◽  
pp. 4953
Author(s):  
Natalie M. Zahr ◽  
Kilian M. Pohl ◽  
Allison J. Kwong ◽  
Edith V. Sullivan ◽  
Adolf Pfefferbaum
Keyword(s):  
White Matter ◽  
Proinflammatory Cytokines ◽  
Soluble Protein ◽  
Subcortical White Matter ◽  
Control Group ◽  
White Matter Volume ◽  
Brain Volumes ◽  
Protein Levels ◽  
Matter Volume ◽  
Patient Groups

Classical inflammation in response to bacterial, parasitic, or viral infections such as HIV includes local recruitment of neutrophils and macrophages and the production of proinflammatory cytokines and chemokines. Proposed biomarkers of organ integrity in Alcohol Use Disorders (AUD) include elevations in peripheral plasma levels of proinflammatory proteins. In testing this proposal, previous work included a group of human immunodeficiency virus (HIV)-infected individuals as positive controls and identified elevations in the soluble proteins TNFα and IP10; these cytokines were only elevated in AUD individuals seropositive for hepatitis C infection (HCV). The current observational, cross-sectional study evaluated whether higher levels of these proinflammatory cytokines would be associated with compromised brain integrity. Soluble protein levels were quantified in 86 healthy controls, 132 individuals with AUD, 54 individuals seropositive for HIV, and 49 individuals with AUD and HIV. Among the patient groups, HCV was present in 24 of the individuals with AUD, 13 individuals with HIV, and 20 of the individuals in the comorbid AUD and HIV group. Soluble protein levels were correlated to regional brain volumes as quantified with structural magnetic resonance imaging (MRI). In addition to higher levels of TNFα and IP10 in the 2 HIV groups and the HCV-seropositive AUD group, this study identified lower levels of IL1β in the 3 patient groups relative to the control group. Only TNFα, however, showed a relationship with brain integrity: in HCV or HIV infection, higher peripheral levels of TNFα correlated with smaller subcortical white matter volume. These preliminary results highlight the privileged status of TNFα on brain integrity in the context of infection.

scholarly journals Induced Pluripotent Stem Cell-Derived Neural Precursors Improve Memory, Synaptic and Pathological Abnormalities in a Mouse Model of Alzheimer’s Disease

Cells ◽  
2021 ◽  
Vol 10 (7) ◽  
pp. 1802
Author(s):  
Enrique Armijo ◽  
George Edwards ◽  
Andrea Flores ◽  
Jorge Vera ◽  
Mohammad Shahnawaz ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Clinical Symptoms ◽  
Memory Loss ◽  
Amyloid Β ◽  
Cerebral Atrophy ◽  
Brain Inflammation ◽  
Neural Precursors ◽  
Model Of Alzheimer’S Disease ◽  
Induced Pluripotent

Alzheimer’s disease (AD) is the most common type of dementia in the elderly population. The disease is characterized by progressive memory loss, cerebral atrophy, extensive neuronal loss, synaptic alterations, brain inflammation, extracellular accumulation of amyloid-β (Aβ) plaques, and intracellular accumulation of hyper-phosphorylated tau (p-tau) protein. Many recent clinical trials have failed to show therapeutic benefit, likely because at the time in which patients exhibit clinical symptoms the brain is irreversibly damaged. In recent years, induced pluripotent stem cells (iPSCs) have been suggested as a promising cell therapy to recover brain functionality in neurodegenerative diseases such as AD. To evaluate the potential benefits of iPSCs on AD progression, we stereotaxically injected mouse iPSC-derived neural precursors (iPSC-NPCs) into the hippocampus of aged triple transgenic (3xTg-AD) mice harboring extensive pathological abnormalities typical of AD. Interestingly, iPSC-NPCs transplanted mice showed improved memory, synaptic plasticity, and reduced AD brain pathology, including a reduction of amyloid and tangles deposits. Our findings suggest that iPSC-NPCs might be a useful therapy that could produce benefit at the advanced clinical and pathological stages of AD.

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