scholarly journals Limbic encephalitis with relapsing polychondritis: persistent white matter lesions and brain atrophy

2018 ◽  
Vol 46 (12) ◽  
pp. 5297-5302 ◽  
Author(s):  
Zilong Zhu ◽  
Decai Tian ◽  
Ning Ren ◽  
Zhihong Zhao ◽  
Xin Wang ◽  
...  
Keyword(s):  
White Matter ◽  
Limbic Encephalitis ◽  
Relapsing Polychondritis ◽  
White Matter Lesions ◽  
Autoimmune Disorder ◽  
Current Case ◽  
System Manifestation ◽  
Temporal Lobe Lesions ◽  
Chinese Male

Relapsing polychondritis (RP) is a rare autoimmune disorder affecting cartilage. Limbic encephalitis is a rare central nervous system manifestation of RP. This current case report describes a 66-year-old Chinese male patient who complained of developing myoclonus in the left leg, ataxia and speech difficulties 3 weeks prior to hospital admission. The patient presented with cognitive impairment, sleep disorder and extrapyramidal symptoms. The patient was diagnosed with RP that affected auricular cartilage, which also manifested as limbic encephalitis. Magnetic resonance imaging showed bilateral temporal lobe lesions involving the hippocampi and basal ganglia. Signal abnormalities in the white matter persisted during the 15-month follow-up period after treatment with corticosteroids and intravenous immunoglobulin. Over the same period, the bilateral hippocampi showed significant atrophy.

White Matter Changes and Cognitive Decline in a Ten-Year Follow-Up Period: A Pilot Study on a Single-Center Cohort from the Leukoaraiosis and Disability Study

2016 ◽  
Vol 41 (5-6) ◽  
pp. 303-313
Author(s):  
Sofia Madureira ◽  
Ana Verdelho ◽  
Carla Moleiro ◽  
Catarina Santos ◽  
Philip Scheltens ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
White Matter ◽  
Verbal Learning ◽  
White Matter Lesions ◽  
Disease Assessment ◽  
California Verbal Learning Test ◽  
Clinical Diagnoses ◽  
Neuropsychological Predictors ◽  
Learning Test

Aims: To describe the contribution of white matter lesions to the long-term neuropsychological profiles of different groups of clinical diagnoses, and to identify neuropsychological predictors of cognitive impairment in a 10-year follow-up. Methods: The Lisbon subcohort of the Leukoaraiosis and Disability (LADIS) study was re-evaluated performing a clinical, functional and cognitive evaluation [including Mini-Mental State Examination (MMSE), Alzheimer's Disease Assessment Scale - Cognition (ADAS-Cog) and ADAS-Cog with the extension for vascular impairment (VADAS-Cog), the 9-word version of the California Verbal Learning Test (CVLT-9), the Trail-Making test and the Stroop test] as well as an MRI scan. Using clinical diagnostic criteria, participants were identified as having no cognitive impairment (NI), cognitive impairment but no dementia (CIND) or dementia (DEM), and the effect of time on clinical diagnosis and neuropsychological profiles was analyzed. Results: From the initial group of 66 participants, 37 out of 41 survivors (90%) were re-evaluated (mean age 81.40 years, 57% women). Fifteen patients (41%) had DEM, 12 (32%) CIND and 10 (27%) NI. Over time, the three groups presented distinct profiles in the MMSE [F2, 62 = 15.85, p = 0.000], ADAS [F2, 62 = 15.85, p = 0.000] and VADAS [F2, 48 = 5.87, p = 0.008]. Logistic regression analysis identified higher scores on MMSE (β = 1.14, p = 0.03, OR = 3.13, 95% CI 1.09-8.97) as predictors of NI after 10 years of follow-up. Conclusion: Higher scores on baseline MMSE were the only neuropsychological predictors of NI after 10 years.

scholarly journals Do cerebral white matter lesions influence the rate of progression from mild cognitive impairment to dementia?

2012 ◽  
Vol 25 (1) ◽  
pp. 120-127 ◽  
Author(s):  
Michael E. Devine ◽  
J. Andres Saez Fonseca ◽  
Zuzana Walker
Keyword(s):  
Cognitive Impairment ◽  
Mild Cognitive Impairment ◽  
White Matter ◽  
White Matter Lesions ◽  
Cerebral White Matter ◽  
Brain Scans ◽  
Mri Brain ◽  
Cerebral White Matter Lesions ◽  
Ct And Mri

ABSTRACTBackground: Cerebral white matter lesions (WML), evident on CT and MRI brain scans, are histopathologically heterogeneous but associated with vascular risk factors and thought mainly to indicate ischemic damage. There has been disagreement over their clinical prognostic value in predicting conversion from mild cognitive impairment (MCI) to dementia.Methods: We scrutinised and rated CT and MRI brain scans for degree of WML in a memory clinic cohort of 129 patients with at least 1 year of follow-up. We examined the relationship between WML severity and time until conversion to dementia for all MCI patients and for amnestic (aMCI) and non-amnestic (naMCI) subgroups separately.Results: Five-year outcome data were available for 87 (67%) of the 129 patients. The proportion of patients converting to dementia was 25% at 1 year and 76% at 5 years. Patients with aMCI converted to dementia significantly earlier than those with naMCI. WML severity was not associated with time to conversion to dementia for either MCI patients in general or aMCI patients in particular. Among naMCI patients, there was a tendency for those with a low degree of WML to survive without dementia for longer than those with a high degree of WML. However, this was not statistically significant.Conclusions: MCI subtype is a significant independent predictor of conversion to dementia, with aMCI patients having higher risk than naMCI for conversion throughout the 5-year follow-up period. WML severity does not influence conversion to dementia for aMCI but might accelerate progression in naMCI.

A follow-up study of blood pressure and cerebral white matter lesions

1999 ◽  
Vol 46 (6) ◽  
pp. 827-833 ◽  
Author(s):  
Frank-Erik De Leeuw ◽  
Jan Cees De Groot ◽  
Matthijs Oudkerk ◽  
Jacqueline C. M. Witteman ◽  
Albert Hofman ◽  
...  
Keyword(s):  
Blood Pressure ◽  
White Matter ◽  
White Matter Lesions ◽  
Cerebral White Matter ◽  
Follow Up Study ◽  
Cerebral White Matter Lesions

Changes in Vascular Risk Factors from Midlife to Late Life and White Matter Lesions: A 20-Year Follow-Up Study

2011 ◽  
Vol 31 (2) ◽  
pp. 119-125 ◽  
Author(s):  
Miika Vuorinen ◽  
Alina Solomon ◽  
Suvi Rovio ◽  
Lasse Nieminen ◽  
Ingemar Kåreholt ◽  
...  
Keyword(s):  
Risk Factors ◽  
White Matter ◽  
White Matter Lesions ◽  
Late Life ◽  
Vascular Risk Factors ◽  
Vascular Risk ◽  
Follow Up Study

Slow Progression of White-Matter Changes

2003 ◽  
Vol 15 (S1) ◽  
pp. 173-176 ◽  
Author(s):  
Reinhold Schmidt ◽  
Helena Schmidt ◽  
Peter Kapeller ◽  
Franz Fazekas
Keyword(s):  
White Matter ◽  
Test Performance ◽  
Statistical Power ◽  
White Matter Lesions ◽  
White Matter Hyperintensity ◽  
Prevention Study ◽  
Clinical Predictors ◽  
Time Period ◽  
Slow Progression

A three-year follow-up of 273 participants (mean age 60 years) of the Austrian Stroke Prevention Study provides the first information on the rate, clinical predictors, and cognitive consequences of MRI white-matter lesions in elderly individuals without neuropsychiatric disease. Lesion progression was found in 17.9% of individuals over a time period of 3 years. Diastolic blood pressure and early confluent or confluent white-matter hyperintensities at baseline were the only significant predictors of white-matter hyperintensity progression. Lesion progression had no influence on the course of neuropsychologic test performance over the observational period, but the statistical power of this analysis was low.

scholarly journals A Patient with Limbic Encephalitis, Ear Perichondritis, and Episcleritis – An Unusual Presentation of Relapsing Polychondritis

2020 ◽  
Vol 12 (3) ◽  
pp. 378-386
Author(s):  
Stanley Angkodjojo ◽  
Crystal Jing Jing Yeo
Keyword(s):  
Limbic Encephalitis ◽  
Case Reports ◽  
Brain Magnetic Resonance Imaging ◽  
Relapsing Polychondritis ◽  
Response To Treatment ◽  
Diagnostic Challenge ◽  
Positron Emission ◽  
Pet Ct ◽  
System Manifestation ◽  
Recurrent Inflammation

Relapsing polychondritis (RPC) is a rare autoimmune disease that is characterized by recurrent inflammation and destruction of cartilaginous tissues. Limbic encephalitis is a rare central nervous system manifestation of RPC that has been mentioned in case reports. Recognition of this association, and reliable methods of diagnosis, including the utility of neuroimaging modalities such as positron emission tomography/computed tomography (PET/CT) can be useful in the evaluation of this diagnostic challenge. We report a patient with limbic encephalitis associated with RPC, where PET/CT was effectively used in the diagnosis, and monitoring of response to treatment. We also demonstrate that it can be a useful modality in certain situations when brain magnetic resonance imaging cannot be done.

scholarly journals Acute disseminated encephalomyelitis following endonasal resection of a craniopharyngioma: illustrative case

2021 ◽  
Vol 2 (16) ◽  
Author(s):  
Konrad Gag ◽  
Jonas Müller ◽  
Marie Süße ◽  
Robert Fleischmann ◽  
Henry W. S. Schroeder
Keyword(s):  
White Matter ◽  
White Matter Lesions ◽  
Acute Disseminated Encephalomyelitis ◽  
Intravenous Immunoglobulins ◽  
Neurological Deficits ◽  
Illustrative Case ◽  
Sensorimotor Deficits ◽  
Fluid Analysis ◽  
Magnetic Resonance Imaging Mri

BACKGROUND Acute disseminated encephalomyelitis (ADEM) is a rare, acquired demyelination syndrome that causes cognitive impairment and focal neurological deficits and may be fatal. The potentially reversible disease mainly affects children, often after vaccination or viral infection, but may be seen rarely in adults. OBSERVATIONS A 50-year-old woman presented with loss of visual acuity of the left eye. Magnetic resonance imaging (MRI) revealed an intra- and suprasellar mass, which was removed successfully. On postoperative day 1, MRI showed gross total resection of the lesion and no surgery-related complications. On postoperative day 2, the patient presented with a progressive left-sided hemiparesis, hemineglect, and decline of cognitive performance. MRI showed white matter edema in both hemispheres. Cerebrospinal fluid analysis revealed mixed pleocytosis (355/µL) without further evidence of infection. In synopsis of the findings, ADEM was diagnosed and treated with intravenous immunoglobulins. Shortly thereafter, the patient recovered, and no sensorimotor deficits were detected in the follow-up examination. LESSONS Pituitary gland pathologies are commonly treated by transsphenoidal surgery, with only minor risks for complications. A case of ADEM after craniopharyngioma resection has not been published before and should be considered in case of progressive neurological deterioration with multiple white matter lesions.

One-year Follow-up of Neuropsychology, MRI, rCBF and Glucose Metabolism (rMRGlu) in Cerebral Microangiopathy

2000 ◽  
Vol 39 (02) ◽  
pp. 43-49 ◽  
Author(s):  
D. Hellwig ◽  
M. Schreckenberger ◽  
R. Schneider ◽  
H.-J. Kaiser ◽  
G. Wagenknecht ◽  
...  
Keyword(s):  
White Matter ◽  
Brain Atrophy ◽  
White Matter Lesions ◽  
Neuropsychological Impairment ◽  
Cortical Damage ◽  
Head Holder ◽  
Cerebral Microangiopathy ◽  
One Year ◽  
Rcbf Spect

Summary Background: MRI shows lacunar infarctions (LI), deep white matter lesions (DWML) and atrophy in cerebral microangiopathy, which is said to lead to vascular dementia. In a first trial series on 57 patients with confirmed pure cerebral microangiopathy (without concomitant macroangiopathy), neuropsychological impairment and (where present) brain atrophy correlated with decreased rCBF and rMRGlu. LI and DWML did not correlate with either neuropsychological impairment or decreased rCBF/rMRGIu. This study was done one year later to detect changes in any of the study parameters. Methods: 26 patients were re-examined for rCBF, rMRGlu, LI, DWML, atrophy and neuropsychological performance (7 cognitive, 3 mnestic, 4 attentiveness tests). Using a special head holder for exact repositioning, rCBF (SPECT) and rMRGlu (PET) were measured and imaged slice by slice. White matter/cortex were quantified using MRI-defined ROIs. Results: After one year the patients did not show significant decreases in rCBF or rMRGlu either in cortex or in white matter (p >0.05), nor did any patient show LI, DWML or atrophy changes on MRI. There were no significant neuropsychological decreases (p >0.05). Conclusions: Cerebral microangiopathy ought to show progressive neuropsychological, functional (rCBF, rMRGlu) and morphological deterioration over periods >1 year. It is unlikely that direct cortical damage (e.g., incomplete infarction) is responsible for neuropsychological impairment since one-year follow-up of our patients revealed no progression of brain atrophy or any other cortical damage.

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