scholarly journals Diagnostic dilemmas of Rasmussen’s encephalitis in adults

2018 ◽  
Vol 146 (9-10) ◽  
pp. 572-576
Author(s):  
Sladjana Andjelic

Introduction. Rasmussen?s encephalitis (RE) represents a rare, progressive, and inflammatory disease of the brain. Its detection in adults is a great challenge in clinical medicine. The aim of this paper is to highlight the diagnostic dilemma of RE in adults. Case outline. A 46-year-old woman was hospitalized due to persistent intense diffuse headaches, followed by nausea and the urge for vomiting that made her wake up during the night. On several occasions, she had transitory speech and memory disorders, and right hand numbness. Magnetic resonance (MR) imaging findings were as follows: occipitoparietal left in the deep white matter, as well as subcortical T2/flair white matter hyperintensities, T1-hypointense change involving the corpus callosum. MR spectroscopy showed an increased level of choline/creatinine (Cr) (2.12), a reduction of N-acetylaspartate/ Cr (1.27), an increased level of myo-inositol/Cr (1.20), and the presence of lactate. The patient refused lumbar puncture. Due to the described changes close to the speech center, cerebral biopsy was not taken. Even after five years, MR and spectroscopic findings are unchanged, while the clinical condition remains stable and unchanged. Conclusion. This case highlights the diagnostic dilemmas that arise in adult-onset RE and suggests that this diagnosis should be considered in patients of any age with the appropriate clinical picture.

2012 ◽  
Vol 33 (7) ◽  
pp. 1156-1167.e1 ◽  
Author(s):  
Sang Won Seo ◽  
Jong-Min Lee ◽  
Kiho Im ◽  
Jun-Sung Park ◽  
Sook-Hui Kim ◽  
...  

Author(s):  
Myriam Irislimane ◽  
François Guilbert ◽  
Jean-Maxime Leroux ◽  
Lionel Carmant ◽  
Dang Khoa Nguyen

A 52-year-old woman was referred for a progressive neurological condition which started a year before with continuous irregular twitching of the right facial and arm muscles as well as the tongue and palate, followed by progressive dysphagia, right hemiparesis, ataxia, dysphasia and dysarthria. Though magnetic resonance imaging (MRI) at clinical presentation and a year after were both normal (Figure A1), Rasmussen's encephalitis (RE) was strongly suspected and treatment with corticosteroids and immunoglobulins were begun. A third MRI, two years after onset, revealed mild T2 hyperintense subcortical white matter changes over the left perisylvian region (Figure A) which confirmed our initial suspicion of RE as she now met clinical, electrophysiological and morphological criterias for RE. While waiting for a cerebral biopsy after unsatisfactory response from antiepileptic drugs, corticosteroids and immunoglobulins, her condition markedly worsened less than three years after onset with the sudden occurrence of status epilepticus requiring intubation and continuous infusions of midazolam and propofol.


2019 ◽  
Vol 1 (1) ◽  
Author(s):  
Tracy d’Arbeloff ◽  
Maxwell L Elliott ◽  
Annchen R Knodt ◽  
Tracy R Melzer ◽  
Ross Keenan ◽  
...  

Abstract White matter hyperintensities proliferate as the brain ages and are associated with increased risk for cognitive decline as well as Alzheimer’s disease and related dementias. As such, white matter hyperintensities have been targeted as a surrogate biomarker in intervention trials with older adults. However, it is unclear at what stage of aging white matter hyperintensities begin to relate to cognition and if they may be a viable target for early prevention. In the Dunedin Study, a population-representative cohort followed since birth, we measured white matter hyperintensities in 843 45-year-old participants using T2-weighted magnetic resonance imaging and we assessed cognitive decline from childhood to midlife. We found that white matter hyperintensities were common at age 45 and that white matter hyperintensity volume was modestly associated with both lower childhood (ß = −0.08, P = 0.013) and adult IQ (ß=−0.15, P < 0.001). Moreover, white matter hyperintensity volume was associated with greater cognitive decline from childhood to midlife (ß=−0.09, P < 0.001). Our results demonstrate that a link between white matter hyperintensities and early signs of cognitive decline is detectable decades before clinical symptoms of dementia emerge. Thus, white matter hyperintensities may be a useful surrogate biomarker for identifying individuals in midlife at risk for future accelerated cognitive decline and selecting participants for dementia prevention trials.


2020 ◽  
Vol 78 (3) ◽  
pp. 907-910
Author(s):  
Eric Jouvent ◽  
Nassira Alili ◽  
Dominique Hervé ◽  
Hugues Chabriat

In a woman with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) followed for 15 years, we observed magnetic resonance imaging white matter hyperintensities that vanished in the anterior temporal poles while the brain volume decreased unexpectedly. These imaging changes were transient and detected when the patient was being treated by valproic acid for stabilizing mood disturbances. This intriguing case supports that mechanisms underlying white matter hyperintensities can vary from one brain area to another and that important modifications of water influx into the brain tissue might be involved in some imaging features of CADASIL.


2017 ◽  
Vol 56 (4) ◽  
pp. 1253-1262 ◽  
Author(s):  
Nagato Kuriyama ◽  
Masafumi Ihara ◽  
Toshiki Mizuno ◽  
Etsuko Ozaki ◽  
Daisuke Matsui ◽  
...  

2002 ◽  
Vol 59 (9) ◽  
pp. 785 ◽  
Author(s):  
Alan J. Thomas ◽  
John T. O'Brien ◽  
Sue Davis ◽  
Clive Ballard ◽  
Robert Barber ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document