scholarly journals Susac’s Syndrome: A Case Presentation and Radiological Approach to this Rare Autoimmune Endotheliopathy

Medicina ◽  
2013 ◽  
Vol 49 (1) ◽  
pp. 3
Author(s):  
Goda Randakevičienė ◽  
Rymantė Gleiznienė ◽  
Rūta Nylander
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
White Matter ◽  
Resonance Imaging ◽  
Neurologic Disorder ◽  
Case Presentation ◽  
Susac’S Syndrome ◽  
Retinal Artery ◽  
Clinical Triad ◽  
The Brain

Susac’s syndrome is an uncommon neurologic disorder, consisting of the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. We report a case of a sudden vision and hearing impairment in a 35-year-old female patient. Magnetic resonance imaging of the brain revealed multiple lesions in the white matter and the corpus callosum, typical of Susac’s syndrome.

Susac's syndrome: a rare cause of fluctuating sensorineural hearing loss

1997 ◽  
Vol 111 (11) ◽  
pp. 1072-1074 ◽  
Author(s):  
Neil D. Bateman ◽  
Ian J. M. Johnson ◽  
Kevin P. Gibbin
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Sensorineural Hearing ◽  
Clinical Severity ◽  
Susac’S Syndrome ◽  
Retinal Artery ◽  
Clinical Triad ◽  
First Time

AbstractSusac's syndrome is extremely rare and is characterized by the clinical triad of encephalopathy, retinal artery occlusion and deafness. A case of this rare syndrome is presented and for the first time the fluctuating nature of the hearing loss is demonstrated. The site and nature of the hearing loss has been investigated. The value of neural hearing loss as a method of monitoring the clinical severity is discussed.

scholarly journals Ultrashort echo time (UTE) magnetic resonance imaging of the short T2 components in white matter of the brain using a clinical 3T scanner

NeuroImage ◽  
2014 ◽  
Vol 87 ◽  
pp. 32-41 ◽  
Author(s):  
Jiang Du ◽  
Guolin Ma ◽  
Shihong Li ◽  
Michael Carl ◽  
Nikolaus M. Szeverenyi ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Resonance Imaging ◽  
Ultrashort Echo Time ◽  
Short T2 ◽  
Echo Time ◽  
The Brain

The Snowball Effect

2018 ◽  
pp. 55-60
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand
Keyword(s):  
Hearing Loss ◽  
White Matter ◽  
Vision Loss ◽  
Demyelinating Disease ◽  
White Matter Lesions ◽  
Leptomeningeal Involvement ◽  
Immune Mediated ◽  
Retinal Artery ◽  
Deep Gray Matter ◽  
The Brain

Susac syndrome is a rare disease involving a triad of subacute encephalopathy, vision loss involving branch retinal artery occlusions (BRAOs), and sensorineural hearing loss. Clinical presentation is variable and generally does not involve the entire triad. The suspected pathophysiology is an immune-mediated endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear. MRI of the brain reveals abnormal white matter lesions similar to demyelinating disease, but with distinct characteristic central callosal lesions, as well as deep gray matter and leptomeningeal involvement. There is no standardized therapy for Susac’s, but suspicion of an immune-mediated endotheliopathy has informed empirical treatment with corticosteroids and various immunosuppressant regimens, which appear to improve the prognosis.

Clinical Correlates of White-Matter Changes on Magnetic Resonance Imaging Scans of the Brain

1991 ◽  
Vol 48 (10) ◽  
pp. 1015-1021 ◽  
Author(s):  
T. R. Mirsen ◽  
D. H. Lee ◽  
C. J. Wong ◽  
J. F. Diaz ◽  
A. J. Fox ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Resonance Imaging ◽  
White Matter Changes ◽  
Clinical Correlates ◽  
The Brain

scholarly journals Neuroradiological findings of an adolescent with early treated phenylketonuria: is phenylalanine restriction enough?

2011 ◽  
Vol 1 (2) ◽  
pp. 25
Author(s):  
Mayara Thays Beckhauser ◽  
Mirella Maccarini Peruchi ◽  
Gisele Rozone De Luca ◽  
Katia Lin ◽  
Sofia Esteves ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Dietary Restriction ◽  
Brain Function ◽  
Continuous Monitoring ◽  
Phenylalanine Hydroxylase ◽  
Resonance Imaging ◽  
Constant Control ◽  
The Brain

Phenylketonuria is caused by mutations in the enzyme phenylalanine hydroxylase gene, that can result in abnormal concentrations of phenylalanine on blood, resulting in metabolites that can cause brain damage. The treatment is based on dietary restriction of phenylalanine, and noncompliance with treatment may result in damage of the brain function. Brain abnormalities can be seen on magnetic resonance imaging of these individuals. Studies indicate that the appearance of abnormalities in white matter reflects high levels of phenylalanine on the blood. This case will show the clinical and neuroradiological aspects of a teenager with constant control of phenylalanine levels. Despite the continuous monitoring and early treatment, the magnetic resonance imaging identified impressive abnormalities in the white matter. This leads us to one question: is the restriction of phenylalanine sufficient to prevent changes in the white matter in patients with phenylketonuria?

White matter lesions in magnetic resonance imaging of the brain in 56 patients with visual vertigo

2018 ◽  
Vol 132 (06) ◽  
pp. 550-553 ◽  
Author(s):  
P A Dimitriadis ◽  
M Saad ◽  
M S Igra ◽  
R Mandavia ◽  
C Bowes ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Patient Group ◽  
White Matter Lesions ◽  
Control Group ◽  
Resonance Imaging ◽  
And Gender ◽  
Visual Vertigo ◽  
The Brain

AbstractBackgroundVisual vertigo is defined as a condition in which there is worsening or triggering of vestibular symptoms in certain visual environments. Previous studies have associated visual vertigo with an increased prevalence of underlying white matter lesions on brain imaging.MethodThis study evaluated the magnetic resonance imaging scans of the brain from a cohort of patients with visual vertigo, and compared the outcomes to an age- and gender-matched group of healthy volunteers.Results and conclusionWhite matter lesions were observed in 17.9 per cent of the patient group and in 16.3 per cent of the control group. The prevalence of white matter lesions in the patient group was not too different to that expected based on age.

scholarly journals Susac syndrome misdiagnosed as multiple sclerosis with exacerbation by interferon beta therapy

2017 ◽  
Vol 31 (2) ◽  
pp. 207-212 ◽  
Author(s):  
Hussein Algahtani ◽  
Bader Shirah ◽  
Muhammad Amin ◽  
Eyad Altarazi ◽  
Hashem Almarzouki
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Interferon Beta ◽  
Young Patients ◽  
Oligoclonal Bands ◽  
Resonance Imaging ◽  
Susac Syndrome ◽  
Retinal Artery

Susac syndrome is a rare autoimmune disorder characterised by the clinical triad of encephalopathy, retinopathy (branch retinal artery occlusions) and hearing loss. The diagnosis of Susac syndrome may be difficult initially, and it is not uncommon for patients with Susac syndrome to be misdiagnosed with multiple sclerosis. In this case report, we describe a patient who was diagnosed as having multiple sclerosis for three years, with further deterioration after starting treatment with interferon beta-1a. The patient had the triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. She had the classic magnetic resonance imaging appearance, with normal magnetic resonance imaging of the spinal cord and absence of oligoclonal bands in the cerebrospinal fluid. Our patient responded well to treatment with a combination therapy and discontinuation of interferon beta-1a. Our observations raise awareness about the importance of the early and correct diagnosis of Susac syndrome, which usually affects young patients, with an excellent prognosis if treated aggressively at an early stage of the disease. Susac syndrome is underdiagnosed and is not uncommonly misdiagnosed as multiple sclerosis. Susac syndrome is a great mimicker of multiple sclerosis, and establishing diagnostic criteria for this syndrome is very useful. In any patient presenting with a progressive disabling neurological disorder associated with callosal lesions and/or hearing loss, and/or visual loss especially in women, Susac syndrome should be suspected.

scholarly journals Leuko-Araiosis: An Ancient Term for a New Problem

1986 ◽  
Vol 13 (S4) ◽  
pp. 533-534 ◽  
Author(s):  
V.C. Hachinski ◽  
P. Potter ◽  
H. Merskey
Keyword(s):  
Magnetic Resonance Imaging ◽  
Central Nervous System ◽  
White Matter ◽  
Pathological Change ◽  
Medical Terminology ◽  
Resonance Imaging ◽  
Mr Images ◽  
The Central Nervous System ◽  
The Brain ◽  
Wide Usage

Abstract:Recent research with computerized tomography (CT) and magnetic resonance imaging (MR) of the brain has revealed a type of tissue change for which no fitting term exists. The change appears as areas of decreased density on CT and changed signals of the white matter in MR images. Because neither a definite pathological change nor a specific clinical deficit has been linked with the CT and MR changes, a designation is required that limits itself to describing the changes themselves.We propose “leuko-araiosis”. The Greek root leuko-, signifying “white”, has found wide usage in modern medical terminology: e.g. leucine, leukocyte, leukorrhoea. Also several precedents exist for its application to the white matter of the central nervous system: e.g. leukoencephalitis, leukomyelitis, leukotomy. Araios is an adjective meaning “rarefied”, and the suffix -osis converts the adjective to a noun meaning “rarefaction, diminution of density”, a word used in the Hippocratic Collection to describe a state of excessive porosity of the lung.A descriptive term precludes premature presuppositions and encourages the search for causes.

The Hidden Markov Brains

2013 ◽  
pp. 195-214
Author(s):  
Tuan D. Pham
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Neurodegenerative Diseases ◽  
White Matter Hyperintensities ◽  
Hidden Markov ◽  
Resonance Imaging ◽  
Brain Age ◽  
The Brain ◽  
Age Prediction

This chapter presents Hidden Markov models (HMM) of the brain on Magnetic Resonance Imaging (MRI) for the inference of white matter hyperintensities and brain age prediction to study the bidirectional vascular depression hypothesis in the elderly and neurodegenerative diseases, respectively. Rating and quantification of cerebral white matter hyperintensities on magnetic resonance imaging are important tasks in various clinical and scientific settings. The authors have proposed that prior knowledge about white matter hyperintensities can be accumulated and utilised to enable a reliable inference of the rating of a new white matter hyperintensity observation. The use of HMM for rating inference of white matter hyperintensities can be used as a computerized rating-assisting tool and can be very economical for diagnostic evaluation of brain tissue lesions. They have also applied HMM for MRI-based brain age prediction. Cortical thinning and intracortical gray matter volume losses are widely observed in normal ageing, while the decreasing rate of the volume loss in subjects with neurodegenerative diseases such as Alzheimer’s disease is reported to be faster than the average speed. Therefore, neurodegenerative disease is considered as accelerated aging. Accurate detection of accelerated ageing of the brain is a relatively new direction of research in computational neuroscience, as it has the potential to offer positive clinical outcome through early intervention.

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