scholarly journals Multiple system atrophy: clinical-radiological correlation. Report of two cases

2007 ◽  
Vol 65 (2b) ◽  
pp. 512-515 ◽  
Author(s):  
Adolfo V. de Albuquerque ◽  
Marcos R.G. de Freitas ◽  
Daniel Cincinatus ◽  
Maria Beatriz B. Harouche
Keyword(s):  
Multiple System Atrophy ◽  
Neurodegenerative Disorder ◽  
Case Reports ◽  
Brain Mri ◽  
Olivopontocerebellar Atrophy ◽  
Striatonigral Degeneration ◽  
Lenticular Nucleus ◽  
The Brain

Multiple system atrophy (MSA) is a sporadic, neurodegenerative disorder, clinically characterized by parkinsonian, autonomic, cerebellar and pyramidal signs. We describe two patients showing different presentations of the same disease. The patient on case 1 presents features of MSA-C or olivopontocerebellar atrophy with the pontine "cross sign" on brain MRI. The second case reports a patient presenting MSA-P or striatonigral degeneration and the brain MRI shows lenticular nucleus sign alteration. We think that brain MRI might increase the accuracy diagnostic of MSA.

scholarly journals Neuropathology of multiple system atrophy: Kurt Jellinger`s legacy

2021 ◽  
Author(s):  
Nicole Campese ◽  
Alessandra Fanciulli ◽  
Nadia Stefanova ◽  
Johannes Haybaeck ◽  
Stefan Kiechl ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Neurodegenerative Disorder ◽  
Substantial Contribution ◽  
Olivopontocerebellar Atrophy ◽  
Striatonigral Degeneration ◽  
Cytoplasmic Inclusions ◽  
Glial Cytoplasmic Inclusions ◽  
Neurodegenerative Process ◽  
Disease Modifying Therapy ◽  
Current State

AbstractMultiple System Atrophy (MSA) is a rare, fatal neurodegenerative disorder. Its etiology and exact pathogenesis still remain poorly understood and currently no disease-modifying therapy is available to halt or slow down this detrimental neurodegenerative process. Hallmarks of the disease are α-synuclein rich glial cytoplasmic inclusions (GCIs). Neuropathologically, various degrees of striatonigral degeneration (SND) and olivopontocerebellar atrophy (OPCA) can be observed. Since the original descriptions of this multifaceted disorder, several steps forward have been made to clarify its neuropathological hallmarks and key pathophysiological mechanisms. The Austrian neuropathologist Kurt Jellinger substantially contributed to the understanding of the underlying neuropathology of this disease, to its standardized assessment and to a broad systematical clinic-pathological correlation. On the occasion of his 90th birthday, we reviewed the current state of the art in the field of MSA neuropathology, highlighting Prof. Jellinger’s substantial contribution.

Vocal fold abductor paralysis as a solitary and fatal manifestation of multiple system atrophy

1998 ◽  
Vol 112 (2) ◽  
pp. 177-178 ◽  
Author(s):  
R. G. M. Hughes ◽  
K. P. Gibbin ◽  
J. Lowe
Keyword(s):  
Multiple System Atrophy ◽  
Vocal Fold ◽  
Vocal Fold Paralysis ◽  
Pathological Process ◽  
Olivopontocerebellar Atrophy ◽  
Medium Term ◽  
Striatonigral Degeneration ◽  
Abductor Paralysis ◽  
Neurological Features ◽  
Magnetic Resonance Imaging Mri

AbstractA patient is presented who had bilateral abductor vocal fold paralysis pathologically proven to be due to multiple system atrophy (MSA) in the absence of other neurological features. MSA is a degenerative neurological condition that includes olivopontocerebellar atrophy, Shy-Drager syndrome and striatonigral degeneration. The usual predominant features of MSA are cerebellar ataxia, autonomic dysfunction and Parkinsonism. Stridor is present in over one third of patients and has been reported previously as a presenting symptom in MSA: however previously reported patients have always gone on to develop other neurological symptoms. The usual investigations of bilateral abductor vocal fold paralysis caused by MSA will not reveal the pathological process and we believe that magnetic resonance imaging (MRI) of the medulla and brain stem and autonomic function tests are probably the investigations of choice. It is a worthwhile exercise attempting to identify MSA as the cause of stridor as the prognosis is good in the medium term if appropriate support is offered.

scholarly journals A historical review of multiple system atrophy with a critical appraisal of cellular and animal models

2021 ◽  
Author(s):  
David J. Marmion ◽  
Wouter Peelaerts ◽  
Jeffrey H. Kordower
Keyword(s):  
Animal Models ◽  
Multiple System Atrophy ◽  
Neurodegenerative Disorder ◽  
Rem Sleep Behavior Disorder ◽  
Alpha Synuclein ◽  
Motor Symptom ◽  
Olivopontocerebellar Atrophy ◽  
Prodromal Phase ◽  
Sleep Behavior ◽  
Cytoplasmic Inclusions

AbstractMultiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), and dysautonomia with cerebellar ataxia or parkinsonian motor features. Isolated autonomic dysfunction with predominant genitourinary dysfunction and orthostatic hypotension and REM sleep behavior disorder are common characteristics of a prodromal phase, which may occur years prior to motor-symptom onset. MSA is a unique synucleinopathy, in which alpha-synuclein (aSyn) accumulates and forms insoluble inclusions in the cytoplasm of oligodendrocytes, termed glial cytoplasmic inclusions (GCIs). The origin of, and precise mechanism by which aSyn accumulates in MSA are unknown, and, therefore, disease-modifying therapies to halt or slow the progression of MSA are currently unavailable. For these reasons, much focus in the field is concerned with deciphering the complex neuropathological mechanisms by which MSA begins and progresses through the course of the disease. This review focuses on the history, etiopathogenesis, neuropathology, as well as cell and animal models of MSA.

Recent advances in neuropathology, biomarkers and therapeutic approach of multiple system atrophy

2017 ◽  
Vol 89 (2) ◽  
pp. 175-184 ◽  
Author(s):  
Shunsuke Koga ◽  
Dennis W Dickson
Keyword(s):  
Multiple System Atrophy ◽  
Cerebellar Ataxia ◽  
Neurodegenerative Disorder ◽  
Cytoplasmic Inclusion ◽  
Olivopontocerebellar Atrophy ◽  
Cytoplasmic Inclusions ◽  
Cellular Origin ◽  
Recent Advances ◽  
Neuronal Cytoplasmic Inclusions ◽  
Variable Combination

Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterised by a variable combination of autonomic failure, levodopa-unresponsive parkinsonism, cerebellar ataxia and pyramidal symptoms. The pathological hallmark is the oligodendrocytic glial cytoplasmic inclusion (GCI) consisting of α-synuclein; therefore, MSA is included in the category of α-synucleinopathies. MSA has been divided into two clinicopathological subtypes: MSA with predominant parkinsonism and MSA with predominant cerebellar ataxia, which generally correlate with striatonigral degeneration and olivopontocerebellar atrophy, respectively. It is increasingly recognised, however, that clinical and pathological features of MSA are broader than previously considered.In this review, we aim to describe recent advances in neuropathology of MSA from a review of the literature and from information derived from review of nearly 200 definite MSA cases in the Mayo Clinic Brain Bank. In light of these new neuropathological findings, GCIs and neuronal cytoplasmic inclusions play an important role in clinicopathological correlates of MSA. We also focus on clinical diagnostic accuracy and differential diagnosis of MSA as well as candidate biomarkers. We also review some controversial topics in MSA. Cognitive impairment, which has been a non-supporting feature of MSA, is considered from both clinical and pathological perspectives. The cellular origin of α-synuclein in GCI and a ‘prion hypothesis’ are discussed. Finally, completed and ongoing clinical trials targeting disease modification, including immunotherapy, are summarised.

scholarly journals Genome-wide association study of autopsy-confirmed Multiple System Atrophy identifies common variants near ZIC1 and ZIC4

2021 ◽  
Author(s):  
Franziska Hopfner ◽  
Anja Katharina Tietz ◽  
Viktoria C. Ruf ◽  
Owen Ross ◽  
Koga Shunsuke ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Association Study ◽  
Genome Wide Association Study ◽  
Chromosome 8 ◽  
Genome Wide Association ◽  
Chromosome 3 ◽  
Olivopontocerebellar Atrophy ◽  
Striatonigral Degeneration ◽  
Cytoplasmic Inclusions ◽  
Genome Wide

Multiple System Atrophy is a rare neurodegenerative disease with alpha-synuclein aggregation in glial cytoplasmic inclusions and either predominant olivopontocerebellar atrophy or striatonigral degeneration, leading to dysautonomia, parkinsonism, and cerebellar ataxia. One prior genome-wide association study in mainly clinically diagnosed patients with Multiple System Atrophy failed to identify genetic variants predisposing for the disease. Since the clinical diagnosis of Multiple System Atrophy yields a high rate of misdiagnosis when compared to the neuropathological gold standard, we studied common genetic variation in only autopsy-confirmed cases (N = 731) and controls (N = 2,898). The most strongly disease-associated markers were rs16859966 on chromosome 3 (P = 8.6 * 10-7, odds ratio (OR) = 1.58, [95% confidence interval (CI) = 1.32-1.89]), rs7013955 on chromosome 8 (P = 3.7 * 10-6, OR = 1.8 [1.40-2.31]), and rs116607983 on chromosome 4 (P = 4.0 * 10-6, OR = 2.93 [1.86-4.63]), all of which were supported by at least one additional genotyped and several imputed single nucleotide polymorphisms with P-values below 5 * 10-5. The genes closest to the chromosome 3 locus are ZIC1 and ZIC4 encoding the zinc finger proteins of cerebellum 1 and 4 (ZIC1 and ZIC4). Since mutations of ZIC1 and ZIC4 and paraneoplastic autoantibodies directed against ZIC4 are associated with severe cerebellar dysfunction, we conducted immunohistochemical analyses in brain tissue of the frontal cortex and the cerebellum from 24 Multiple System Atrophy patients. Strong immunohistochemical expression of ZIC4 was detected in a subset of neurons of the dentate nucleus in all healthy controls and in patients with striatonigral degeneration, whereas ZIC4 positive neurons were significantly reduced in patients with olivopontocerebellar atrophy. These findings point to a potential ZIC4-mediated vulnerability of neurons in Multiple System Atrophy.

Severe Head Injury linked to Subsequent Development of Malignant Brain Tumour within a Short Period- A Case Report

2018 ◽  
Vol 3 (2) ◽  
Keyword(s):  
Head Injury ◽  
Brain Tumour ◽  
Severe Head Injury ◽  
Case Reports ◽  
Subsequent Development ◽  
Malignant Brain Tumour ◽  
Tumour Development ◽  
Short Period ◽  
The Brain

There have been a few case reports of head injury leading to brain tumour development in the same region as the brain injury. Here we report a case where the patient suffered a severe head injury with contusion. He recovered clinically with conservative management. Follow up Computed Tomography scan of the brain a month later showed complete resolution of the lesion. He subsequently developed malignant brain tumour in the same region as the original contusion within a very short period of 15 months. Head injury patients need close follow up especially when severe. The link between severity of head injury and malignant brain tumour development needs further evaluation. Role of anti-inflammatory agents for prevention of post traumatic brain tumours needs further exploration.

Electromagnetic field in Alzheimer’s disease: a literature review of recent preclinical and clinical studies

2020 ◽  
Vol 17 ◽  
Author(s):  
Reem Habib Mohamad Ali Ahmad ◽  
Marc Fakhoury ◽  
Nada Lawand
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Neurodegenerative Disorder ◽  
In Vivo Studies ◽  
Key Factors ◽  
Potential Benefits ◽  
Preclinical And Clinical Studies ◽  
The Brain

: Alzheimer’s disease (AD) is a neurodegenerative disorder characterized by the progressive loss of neurons leading to cognitive and memory decay. The main signs of AD include the irregular extracellular accumulation of amyloidbeta (Aβ) protein in the brain and the hyper-phosphorylation of tau protein inside neurons. Changes in Aβ expression or aggregation are considered key factors in the pathophysiology of sporadic and early-onset AD and correlate with the cognitive decline seen in patients with AD. Despite decades of research, current approaches in the treatment of AD are only symptomatic in nature and are not effective in slowing or reversing the course of the disease. Encouragingly, recent evidence revealed that exposure to electromagnetic fields (EMF) can delay the development of AD and improve memory. This review paper discusses findings from in vitro and in vivo studies that investigate the link between EMF and AD at the cellular and behavioural level, and highlights the potential benefits of EMF as an innovative approach for the treatment of AD.

scholarly journals Content-Based Estimation of Brain MRI Tilt in Three Orthogonal Directions

2021 ◽  
Author(s):  
Pooja Prabhu ◽  
A. K. Karunakar ◽  
Sanjib Sinha ◽  
N. Mariyappa ◽  
G. K. Bhargava ◽  
...  
Keyword(s):  
Large Scale ◽  
Brain Mri ◽  
Similarity Measures ◽  
Principal Component ◽  
Brain Anatomy ◽  
Morphological Operations ◽  
Mr Images ◽  
Tilt Correction ◽  
Brain Mr Images ◽  
The Brain

AbstractIn a general scenario, the brain images acquired from magnetic resonance imaging (MRI) may experience tilt, distorting brain MR images. The tilt experienced by the brain MR images may result in misalignment during image registration for medical applications. Manually correcting (or estimating) the tilt on a large scale is time-consuming, expensive, and needs brain anatomy expertise. Thus, there is a need for an automatic way of performing tilt correction in three orthogonal directions (X, Y, Z). The proposed work aims to correct the tilt automatically by measuring the pitch angle, yaw angle, and roll angle in X-axis, Z-axis, and Y-axis, respectively. For correction of the tilt around the Z-axis (pointing to the superior direction), image processing techniques, principal component analysis, and similarity measures are used. Also, for correction of the tilt around the X-axis (pointing to the right direction), morphological operations, and tilt correction around the Y-axis (pointing to the anterior direction), orthogonal regression is used. The proposed approach was applied to adjust the tilt observed in the T1- and T2-weighted MR images. The simulation study with the proposed algorithm yielded an error of 0.40 ± 0.09°, and it outperformed the other existing studies. The tilt angle (in degrees) obtained is ranged from 6.2 ± 3.94, 2.35 ± 2.61, and 5 ± 4.36 in X-, Z-, and Y-directions, respectively, by using the proposed algorithm. The proposed work corrects the tilt more accurately and robustly when compared with existing studies.

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